Corticosteroids Health Dictionary

Asthma is a common disorder of breathing characterised by widespread narrowing of smaller airways within the lung. In the UK the prevalence among children in the 5–12 age group is around 10 per cent, with up to twice the number of boys affected as girls. Among adults, however, the sex incidence becomes about equal. The main symptom is shortness of breath. A major feature of asthma is the reversibility of the airway-narrowing and, consequently, of the breathlessness. This variability in the obstruction may occur spontaneously or in response to treatment.

Cause Asthma runs in families, so that parents with asthma have a strong risk of having children with asthma, or with other atopic (see ATOPY) illnesses such as HAY FEVER or eczema (see DERMATITIS). There is therefore a great deal of interest in the genetic basis of the condition. Several GENES seem to be associated with the condition of atopy, in which subjects have a predisposition to form ANTIBODIES of the IgE class against allergens (see ALLERGEN) they encounter – especially inhaled allergens.

The allergic response in the lining of the airway leads to an in?ammatory reaction. Many cells are involved in this in?ammatory process, including lymphocytes, eosinophils, neutrophils and mast cells. The cells are attracted and controlled by a complex system of in?ammatory mediators. The in?amed airway-wall produced in this process is then sensitive to further allergic stimuli or to non-speci?c challenges such as dust, smoke or drying from the increased respiration during exercise. Recognition of this in?ammation has concentrated attention on anti-in?ammatory aspects of treatment.

Continued in?ammation with poor control of asthma can result in permanent damage to the airway-wall such that reversibility is reduced and airway-narrowing becomes permanent. Appropriate anti-in?ammatory therapy may help to prevent this damage.

Many allergens can be important triggers of asthma. House-dust mite, grass pollen and animal dander are the commonest problems. Occupational factors such as grain dusts, hard-metals fumes and chemicals in the plastic and paint industry are important in some adults. Viral infections are another common trigger, especially in young children.

The prevalence of asthma appears to be on the increase in most countries. Several factors have been linked to this increase; most important may be the vulnerability of the immature immune system (see IMMUNITY) in infants. High exposure to allergens such as house-dust mite early in life may prime the immune system, while reduced exposure to common viral infections may delay the maturation of the immune system. In addition, maternal smoking in pregnancy and infancy increases the risk.

Clinical course The major symptoms of asthma are breathlessness and cough. Occasionally cough may be the only symptom, especially in children, where night-time cough may be mistaken for recurrent infection and treated inappropriately with antibiotics.

The onset of asthma is usually in childhood, but it may begin at any age. In childhood, boys are affected more often than girls but by adulthood the sex incidence is equal. Children who have mild asthma are more likely to grow out of the condition as they go through their teenaged years, although symptoms may recur later.

The degree of airway-narrowing, and its change with time and treatment, can be monitored by measuring the peak expiratory ?ow with a simple monitor at home – a peak-?ow meter. The typical pattern shows the peak ?ow to be lowest in the early morning and this ‘morning dipping’ is often associated with disturbance of sleep.

Acute exacerbations of asthma may be provoked by infections or allergic stimuli. If they do not respond quickly and fully to medication, expert help should be sought urgently since oxygen and higher doses of drugs will be necessary to control the attack. In a severe attack the breathing rate and the pulse rate rise and the chest sounds wheezy. The peak-?ow rate of air into the lungs falls. Patients may be unable to talk in full sentences without catching their breath, and the reduced oxygen in the blood in very severe attacks may produce the blue colour of CYANOSIS in the lips and tongue. Such acute attacks can be very frightening for the patient and family.

Some cases of chronic asthma are included in the internationally agreed description CHRONIC OBSTRUCTIVE PULMONARY DISEASE (COPD) – a chronic, slowly progressive disorder characterised by obstruction of the air?ow persisting over several months.

Treatment The ?rst important consideration in the treatment of asthma is avoidance of precipitating factors. When this is a speci?c animal or occupational exposure, this may be possible; it is however more di?cult for house-dust mite or pollens. Exercise-induced asthma should be treated adequately rather than avoiding exercise.

Desensitisation injections using small quantities of speci?c allergens are used widely in some countries, but rarely in the UK as they are considered to have limited value since most asthma is precipitated by many stimuli and controlled adequately with simple treatment.

There are two groups of main drugs for the treatment of asthma. The ?rst are the bronchodilators which relax the smooth muscle in the wall of the airways, increase their diameter and relieve breathlessness. The most useful agents are the beta adrenergic agonists (see ADRENERGIC RECEPTORS) such as salbutamol and terbutaline. They are best given by inhalation into the airways since this reduces the general side-effects from oral use. These drugs are usually given to reverse airway-narrowing or to prevent its onset on exercise. However, longer-acting inhaled beta agonists such as salmeterol and formoterol or the theophyllines given in tablet form can be used regularly as prevention. The beta agonists can cause TREMOR and PALPITATION in some patients.

The second group of drugs are the antiin?ammatory agents that act to reduce in?ammation of the airway. The main agents in this group are the CORTICOSTEROIDS. They must be taken regularly, even when symptoms are absent. Given by inhalation they have few side-effects. In acute attacks, short courses of oral steroids are used; in very severe disease regular oral steroids may be needed. Other drugs have a role in suppressing in?ammation: sodium cromoglycate has been available for some years and is generally less e?ective than inhaled steroids. Newer agents directed at speci?c steps in the in?ammatory pathway, such as leukotriene receptor-antagonists, are alternative agents.

Treatment guidelines have been produced by various national and international bodies, such as the British Thoracic Society. Most have set out treatment in steps according to severity, with objectives for asthma control based on symptoms and peak ?ow. Patients should have a management plan that sets out their regular treatment and their appropriate response to changes in their condition.

Advice and support for research into asthma is provided by the National Asthma Campaign.

See www.brit-thoracic.org.uk

Prognosis Asthma is diagnosed in 15–20 per cent of all pre-school children in the developed world. Yet by the age of 15 it is estimated that fewer than 5 per cent still have symptoms. A study in 2003 reported on a follow-up of persons born in 1972–3 who developed asthma and still had problems at the age of nine. By the time these persons were aged 26, 27 per cent were still having problems; around half of that number had never been free from the illness and the other half had apparently lost it for a few years but it had returned.... asthma

Also known as periarteritis nodosa. A rare but potentially serious disease, probably caused by a disturbance of the immune system (see IMMUNITY). Prolonged fever and obscure symptoms referable to any system of the body are associated with local areas of in?ammation along the arteries, giving rise to nodules in their walls. Large doses of CORTICOSTEROIDS, coupled with IMMUNOSUPPRESSANT treatment, usually curtail the disorder. Recovery occurs in about 50 per cent of cases.... polyarteritis nodosa

A chronic in?ammation of the synovial lining (see SYNOVIAL MEMBRANE) of several joints, tendon sheaths or bursae which is not due to SEPSIS or a reaction to URIC ACID crystals. It is distinguished from other patterns of in?ammatory arthritis by the symmetrical involvement of a large number of peripheral joints; by the common blood-?nding of rheumatoid factor antibody; by the presence of bony erosions around joints; and, in a few, by the presence of subcutaneous nodules with necrobiotic (decaying) centres.

Causes There is a major immunogenetic predisposition to rheumatoid arthritis in people carrying the HLA-DR4 antigen (see HLA SYSTEM). Other minor immunogenetic factors have also been implicated. In addition, there is a degree of familial clustering which suggests other unidenti?ed genetic factors. Genetic factors cannot alone explain aetiology, and environmental and chance factors must be important, but these have yet to be identi?ed.

Epidemiology Rheumatoid arthritis more commonly occurs in women from the age of 30 onwards, the sex ratio being approximately 4:1. Typical rheumatoid arthritis may occur in adolescence, but in childhood chronic SYNOVITIS usually takes one of a number of di?erent patterns, classi?ed under juvenile chronic arthritis.

Pathology The primary lesion is an in?ammation of the synovial membrane of joints. The synovial ?uid becomes diluted with in?ammatory exudate: if this persists for months it leads to progressive destruction of articular CARTILAGE and BONE. Cartilage is replaced by in?ammatory tissue known as pannus; a similar tissue invades bone to form erosions. Synovitis also affects tendon sheaths, and may lead to adhesion ?brosis or attrition and rupture of tendons. Subcutaneous and other bursae may be involved. Necrobiotic nodules also occur at sites outside synovium, including the subcutaneous tissues, the lungs, the pericardium and the pleura.

Clinical features Rheumatoid arthritis varies from the very mild to the severely disabling. Many mild cases probably go undiagnosed. At least 50 per cent of patients continue to lead a reasonably normal life; around 25 per cent are signi?cantly disabled in terms of work and leisure activities; and a minority become markedly disabled and are limited in their independence. There is often an early acute phase, followed by substantial remission, but in other patients gradual step-wise deterioration may occur, with progressive involvement of an increasing number of joints.

The diagnosis of rheumatoid arthritis is largely based on clinical symptoms and signs. Approximately 70 per cent of patients have rheumatoid factor ANTIBODIES in the SERUM but, because of the large number of false positives and false negatives, this test has very little value in clinical practice. It may be a useful pointer to a worse prognosis in early cases if the level is high. X-RAYS may help in diagnosing early cases and are particularly helpful when considering surgery or possible complications such as pathological fracture. Patients commonly develop ANAEMIA, which may be partly due to gastrointestinal blood loss from antiin?ammatory drug treatment (see below).

Treatment involves physical, pharmacological, and surgical measures, together with psychological and social support tailored to the individual patient’s needs. Regular activity should be maintained. Resting of certain joints such as the wrist with splints may be helpful at night or to assist prolonged manual activities. Sound footwear is important. Early use of antirheumatic drugs reduces long-term disability. Drug treatment includes simple ANALGESICS, NON-STEROIDAL ANTI-INFLAMMATORY DRUGS (NSAIDS), and slow-acting drugs including GOLD SALTS (in the form of SODIUM AUROTHIOMALATE), PENICILLAMINE, SULFASALAZINE, METHOTREXATE and AZATHIOPRINE.

The non-steroidal agents are largely e?ective in reducing pain and early-morning sti?ness, and have no e?ect on the chronic in?ammatory process. It is important, especially in the elderly, to explain to patients the adverse effects of NSAIDs, the dosage of which can be cut by prescribing paracetamol at the same time. Combinations of anti-rheumatic drugs seem better than single agents. The slow-acting drugs take approximately three months to act but have a more global e?ect on chronic in?ammation, with a greater reduction in swelling and an associated fall in erythrocyte sedimentation rate (ESR) and rise in the level of HAEMOGLOBIN. Local CORTICOSTEROIDS are useful, given into individual joints. Systemic corticosteroids carry serious problems if continued long term, but may be useful under special circumstances. Much research is currently going on into the use of tumour necrosis factor antagonists such as INFLIXIMAB and etanercept, but their precise role remains uncertain.... rheumatoid arthritis

An early corticosteroid drug (see CORTICOSTEROIDS), now obsolete and replaced by PREDNISOLONE and HYDROCORTISONE.... cortisone

A CORTICOSTEROIDS derivative. As an antiin?ammatory agent it is approximately 30 times as e?ective as cortisone and eight times as e?ective as prednisolone. On the other hand, it has practically none of the salt-retaining properties of cortisone.... dexamethasone

Introduction of ?uid into the RECTUM via the

Percutaneous nephroscope used for examining the interior of the kidney. It is passed into the pelvis of the kidney through a track from the surface of the skin. (The track is made with a needle and guide wire.) Instruments can be passed through the nephroscope under direct vision to remove calculi.

ANUS. Enemas may be given to clear the intestine of faeces prior to intestinal surgery or to relieve severe constipation. They may also be used to give barium for diagnostic X-rays as well as drugs such as CORTICOSTEROIDS, used to treat ULCERATIVE COLITIS. The patient is placed on his or her side with a support under the hips. A catheter (see CATHETERS) with a lubricated end is inserted into the rectum and warmed enema ?uid gently injected. Disposable enemas and miniature enemas, which can be self-administered, are widely used; they contain preprepared solution.... enema

A connective-tissue disease affecting the muscles throughout the body. This rare disorder, which is associated with DERMATOMYOSITIS, may be acute or chronic but it usually affects the muscles of the shoulders or hip areas. The muscles weaken and are tender to the touch. Di?use in?ammatory changes occur and symptomatic relief may be obtained with CORTICOSTEROIDS.... polymyositis

Also known as salaam attacks, these are a rare but serious type of EPILEPSY, usually starting in the ?rst eight months of life. The spasms are short and occur as involuntary ?exing of the neck, arms, trunk and legs. They may occur several times a day. If the baby is sitting, it may collapse into a ‘salaam’ position; more usually there is a simple body jerk, sometimes accompanied by a sudden cry. An electroencephalogram (see ELECTROENCEPHALOGRAPHY (EEG)) shows a picture of totally disorganised electrical activity called hypsarrhythmia. The condition results from any one of many brain injuries, infections or metabolic insults that may have occurred before, during, or in the ?rst few months after birth. Its importance is that in most cases, the baby’s development is seriously affected such that they are likely to be left with a profound learning disability. Consequently, prompt diagnosis is important. Treatment is with CORTICOSTEROIDS or with certain anti-convulsants – the hope being that prompt and aggressive treatment might prevent further brain damage leading to learning disability.... infantile spasms

An uncommon chronic in?ammatory disease of unknown origin which can affect many organs, particularly the SKIN, eyes (see EYE) and LUNGS. Commonly, it presents as ERYTHEMA nodosum in association with lymph-gland enlargement within the chest. In the eyes it causes UVEITIS. BIOPSY of affected tissue allows diagnosis, which is con?rmed by a KVEIM TEST. Often sarcoidosis is self-limiting, but in severe cases oral CORTICOSTEROIDS may be needed.... sarcoidosis

This chronic, relapsing in?ammatory skin disease is extremely common, affecting about 2 per cent of the UK population. Frequently it is mild and trivial, affecting only the points of the elbows or knees and the scalp, but in a substantial minority of sufferers the disease is much more widespread and causes considerable discomfort and social embarrassment. Rarely, it can be universal and even life-threatening.

The predisposition to psoriasis is genetic, multiple genes being involved, but postnatal factors such as acute infection, hormonal disturbance, pregnancy and drugs can in?uence or provoke it. The sexes are equally affected and onset is most common in the second or third decade of life.

The psoriatic lesion is dull red, scaly and well de?ned. Scale is shed constantly, either in tiny pieces or as large plaques. The scalp is usually affected but the disease does not cause signi?cant hair loss. The ?ngernails may be pitted or ridged and the toenails grossly thickened. Several clinical patterns occur: in guttate psoriasis, a sudden explosion of multiple tiny lesions may follow a streptococcal throat infection, especially in children. Larger lesions are characteristic of discoid (plaque) psoriasis, the usual adult form. In the elderly the plaques may be mainly in the large body folds – ?exural psoriasis. Rarely, psoriasis may be universal (psoriatic erythroderma), or a sterile pustular eruption may supervene (pustular psoriasis).

Mucous membranes in the mouth and elsewhere are not affected. Psoriasis does not affect internal organs, but in about 1 per cent of subjects an in?ammatory joint disease (psoriatic arthritis) may be associated with the condition.

Treatment There is no absolute cure, but several agents used topically are of value including coal-tar extracts, DITHRANOL, CORTICOSTEROIDS and synthetic derivatives of vitamins A and D. Ultraviolet B phototherapy (and natural sunlight) bene?ts most but not all psoriatics. Systemic therapy, including PHOTOCHEMOTHERAPY, is reserved for severe forms of psoriasis. METHOTREXATE, CICLOSPORIN A and oral RETINOIDS are the most e?ective drugs, but they are potentially dangerous and require expert monitoring.

Patient information may be obtained through the Psoriasis Association.... psoriasis

Chronic inflammation and ulceration of the gut, especially the terminal ileum from changes in the gut blood vessels. Commences with ulceration which deepens, becomes fibrotic and leads to stricture. Defective immune system. Resistance low. May be associated with eye conditions and Vitamin B12 deficiency.

Symptoms: malaise, bloody alternating diarrhoea and constipation; right side colicky abdominal pain worse after meals; flatulence, loss of weight and appetite. Intestinal obstruction can usually be palpated. Blood count. A blood count high in whites indicates an abscess – a serious condition which may require surgical repair during which segments of the gut may have to be removed. Malignant change rare. Differential diagnosis. Ulcerative colitis, appendicitis, appendix abscess, irritable bowel syndrome.

Cracks or ulcers at corners of the mouth may be a good marker of Crohn’s Disease.

Treatment. Select one of the following. Herbal treatment offers a safe alternative to steroids by inducing remission in acute exacerbation. Good responses have been observed from the anti-bacterials Wild Yam and Goldenseal. Fenugreek seeds are of special value. Comfrey (tissue regeneration). Irish Moss.

Teas: Chamomile, Comfrey leaves, Hops, Marshmallow leaves, Meadowsweet, Shepherd’s Purse (Dr A. Vogel), Lobelia. Silverweed and Cranesbill are excellent for internal bleeding; Poke root for intestinal ulceration.

Decoction. Fenugreek seeds: 2 teaspoons to large cup water simmered gently 10 minutes. 1 cup freely. The seeds also should be consumed.

Tablets/capsules. Wild Yam, Fenugreek, Ginger, Goldenseal, Lobelia, Slippery Elm.

Powders. Formula. Wild Yam 2; Meadowsweet 2; Goldenseal 1. Dose: 500mg (two 00 capsules or one- third teaspoon) thrice daily.

Liquid Extracts. (1) Formula. Wild Yam 1, Echinacea 2. 30-60 drops in water thrice daily. Or, (2) Formula: Turkey Rhubarb 2, Goldenseal 1, Caraway half. 20-30 drops in water thrice daily.

Tinctures. Formula. Bayberry 2, Goldenseal 1, Cardamoms 1. Dose: One to two 5ml teaspoons thrice daily.

Ispaghula seeds. 2-4 teaspoons thrice daily.

Tea Tree oil Suppositories. Insertion at night.

Diet. Bland, little fibre, Slippery Elm gruel. Irish Moss preparations. Increase fluid intake. Reject: broccoli, tomatoes, lima, Soya, Brussels sprouts, pinto beans, cocoa, chocolate, cow’s milk, peas, onions, turnips, radishes. Accept fish oils.

Addenbrookes Hospital, Cambridge. Reject foods containing wheat and all dairy produce.

Supplements. Vitamins A, B12, C, Calcium, Iron, Magnesium, Potassium, Zinc.

Study. In a study carried out by UK researchers (1993) food allergies were found to be the most common cause of the disease. Results suggested that dietary changes may be as effective as corticosteroids in easing symptoms. The most common allergens were corn, wheat, milk, yeast, egg, potato, rye, tea, coffee, apples, mushrooms, oats, chocolate. An elemental diet with a formula of nutrients (E028, produced by Hospital Supplies, Liverpool) was used in trials. (The Lancet, 6.11.1993)

Notes. Crohn’s Disease is associated with Erythema nodosum, more frequently recognised in childhood. A frequent cause is cow’s milk intolerance. Smoking adds to the risk of Crohn’s disease.

In susceptible people, the food additives titanium dioxide and aluminosilicates may evoke a latent inflammatory response resulting in Crohn’s disease, ulcerative colitis or bowel cancer. These chemicals may be found in the intestinal lymphoid aggregations in gut mucosa. (Jonathan Powell, Gastro-intestinal Laboratory, St Thomas’s Hospital, London) (Titanium dioxide rarely occurs naturally but is added to confectionery, drinking water and anti-caking agents.) ... crohn’s disease

An inflammation of the skin, usually causing itching and sometimes scaling or blisters. There are several different types of eczema; some forms are known as dermatitis.

Atopic eczema is a chronic, superficial inflammation that occurs in people with an inherited tendency towards allergy. The condition is common in babies. An intensely itchy rash occurs, usually on the face, in the elbow creases, and behind the knees. The skin often scales, and small red pimples may appear. For mild cases, emollients help keep the skin soft. In severe cases, corticosteroid ointments may be used. Antihistamine drugs may reduce itching. Excluding certain foods from the diet may be helpful. Atopic eczema often clears up on its own as a child grows older.

Nummular eczema usually occurs in adults. The cause is unknown. It produces circular, itchy, scaling patches anywhere on the skin, similar to those of tinea (ringworm). Topical corticosteroids may reduce the inflammation, but the disorder is often persistent.

Hand eczema is usually caused by irritant substances such as detergents, but may occur for no apparent reason. Itchy blisters develop, usually on the palms, and the skin may become scaly and cracked. Hand eczema usually improves if emollients are used and cotton gloves with rubber gloves over them are wornwhen coming into contact with irritants. If the eczema is severe, corticosteroids may be prescribed.

Stasis eczema occurs in people with varicose veins. The skin on the legs may become irritated, inflamed, and discoloured. The most important factor is swelling of the legs, which may be controlled with compression bandages or stockings. Ointments containing corticosteroids may give temporary relief.... eczema

Abortion is de?ned as the expulsion of a FETUS before it is normally viable, usually before 24 weeks of pregnancy. (There are exceptional cases nowadays in which fetuses as young as 22 weeks’ gestation have survived.) (See also PREGNANCY AND LABOUR.)

Spontaneous abortion Often called miscarriage, this may occur at any time before 28 weeks; 85 per cent occur in the ?rst 12 weeks of pregnancy. Of all diagnosed pregnancies, 25 per cent end in spontaneous abortion.

Spontaneous abortions occurring in early pregnancy are almost always associated with chromosomal abnormalities of the fetus. Other causes are uterine shape, maternal disorders such as DIABETES MELLITUS, diseases of the thyroid gland (see under ENDOCRINE GLANDS), and problems with the immune system (see IMMUNITY). Recurrent spontaneous abortion (that is, three or more) seems to be a particular problem in women who have an abnormal response of their immune system to pregnancy. Other factors include being older, having had a lot of babies previously, cigarette smoking and spontaneous (but not therapeutic) abortions in the past.

Early ULTRASOUND scans have altered the management of spontaneous abortions. These make it possible to distinguish between threatened abortion, where a woman has had some vaginal bleeding but the fetus is alive; inevitable abortion, where the neck of the uterus has started to open up; incomplete abortion, where part of the fetus or placenta is lost but some remains inside the uterus; and complete abortion. There is no evidence that bed rest is e?ective in stopping a threatened abortion becoming inevitable.

Inevitable or incomplete abortion will usually require a gynaecologist to empty (evacuate) the uterus. (Complete miscarriage requires no treatment.) Evacuation of the uterus is carried out using local or general anaesthetic, usually gentle dilatation of the neck of the uterus (cervix), and curetting-out the remaining products of the pregnancy.

A few late abortions are associated with the cervix opening too early, abnormal structural abnormalities of the uterus, and possibly infection in the mother.

Drugs are often used to suppress uterine contractions, but evidence-based studies show that these do not generally improve fetal salvage. In proven cases of cervical incompetence, the cervix can be closed with a suture which is removed at 37 weeks’ gestation. The evidence for the value of this procedure is uncertain.

Therapeutic abortion In the UK, before an abortion procedure is legally permitted, two doctors must agree and sign a form de?ned under the 1967 Abortion Act that the continuation of the pregnancy would involve risk – greater than if the pregnancy were terminated – of injury to the physical and/or mental health of the mother or any existing child(ren).

Legislation in 1990 modi?ed the Act, which had previously stated that, at the time of the abortion, the pregnancy should not have exceeded the 24th week. Now, an abortion may legally be performed if continuing the pregnancy would risk the woman’s life, or the mental health of the woman or her existing child(ren) is at risk, or if there is a substantial risk of serious handicap to the baby. In 95 per cent of therapeutic terminations in the UK the reason is ‘risk of injury to the physical or mental health of the woman’.

There is no time limit on therapeutic abortion where the termination is done to save the mother’s life, there is substantial risk of serious fetal handicap, or of grave permanent injury to the health of the mother.

About 190,000 terminations are carried out in the UK each year and only 1–1.5 per cent are over 20 weeks’ gestation, with the vast majority of these late abortions being for severe, late-diagnosed, fetal abnormality.

The maternal mortality from therapeutic abortion is less than 1 per 100,000 women and, provided that the procedure is performed skilfully by experienced doctors before 12 weeks of pregnancy, it is very safe. There is no evidence that therapeutic abortion is associated with any reduction in future fertility, increased rates of spontaneous abortion or preterm birth in subsequent pregnancies.

Methods of abortion All abortions must be carried out in premises licensed for doing so or in NHS hospitals. The method used is either surgical or medical, with the latter being used more and the former less as time goes on. Proper consent must be obtained, signed for and witnessed. Women under 16 years of age can consent to termination provided that the doctors obtaining the consent are sure she clearly understands the procedure and its implications. Parental consent in the under-16s is not legally required, but counselling doctors have a duty to record that they have advised young people to inform their parents. However, many youngsters do not do so. The woman’s partner has no legal say in the decision to terminate her pregnancy.

MEDICAL METHODS A combination of two drugs, mifepristone and a prostaglandin (or a prostaglandin-like drug, misoprostol – see PROSTAGLANDINS), may be used to terminate a pregnancy up to 63 days’ gestation. A similar regime can be used between nine and 12 weeks but at this gestation there is a 5 per cent risk of post-treatment HAEMORRHAGE.

An ultrasound scan is ?rst done to con?rm pregnancy and gestation. The sac containing the developing placenta and fetus must be in the uterus; the woman must be under 35 years of age if she is a moderate smoker, but can be over 35 if she is a non-smoker. Reasons for not using this method include women with diseases of the ADRENAL GLANDS, on long-term CORTICOSTEROIDS, and those who have a haemorrhagic disorder or who are on ANTICOAGULANTS. The drugs cannot be used in women with severe liver or kidney disease, and caution is required in those with CHRONIC OBSTRUCTIVE PULMONARY DISEASE (COPD), disease of the cardiovascular system, or prosthetic heart valves (see PROSTHESIS), as well as with those who have had a CAESAREAN SECTION or an ECTOPIC PREGNANCY in the past or who are being treated for HYPERTENSION.

Some clinics use this drug combination for pregnancies older than 12 weeks. In pregnancies approaching viability (20 weeks), pretreatment fetocide (killing of the fetus) with intrauterine drug therapy may be required.

SURGICAL METHODS Vacuum curettage is a method used up to 14–15 weeks. Some very experienced gynaecologists will perform abortions surgically by dilating the cervix and evacuating the uterine contents up to 22 weeks’ gestation. The greater the size of the pregnancy, the higher the risk of haemorrhage and perforation of the uterus. In the UK, illegal abortion is rare but in other countries this is not the case. Where illegal abortions are done, the risks of infection and perforation are high and death a de?nite risk. Legal abortions are generally safe. In the USA, partial-birth abortions are spoken of but, in fact, there is no such procedure recorded in the UK medical journals.... abortion

Alopecia areata is a common form of reversible hair loss which may be patchy, total on the scalp, eyebrows or eyelashes, or universal on the body. The onset is sudden at any age and the affected scalp-skin looks normal. The hair follicles remain intact but ‘switched o?’ and usually hair growth recovers spontaneously. No consistently e?ective treatment is available but injections of CORTICOSTEROIDS, given with a spray gun into the scalp, may be useful. The regrown hair may be white at ?rst but pigmentation recovers later.... alopecia areata

The cause of Addison’s disease (also called chronic adrenal insu?ciency and hypocortisolism) is a de?ciency of the adrenocortical hormones CORTISOL, ALDOSTERONE and androgens (see ANDROGEN) due to destruction of the adrenal cortex (see ADRENAL GLANDS). It occurs in about 1 in 25,000 of the population. In the past, destruction of the adrenal cortex was due to TUBERCULOSIS (TB), but nowadays fewer than 20 per cent of patients have TB while 70 per cent suffer from autoimmune damage. Rare causes of Addison’s disease include metastases (see METASTASIS) from CARCINOMA, usually of the bronchus; granulomata (see GRANULOMA); and HAEMOCHROMATOSIS. It can also occur as a result of surgery for cancer of the PITUITARY GLAND destroying the cells which produce ACTH (ADRENOCORTICOTROPHIC HORMONE)

– the hormone which provokes the adrenal cortex into action.

Symptoms The clinical symptoms appear slowly and depend upon the severity of the underlying disease process. The patient usually complains of appetite and weight loss, nausea, weakness and fatigue. The skin becomes pigmented due to the increased production of ACTH. Faintness, especially on standing, is due to postural HYPOTENSION secondary to aldosterone de?ciency. Women lose their axillary hair and both sexes are liable to develop mental symptoms such as DEPRESSION. Acute episodes – Addisonian crises – may occur, brought on by infection, injury or other stressful events; they are caused by a fall in aldosterone levels, leading to abnormal loss of sodium and water via the kidneys, dehydration, low blood pressure and confusion. Patients may develop increased tanning of the skin from extra pigmentation, with black or blue discoloration of the skin, lips, mouth, rectum and vagina occurring. ANOREXIA, nausea and vomiting are common and the sufferer may feel cold.

Diagnosis This depends on demonstrating impaired serum levels of cortisol and inability of these levels to rise after an injection of ACTH.

Treatment consists in replacement of the de?cient hormones. HYDROCORTISONE tablets are commonly used; some patients also require the salt-retaining hormone, ?udrocortisone. Treatment enables them to lead a completely normal life and to enjoy a normal life expectancy. Before surgery, or if the patient is pregnant and unable to take tablets, injectable hydrocortisone may be needed. Rarely, treated patients may have a crisis, perhaps because they have not been taking their medication or have been vomiting it. Emergency resuscitation is needed with ?uids, salt and sugar. Because of this, all patients should carry a card detailing their condition and necessary management. Treatment of any complicating infections such as tuberculosis is essential. Sometimes DIABETES MELLITUS coexists with Addison’s disease and must be treated.

Secondary adrenal insu?ciency may occur in panhypopituitarism (see PITUITARY GLAND), in patients treated with CORTICOSTEROIDS or after such patients have stopped treatment.... addison’s disease

Also known as suprarenal glands, these are two small triangular ENDOCRINE GLANDS situated one upon the upper end of each kidney. (See diagram of ABDOMEN.)

Structure Each suprarenal gland has an enveloping layer of ?brous tissue. Within this, the gland shows two distinct parts: an outer, ?rm, deep-yellow cortical (see CORTEX) layer, and a central, soft, dark-brown medullary (see MEDULLA) portion. The cortical part consists of columns of cells running from the surface inwards, whilst in the medullary portion the cells are arranged irregularly and separated from one another by large capillary blood vessels.

Functions Removal of the suprarenal glands in animals is speedily followed by great muscular prostration and death within a few days. In human beings, disease of the suprarenal glands usually causes ADDISON’S DISEASE, in which the chief symptoms are increasing weakness and bronzing of the skin. The medulla of the glands produces a substance – ADRENALINE – the effects of which closely resemble those brought about by activity of the SYMPATHETIC NERVOUS SYSTEM: dilated pupils, hair standing on end, quickening and strengthening of the heartbeat, immobilisation of the gut, increased output of sugar from the liver into the bloodstream. Several hormones (called CORTICOSTEROIDS) are produced in the cortex of the gland and play a vital role in the metabolism of the body. Some (such as aldosterone) control the electrolyte balance of the body and help to maintain the blood pressure and blood volume. Others are concerned in carbohydrate metabolism, whilst others again are concerned with sex physiology. HYDROCORTISONE is the most important hormone of the adrenal cortex, controlling as it does the body’s use of carbohydrates, fats and proteins. It also helps to suppress in?ammatory reactions and has an in?uence on the immune system.... adrenal glands

A drug that inhibits the synthesis of adrenal CORTICOSTEROIDS. It is proving to be of value in the treatment of cancer of the breast in post-menopausal women.... aminoglutethimide

One of the CORTICOSTEROIDS used as an aerosol inhalant. It must be used regularly for its best e?ect. Unlike systemic corticosteroids, inhaled forms are much less likely to suppress adrenal-gland activity and have fewer side-effects.... beclomethasone dipropionate

One of the CORTICOSTEROIDS which has an action comparable to that of PREDNISOLONE, but in much lower dosage. In the form of betamethasone valerate it is used as an application to the skin as an ointment or cream.... betamethasone

Bone is not an inert sca?olding for the human body. It is a living, dynamic organ, being continuously remodelled in response to external mechanical and chemical in?uences and acting as a large reservoir for calcium and phosphate. It is as susceptible to disease as any other organ, but responds in a way rather di?erent from the rest of the body.

Bone fractures These occur when there is a break in the continuity of the bone. This happens either as a result of violence or because the bone is unhealthy and unable to withstand normal stresses.

SIMPLE FRACTURES Fractures where the skin remains intact or merely grazed. COMPOUND FRACTURES have at least one wound which is in communication with the fracture, meaning that bacteria can enter the fracture site and cause infection. A compound fracture is also more serious than a simple fracture because there is greater potential for blood loss. Compound fractures usually need hospital admission, antibiotics and careful reduction of the fracture. Debridement (cleaning and excising dead tissue) in a sterile theatre may also be necessary.

The type of fracture depends on the force which has caused it. Direct violence occurs when an object hits the bone, often causing a transverse break – which means the break runs horizontally across the bone. Indirect violence occurs when a twisting injury to the ankle, for example, breaks the calf-bone (the tibia) higher up. The break may be more oblique. A fall on the outstretched hand may cause a break at the wrist, in the humerus or at the collar-bone depending on the force of impact and age of the person. FATIGUE FRACTURES These occur after the bone has been under recurrent stress. A typical example is the march fracture of the second toe, from which army recruits suffer after long marches. PATHOLOGICAL FRACTURES These occur in bone which is already diseased – for example, by osteoporosis (see below) in post-menopausal women. Such fractures are typically crush fractures of the vertebrae, fractures of the neck of the femur, and COLLES’ FRACTURE (of the wrist). Pathological fractures also occur in bone which has secondary-tumour deposits. GREENSTICK FRACTURES These occur in young children whose bones are soft and bend, rather than break, in response to stress. The bone tends to buckle on the side opposite to the force. Greenstick fractures heal quickly but still need any deformity corrected and plaster of Paris to maintain the correction. COMPLICATED FRACTURES These involve damage to important soft tissue such as nerves, blood vessels or internal organs. In these cases the soft-tissue damage needs as much attention as the fracture site. COMMINUTED FRACTURES A fracture with more than two fragments. It usually means that the injury was more violent and that there is more risk of damage to vessels and nerves. These fractures are unstable and take longer to unite. Rehabilitation tends to be protracted. DEPRESSED FRACTURES Most commonly found in skull fractures. A fragment of bone is forced inwards so that it lies lower than the level of the bone surrounding it. It may damage the brain beneath it.

HAIR-LINE FRACTURES These occur when the bone is broken but the force has not been severe enough to cause visible displacement. These fractures may be easily missed. Symptoms and signs The fracture site is usually painful, swollen and deformed. There is asymmetry of contour between limbs. The limb is held uselessly. If the fracture is in the upper

limb, the arm is usually supported by the patient; if it is in the lower limb then the patient is not able to bear weight on it. The limb may appear short because of muscle spasm.

Examination may reveal crepitus – a bony grating – at the fracture site. The diagnosis is con?rmed by radiography.

Treatment Healing of fractures (union) begins with the bruise around the fracture being resorbed and new bone-producing cells and blood vessels migrating into the area. Within a couple of days they form a bridge of primitive bone across the fracture. This is called callus.

The callus is replaced by woven bone which gradually matures as the new bone remodels itself. Treatment of fractures is designed to ensure that this process occurs with minimal residual deformity to the bone involved.

Treatment is initially to relieve pain and may involve temporary splinting of the fracture site. Reducing the fracture means restoring the bones to their normal position; this is particularly important at the site of joints where any small displacement may limit movement considerably.

with plaster of Paris. If closed traction does not work, then open reduction of the fracture may

be needed. This may involve ?xing the fracture with internal-?xation methods, using metal plates, wires or screws to hold the fracture site in a rigid position with the two ends closely opposed. This allows early mobilisation after fractures and speeds return to normal use.

External ?xators are usually metal devices applied to the outside of the limb to support the fracture site. They are useful in compound fractures where internal ?xators are at risk of becoming infected.

Consolidation of a fracture means that repair is complete. The time taken for this depends on the age of the patient, the bone and the type of fracture. A wrist fracture may take six weeks, a femoral fracture three to six months in an adult.

Complications of fractures are fairly common. In non-union, the fracture does not unite

– usually because there has been too much mobility around the fracture site. Treatment may involve internal ?xation (see above). Malunion means that the bone has healed with a persistent deformity and the adjacent joint may then develop early osteoarthritis.

Myositis ossi?cans may occur at the elbow after a fracture. A big mass of calci?ed material develops around the fracture site which restricts elbow movements. Late surgical removal (after 6–12 months) is recommended.

Fractured neck of FEMUR typically affects elderly women after a trivial injury. The bone is usually osteoporotic. The leg appears short and is rotated outwards. Usually the patient is unable to put any weight on the affected leg and is in extreme pain. The fractures are classi?ed according to where they occur:

subcapital where the neck joins the head of the femur.

intertrochanteric through the trochanter.

subtrochanteric transversely through the upper end of the femur (rare). Most of these fractures of the neck of femur

need ?xing by metal plates or hip replacements, as immobility in this age group has a mortality of nearly 100 per cent. Fractures of the femur shaft are usually the result of severe trauma such as a road accident. Treatment may be conservative or operative.

In fractures of the SPINAL COLUMN, mere damage to the bone – as in the case of the so-called compression fracture, in which there is no damage to the spinal cord – is not necessarily serious. If, however, the spinal cord is damaged, as in the so-called fracture dislocation, the accident may be a very serious one, the usual result being paralysis of the parts of the body below the level of the injury. Therefore the higher up the spine is fractured, the more serious the consequences. The injured person should not be moved until skilled assistance is at hand; or, if he or she must be removed, this should be done on a rigid shutter or door, not on a canvas stretcher or rug, and there should be no lifting which necessitates bending of the back. In such an injury an operation designed to remove a displaced piece of bone and free the spinal cord from pressure is often necessary and successful in relieving the paralysis. DISLOCATIONS or SUBLUXATION of the spine are not uncommon in certain sports, particularly rugby. Anyone who has had such an injury in the cervical spine (i.e. in the neck) should be strongly advised not to return to any form of body-contact or vehicular sport.

Simple ?ssured fractures and depressed fractures of the skull often follow blows or falls on the head, and may not be serious, though there is always a risk of damage which is potentially serious to the brain at the same time.

Compound fractures may result in infection within the skull, and if the skull is extensively broken and depressed, surgery is usually required to check any intercranial bleeding or to relieve pressure on the brain.

The lower jaw is often fractured by a blow on the face. There is generally bleeding from the mouth, the gum being torn. Also there are pain and grating sensations on chewing, and unevenness in the line of the teeth. The treatment is simple, the line of teeth in the upper jaw forming a splint against which the lower jaw is bound, with the mouth closed.

Congenital diseases These are rare but may produce certain types of dwar?sm or a susceptibility to fractures (osteogenesis imperfecta).

Infection of bone (osteomyelitis) may occur after an open fracture, or in newborn babies with SEPTICAEMIA. Once established it is very di?cult to eradicate. The bacteria appear capable of lying dormant in the bone and are not easily destroyed with antibiotics so that prolonged treatment is required, as might be surgical drainage, exploration or removal of dead bone. The infection may become chronic or recur.

Osteomalacia (rickets) is the loss of mineralisation of the bone rather than simple loss of bone mass. It is caused by vitamin D de?ciency and is probably the most important bone disease in the developing world. In sunlight the skin can synthesise vitamin D (see APPENDIX 5: VITAMINS), but normally rickets is caused by a poor diet, or by a failure to absorb food normally (malabsorbtion). In rare cases vitamin D cannot be converted to its active state due to the congenital lack of the speci?c enzymes and the rickets will fail to respond to treatment with vitamin D. Malfunction of the parathyroid gland or of the kidneys can disturb the dynamic equilibrium of calcium and phosphate in the body and severely deplete the bone of its stores of both calcium and phosphate.

Osteoporosis A metabolic bone disease resulting from low bone mass (osteopenia) due to excessive bone resorption. Su?erers are prone to bone fractures from relatively minor trauma. With bone densitometry it is now possible to determine individuals’ risk of osteoporosis and monitor their response to treatment.

By the age of 90 one in two women and one in six men are likely to sustain an osteoporosis-related fracture. The incidence of fractures is increasing more than would be expected from the ageing of the population, which may re?ect changing patterns of exercise or diet.

Osteoporosis may be classi?ed as primary or secondary. Primary consists of type 1 osteoporosis, due to accelerated trabecular bone loss, probably as a result of OESTROGENS de?ciency. This typically leads to crush fractures of vertebral bodies and fractures of the distal forearm in women in their 60s and 70s. Type 2 osteoporosis, by contrast, results from the slower age-related cortical and travecular bone loss that occurs in both sexes. It typically leads to fractures of the proximal femur in elderly people.

Secondary osteoporosis accounts for about 20 per cent of cases in women and 40 per cent of cases in men. Subgroups include endocrine (thyrotoxicosis – see under THYROID GLAND, DISEASES OF, primary HYPERPARATHYROIDISM, CUSHING’S SYNDROME and HYPOGONADISM); gastrointestinal (malabsorption syndrome, e.g. COELIAC DISEASE, or liver disease, e.g. primary biliary CIRRHOSIS); rheumatological (RHEUMATOID ARTHRITIS or ANKYLOSING SPONDYLITIS); malignancy (multiple MYELOMA or metastatic CARCINOMA); and drugs (CORTICOSTEROIDS, HEPARIN). Additional risk factors for osteoporosis include smoking, high alcohol intake, physical inactivity, thin body-type and heredity.

Individuals at risk of osteopenia, or with an osteoporosis-related fracture, need investigation with spinal radiography and bone densitometry. A small fall in bone density results in a large increase in the risk of fracture, which has important implications for preventing and treating osteoporosis.

Treatment Antiresorptive drugs: hormone replacement therapy – also valuable in treating menopausal symptoms; treatment for at least ?ve years is necessary, and prolonged use may increase risk of breast cancer. Cyclical oral administration of disodium etidronate – one of the bisphosphonate group of drugs – with calcium carbonate is also used (poor absorption means the etidronate must be taken on an empty stomach). Calcitonin – currently available as a subcutaneous injection; a nasal preparation with better tolerance is being developed. Calcium (1,000 mg daily) seems useful in older patients, although probably ine?ective in perimenopausal women, and it is a safe preparation. Vitamin D and calcium – recent evidence suggests value for elderly patients. Anabolic steroids, though androgenic side-effects (masculinisation) make these unacceptable for most women.

With established osteoporosis, the aim of treatment is to relieve pain (with analgesics and physical measures, e.g. lumbar support) and reduce the risk of further fractures: improvement of bone mass, the prevention of falls, and general physiotherapy, encouraging a healthier lifestyle with more daily exercise.

Further information is available from the National Osteoporosis Society.

Paget’s disease (see also separate entry) is a common disease of bone in the elderly, caused by overactivity of the osteoclasts (cells concerned with removal of old bone, before new bone is laid down by osteoblasts). The bone affected thickens and bows and may become painful. Treatment with calcitonin and bisphosphonates may slow down the osteoclasts, and so hinder the course of the disease, but there is no cure.

If bone loses its blood supply (avascular necrosis) it eventually fractures or collapses. If the blood supply does not return, bone’s normal capacity for healing is severely impaired.

For the following diseases see separate articles: RICKETS; ACROMEGALY; OSTEOMALACIA; OSTEOGENESIS IMPERFECTA.

Tumours of bone These can be benign (non-cancerous) or malignant (cancerous). Primary bone tumours are rare, but secondaries from carcinoma of the breast, prostate and kidneys are relatively common. They may form cavities in a bone, weakening it until it breaks under normal load (a pathological fracture). The bone eroded away by the tumour may also cause problems by causing high levels of calcium in the plasma.

EWING’S TUMOUR is a malignant growth affecting long bones, particularly the tibia (calfbone). The presenting symptoms are a throbbing pain in the limb and a high temperature. Treatment is combined surgery, radiotherapy and chemotherapy.

MYELOMA is a generalised malignant disease of blood cells which produces tumours in bones which have red bone marrow, such as the skull and trunk bones. These tumours can cause pathological fractures.

OSTEOID OSTEOMA is a harmless small growth which can occur in any bone. Its pain is typically removed by aspirin.

OSTEOSARCOMA is a malignant tumour of bone with a peak incidence between the ages of ten and 20. It typically involves the knees, causing a warm tender swelling. Removal of the growth with bone conservation techniques can often replace amputation as the de?nitive treatment. Chemotherapy can improve long-term survival.... bone, disorders of

A drug used in the prophylactic (preventive) treatment of allergic disorders (see ALLERGY), particularly ASTHMA, conjunctivitis (see EYE, DISORDERS OF), nasal allergies, and food allergies – especially in children. Although inappropriate for the treatment of acute attacks of asthma, regular inhalations of the drug can reduce its incidence, and allow the dose of BRONCHODILATORS and oral CORTICOSTEROIDS to be cut.... disodium cromoglycate

A term generally used to describe an adverse reaction by the body to any substance ingested by the affected individual. Strictly, allergy refers to any reactions incited by an abnormal immunological response to an ALLERGEN, and susceptibility has a strong genetic component. Most allergic disorders are linked to ATOPY, the predisposition to generate the allergic antibody immunoglobulin E (IgE) to common environmental agents (see ANTIBODIES; IMMUNOGLOBULINS). Because IgE is able to sensitise MAST CELLS (which play a part in in?ammatory and allergic reactions) anywhere in the body, atopic individuals often have disease in more than one organ. Since the allergic disorder HAY FEVER was ?rst described in 1819, allergy has moved from being a rare condition to one a?icting almost one in two people in the developed world, with substances such as grass and tree pollen, house-dust mite, bee and wasp venom, egg and milk proteins, peanuts, antibiotics, and other airborne environmental pollutants among the triggering factors. Increasing prevalence of allergic reactions has been noticeable during the past two decades, especially in young people with western lifestyles.

A severe or life-threatening reaction is often termed ANAPHYLAXIS. Many immune mechanisms also contribute to allergic disorders; however, adverse reactions to drugs, diagnostic materials and other substances often do not involve recognised immunological mechanisms and the term ‘hypersensitivity’ is preferable. (See also IMMUNITY.)

Adverse reactions may manifest themselves as URTICARIA, wheezing or di?culty in breathing owing to spasm of the BRONCHIOLES, swollen joints, nausea, vomiting and headaches. Severe allergic reactions may cause a person to go into SHOCK. Although symptoms of an allergic reaction can usually be controlled, treatment of the underlying conditon is more problematic: hence, the best current approach is for susceptible individuals to ?nd out what it is they are allergic to and avoid those agents. For some people, such as those sensitive to insect venom, IMMUNOTHERAPY or desensitisation is often e?ective. If avoidance measures are unsuccessful and desensitisation ine?ective, the in?ammatory reactions can be controlled with CORTICOSTEROIDS, while the troublesome symptoms can be treated with ANTIHISTAMINE DRUGS and SYMPATHOMIMETICS. All three types of drugs may be needed to treat severe allergic reactions.

One interesting hypothesis is that reduced exposure to infective agents, such as bacteria, in infancy may provoke the development of allergy in later life.

Predicted developments in tackling allergic disorders, according to Professor Stephen Holgate writing in the British Medical Journal (22 January 2000) include:

Identi?cation of the principal environmental factors underlying the increase in incidence, to enable preventive measures to be planned.

Safe and e?ective immunotherapy to prevent and reverse allergic disease.

Treatments that target the protein reactions activated by antigens.

Identi?cation of how IgE is produced in the body, and thus of possible ways to inhibit this process.

Identi?cation of genes affecting people’s susceptibility to allergic disease.... allergy

In?ammation of the alveoli (see ALVEOLUS) of the lungs caused by an allergic reaction. When the in?ammation is caused by infection it is called PNEUMONIA, and when by a chemical or physical agent it is called pneumonitis. It may be associated with systemic sclerosis or RHEUMATOID ARTHRITIS.

Extrinsic allergic alveolitis is the condition induced by the lungs becoming allergic (see ALLERGY) to various factors or substances. It includes BAGASSOSIS, FARMER’S LUNG and BUDGERIGAR-FANCIER’S LUNG, and is characterised by the onset of shortness of breath, tightness of the chest, cough and fever. The onset may be sudden or gradual. Treatment consists of removal of the affected individual from the o?ending material to which he or she has become allergic. CORTICOSTEROIDS give temporary relief.

Fibrosing alveolitis In this disease there is di?use FIBROSIS of the walls of the alveoli of the lungs. This causes loss of lung volume with both forced expiratory volume and vital capacity affected, but the ratio between them remaining normal. The patient complains of cough and progressive DYSPNOEA. Typically the patient will be cyanosed (blue – see CYANOSIS), clubbed (see CLUBBING), and have crackles in the mid- and lower-lung ?elds. Blood gases will reveal HYPOXIA and, in early disease, hypocapnia (de?ciency of carbon dioxide in the blood due to hyperventilation). There is an association with RHEUMATOID ARTHRITIS (about one-eighth of cases), systemic lupus erythematosus (see under LUPUS), and systemic SCLEROSIS. Certain drugs – for example, bleomycin, busulphan and hexamethonium – may also cause this condition, as may high concentrations of oxygen, and inhalation of CADMIUM fumes.... alveolitis

Organs whose function it is to secrete into the blood or lymph, substances known as HORMONES. These play an important part in general changes to or the activities of other organs at a distance. Various diseases arise as the result of defects or excess in the internal secretions of the di?erent glands. The chief endocrine glands are:

Adrenal glands These two glands, also known as suprarenal glands, lie immediately above the kidneys. The central or medullary portion of the glands forms the secretions known as ADRENALINE (or epinephrine) and NORADRENALINE. Adrenaline acts upon structures innervated by sympathetic nerves. Brie?y, the blood vessels of the skin and of the abdominal viscera (except the intestines) are constricted, and at the same time the arteries of the muscles and the coronary arteries are dilated; systolic blood pressure rises; blood sugar increases; the metabolic rate rises; muscle fatigue is diminished. The super?cial or cortical part of the glands produces steroid-based substances such as aldosterone, cortisone, hydrocortisone, and deoxycortone acetate, for the maintenance of life. It is the absence of these substances, due to atrophy or destruction of the suprarenal cortex, that is responsible for the condition known as ADDISON’S DISEASE. (See CORTICOSTEROIDS.)

Ovaries and testicles The ovary (see OVARIES) secretes at least two hormones – known, respectively, as oestradiol (follicular hormone) and progesterone (corpus luteum hormone). Oestradiol develops (under the stimulus of the anterior pituitary lobe – see PITUITARY GLAND below, and under separate entry) each time an ovum in the ovary becomes mature, and causes extensive proliferation of the ENDOMETRIUM lining the UTERUS, a stage ending with shedding of the ovum about 14 days before the onset of MENSTRUATION. The corpus luteum, which then forms, secretes both progesterone and oestradiol. Progesterone brings about great activity of the glands in the endometrium. The uterus is now ready to receive the ovum if it is fertilised. If fertilisation does not occur, the corpus luteum degenerates, the hormones cease acting, and menstruation takes place.

The hormone secreted by the testicles (see TESTICLE) is known as TESTOSTERONE. It is responsible for the growth of the male secondary sex characteristics.

Pancreas This gland is situated in the upper part of the abdomen and, in addition to the digestive enzymes, it produces INSULIN within specialised cells (islets of Langerhans). This controls carbohydrate metabolism; faulty or absent insulin production causes DIABETES MELLITUS.

Parathyroid glands These are four minute glands lying at the side of, or behind, the thyroid (see below). They have a certain e?ect in controlling the absorption of calcium salts by the bones and other tissues. When their secretion is defective, TETANY occurs.

Pituitary gland This gland is attached to the base of the brain and rests in a hollow on the base of the skull. It is the most important of all endocrine glands and consists of two embryologically and functionally distinct lobes.

The function of the anterior lobe depends on the secretion by the HYPOTHALAMUS of certain ‘neuro-hormones’ which control the secretion of the pituitary trophic hormones. The hypothalamic centres involved in the control of speci?c pituitary hormones appear to be anatomically separate. Through the pituitary trophic hormones the activity of the thyroid, adrenal cortex and the sex glands is controlled. The anterior pituitary and the target glands are linked through a feedback control cycle. The liberation of trophic hormones is inhibited by a rising concentration of the circulating hormone of the target gland, and stimulated by a fall in its concentration. Six trophic (polypeptide) hormones are formed by the anterior pituitary. Growth hormone (GH) and prolactin are simple proteins formed in the acidophil cells. Follicle-stimulating hormone (FSH), luteinising hormone (LH) and thyroid-stimulating hormone (TSH) are glycoproteins formed in the basophil cells. Adrenocorticotrophic hormone (ACTH), although a polypeptide, is derived from basophil cells.

The posterior pituitary lobe, or neurohypophysis, is closely connected with the hypothalamus by the hypothalamic-hypophyseal tracts. It is concerned with the production or storage of OXYTOCIN and vasopressin (the antidiuretic hormone).

PITUITARY HORMONES Growth hormone, gonadotrophic hormone, adrenocorticotrophic hormone and thyrotrophic hormones can be assayed in blood or urine by radio-immunoassay techniques. Growth hormone extracted from human pituitary glands obtained at autopsy was available for clinical use until 1985, when it was withdrawn as it is believed to carry the virus responsible for CREUTZFELDT-JAKOB DISEASE (COD). However, growth hormone produced by DNA recombinant techniques is now available as somatropin. Synthetic growth hormone is used to treat de?ciency of the natural hormone in children and adults, TURNER’S SYNDROME and chronic renal insu?ciency in children.

Human pituitary gonadotrophins are readily obtained from post-menopausal urine. Commercial extracts from this source are available and are e?ective for treatment of infertility due to gonadotrophin insu?ciency.

The adrenocorticotrophic hormone is extracted from animal pituitary glands and has been available therapeutically for many years. It is used as a test of adrenal function, and, under certain circumstances, in conditions for which corticosteroid therapy is indicated (see CORTICOSTEROIDS). The pharmacologically active polypeptide of ACTH has been synthesised and is called tetracosactrin. Thyrotrophic hormone is also available but it has no therapeutic application.

HYPOTHALAMIC RELEASING HORMONES which affect the release of each of the six anterior pituitary hormones have been identi?ed. Their blood levels are only one-thousandth of those of the pituitary trophic hormones. The release of thyrotrophin, adrenocorticotrophin, growth hormone, follicle-stimulating hormone and luteinising hormone is stimulated, while release of prolactin is inhibited. The structure of the releasing hormones for TSH, FSH-LH, GH and, most recently, ACTH is known and they have all been synthesised. Thyrotrophin-releasing hormone (TRH) is used as a diagnostic test of thyroid function but it has no therapeutic application. FSH-LH-releasing hormone provides a useful diagnostic test of gonadotrophin reserve in patients with pituitary disease, and is now used in the treatment of infertility and AMENORRHOEA in patients with functional hypothalamic disturbance. As this is the most common variety of secondary amenorrhoea, the potential use is great. Most cases of congenital de?ciency of GH, FSH, LH and ACTH are due to defects in the hypothalamic production of releasing hormone and are not a primary pituitary defect, so that the therapeutic implication of this synthesised group of releasing hormones is considerable.

GALACTORRHOEA is frequently due to a microadenoma (see ADENOMA) of the pituitary. DOPAMINE is the prolactin-release inhibiting hormone. Its duration of action is short so its therapeutic value is limited. However, BROMOCRIPTINE is a dopamine agonist with a more prolonged action and is e?ective treatment for galactorrhoea.

Thyroid gland The functions of the thyroid gland are controlled by the pituitary gland (see above) and the hypothalamus, situated in the brain. The thyroid, situated in the front of the neck below the LARYNX, helps to regulate the body’s METABOLISM. It comprises two lobes each side of the TRACHEA joined by an isthmus. Two types of secretory cells in the gland – follicular cells (the majority) and parafollicular cells – secrete, respectively, the iodine-containing hormones THYROXINE (T4) and TRI-IODOTHYRONINE (T3), and the hormone CALCITONIN. T3 and T4 help control metabolism and calcitonin, in conjunction with parathyroid hormone (see above), regulates the body’s calcium balance. De?ciencies in thyroid function produce HYPOTHYROIDISM and, in children, retarded development. Excess thyroid activity causes thyrotoxicosis. (See THYROID GLAND, DISEASES OF.)... endocrine glands

A feeling of well-being. This may occur normally; for instance, when someone has passed an examination. In some neurological or psychiatric conditions, however, patients may have an exaggerated and quite unjusti?ed feeling of euphoria. This is then a symptom of the underlying condition. Euphoria may also be drug-induced – by drugs of addiction or by therapeutic drugs such as CORTICOSTEROIDS.... euphoria

Arcus senilis The white ring or crescent which tends to form at the edge of the cornea with age. It is uncommon in the young, when it may be associated with high levels of blood lipids (see LIPID).

Astigmatism (See ASTIGMATISM.)

Blepharitis A chronic in?ammation of the lid margins. SEBORRHOEA and staphylococcal infection are likely contributors. The eyes are typically intermittently red, sore and gritty over months or years. Treatment is di?cult and may fail. Measures to reduce debris on the lid margins, intermittent courses of topical antibiotics, steroids or systemic antibiotics may help the sufferer.

Blepharospasm Involuntary closure of the eye. This may accompany irritation but may also occur without an apparent cause. It may be severe enough to interfere with vision. Treatment involves removing the source of irritation, if present. Severe and persistent cases may respond to injection of Botulinum toxin into the orbicularis muscle.

Cataract A term used to describe any opacity in the lens of the eye, from the smallest spot to total opaqueness. The prevalence of cataracts is age-related: 65 per cent of individuals in their sixth decade have some degree of lens opacity, while all those over 80 are affected. Cataracts are the most important cause of blindness worldwide. Symptoms will depend on whether one or both eyes are affected, as well as the position and density of the cataract(s). If only one eye is developing a cataract, it may be some time before the person notices it, though reading may be affected. Some people with cataracts become shortsighted, which in older people may paradoxically ‘improve’ their ability to read. Bright light may worsen vision in those with cataracts.

The extent of visual impairment depends on the nature of the cataracts, and the ?rst symptoms noticed by patients include di?culty in recognising faces and in reading, while problems watching television or driving, especially at night, are pointers to the condition. Cataracts are common but are not the only cause of deteriorating vision. Patients with cataracts should be able to point to the position of a light and their pupillary reactions should be normal. If a bright light is shone on the eye, the lens may appear brown or, in advanced cataracts, white (see diagram).

While increasing age is the commonest cause of cataract in the UK, patients with DIABETES MELLITUS, UVEITIS and a history of injury to the eye can also develop the disorder. Prolonged STEROID treatment can result in cataracts. Children may develop cataracts, and in them the condition is much more serious as vision may be irreversibly impaired because development of the brain’s ability to interpret visual signals is hindered. This may happen even if the cataracts are removed, so early referral for treatment is essential. One of the physical signs which doctors look for when they suspect cataract in adults as well as in children is the ‘red re?ex’. This is observable when an ophthalmoscopic examination of the eye is made (see OPHTHALMOSCOPE). Identi?cation of this red re?ex (a re?ection of light from the red surface of the retina –see EYE) is a key diagnostic sign in children, especially young ones.

There is no e?ective medical treatment for established cataracts. Surgery is necessary and the decision when to operate depends mainly on how the cataract(s) affect(s) the patient’s vision. Nowadays, surgery can be done at any time with limited risk. Most patients with a vision of 6/18 – 6/10 is the minimum standard for driving – or worse in both eyes should

E

bene?t from surgery, though elderly people may tolerate visual acuity of 6/18 or worse, so surgery must be tailored to the individual’s needs. Younger people with a cataract will have more demanding visual requirements and so may opt for an ‘earlier’ operation. Most cataract surgery in Britain is now done under local anaesthetic and uses the ‘phaco-emulsi?cation’ method. A small hole is made in the anterior capsule of the lens after which the hard lens nucleus is liqui?ed ultrasonically. A replacement lens is inserted into the empty lens bag (see diagram). Patients usually return to their normal activities within a few days of the operation. A recent development under test in the USA for children requiring cataract operations is an intra-ocular ?exible implant whose magnifying power can be altered as a child develops, thus precluding the need for a series of corrective operations as happens now.

Chalazion A ?rm lump in the eyelid relating to a blocked meibomian gland, felt deep within the lid. Treatment is not always necessary; a proportion spontaneously resolve. There can be associated infection when the lid becomes red and painful requiring antibiotic treatment. If troublesome, the chalazion can be incised under local anaesthetic.

Conjunctivitis In?ammation of the conjunctiva (see EYE) which may affect one or both eyes. Typically the eye is red, itchy, sticky and gritty but is not usually painful. Redness is not always present. Conjunctivitis can occasionally be painful, particularly if there is an associated keratitis (see below) – for example, adenovirus infection, herpetic infection.

The cause can be infective (bacteria, viruses or CHLAMYDIA), chemical (e.g. acids, alkalis) or allergic (e.g. in hay fever). Conjunctivitis may also be caused by contact lenses, and preservatives or even the drugs in eye drops may cause conjunctival in?ammation. Conjunctivitis may addtionally occur in association with other illnesses – for example, upper-respiratory-tract infection, Stevens-Johnson syndrome (see ERYTHEMA – erythema multiforme) or REITER’S SYNDROME. The treatment depends on the cause. In many patients acute conjunctivitis is self-limiting.

Dacryocystitis In?ammation of the lacrimal sac. This may present acutely as a red, painful swelling between the nose and the lower lid. An abscess may form which points through the skin and which may need to be drained by incision. Systemic antibiotics may be necessary. Chronic dacryocystitis may occur with recurrent discharge from the openings of the tear ducts and recurrent swelling of the lacrimal sac. Obstruction of the tear duct is accompanied by watering of the eye. If the symptoms are troublesome, the patient’s tear passageways need to be surgically reconstructed.

Ectropion The lid margin is everted – usually the lower lid. Ectropion is most commonly associated with ageing, when the tissues of the lid become lax. It can also be caused by shortening of the skin of the lids such as happens with scarring or mechanical factors – for example, a tumour pulling the skin of the lower lid downwards. Ectropion tends to cause watering and an unsightly appearance. The treatment is surgical.

Entropion The lid margin is inverted – usually the lower lid. Entropion is most commonly associated with ageing, when the tissues of the lid become lax. It can also be caused by shortening of the inner surfaces of the lids due to scarring – for example, TRACHOMA or chemical burns. The inwardly directed lashes cause irritation and can abrade the cornea. The treatment is surgical.

Episcleritis In?ammation of the EPISCLERA. There is usually no apparent cause. The in?ammation may be di?use or localised and may affect one or both eyes. It sometimes recurs. The affected area is usually red and moderately painful. Episcleritis is generally not thought to be as painful as scleritis and does not lead to the same complications. Treatment is generally directed at improving the patient’s symptoms. The in?ammation may respond to NON-STEROIDAL ANTI-INFLAMMATORY DRUGS (NSAIDS) or topical CORTICOSTEROIDS.

Errors of refraction (Ametropia.) These will occur when the focusing power of the lens and cornea does not match the length of the eye, so that rays of light parallel to the visual axis are not focused at the fovea centralis (see EYE). There are three types of refractive error: HYPERMETROPIA or long-sightedness. The refractive power of the eye is too weak, or the eye is too short so that rays of light are brought to a focus at a point behind the retina. Longsighted people can see well in the distance but generally require glasses with convex lenses for reading. Uncorrected long sight can lead to headaches and intermittent blurring of vision following prolonged close work (i.e. eye strain). As a result of ageing, the eye becomes gradually long-sighted, resulting in many people needing reading glasses in later life: this normal process is known as presbyopia. A particular form of long-sightedness occurs after cataract extraction (see above). MYOPIA(Short sight or near sight.) Rays of light are brought to a focus in front of the retina because the refractive power of the eye is too great or the eye is too short. Short-sighted people can see close to but need spectacles with concave lenses in order to see in the distance. ASTIGMATISMThe refractive power of the eye is not the same in each meridian. Some rays of light may be focused in front of the retina while others are focused on or behind the retina. Astigmatism can accompany hypermetropia or myopia. It may be corrected by cylindrical lenses: these consist of a slice from the side of a cylinder (i.e. curved in one meridian and ?at in the meridian at right-angles to it).

Keratitis In?ammation of the cornea in response to a variety of insults – viral, bacterial, chemical, radiation, or mechanical trauma. Keratitis may be super?cial or involve the deeper layers, the latter being generally more serious. The eye is usually red, painful and photophobic. Treatment is directed at the cause.

Nystagmus Involuntary rhythmic oscillation of one or both eyes. There are several causes including nervous disorders, vestibular disorders, eye disorders and certain drugs including alcohol.

Ophthalmia In?ammation of the eye, especially the conjunctiva (see conjunctivitis, above). Ophthalmia neonatorum is a type of conjunctivitis that occurs in newborn babies. They catch the disease when passing through an infected birth canal during their mother’s labour (see PREGNANCY AND LABOUR). CHLAMYDIA and GONORRHOEA are the two most common infections. Treatment is e?ective with antibiotics: untreated, the infection may cause permanent eye damage.

Pinguecula A benign degenerative change in the connective tissue at the nasal or temporal limbus (see EYE). This is visible as a small, ?attened, yellow-white lump adjacent to the cornea.

Pterygium Overgrowth of the conjunctival tissues at the limbus on to the cornea (see EYE). This usually occurs on the nasal side and is associated with exposure to sunlight. The pterygium is surgically removed for cosmetic reasons or if it is thought to be advancing towards the visual axis.

Ptosis Drooping of the upper lid. May occur because of a defect in the muscles which raise the lid (levator complex), sometimes the result of ageing or trauma. Other causes include HORNER’S SYNDROME, third cranial nerve PALSY, MYASTHENIA GRAVIS, and DYSTROPHIA MYOTONICA. The cause needs to be determined and treated if possible. The treatment for a severely drooping lid is surgical, but other measures can be used to prop up the lid with varying success.

Retina, disorders of The retina can be damaged by disease that affects the retina alone, or by diseases affecting the whole body.

Retinopathy is a term used to denote an abnormality of the retina without specifying a cause. Some retinal disorders are discussed below. DIABETIC RETINOPATHY Retinal disease occurring in patients with DIABETES MELLITUS. It is the commonest cause of blind registration in Great Britain of people between the ages of 20 and 65. Diabetic retinopathy can be divided into several types. The two main causes of blindness are those that follow: ?rst, development of new blood vessels from the retina, with resultant complications and, second, those following ‘water logging’ (oedema) of the macula. Treatment is by maintaining rigid control of blood-sugar levels combined with laser treatment for certain forms of the disease – in particular to get rid of new blood vessels. HYPERTENSIVE RETINOPATHY Retinal disease secondary to the development of high blood pressure. Treatment involves control of the blood pressure (see HYPERTENSION). SICKLE CELL RETINOPATHY People with sickle cell disease (see under ANAEYIA) can develop a number of retinal problems including new blood vessels from the retina. RETINOPATHY OF PREMATURITY (ROP) Previously called retrolental ?broplasia (RLF), this is a disorder affecting low-birth-weight premature babies exposed to oxygen. Essentially, new blood vessels develop which cause extensive traction on the retina with resultant retinal detachment and poor vision. RETINAL ARTERY OCCLUSION; RETINAL VEIN OCCLUSION These result in damage to those areas of retina supplied by the affected blood vessel: the blood vessels become blocked. If the peripheral retina is damaged the patient may be completely symptom-free, although areas of blindness may be detected on examination of ?eld of vision. If the macula is involved, visual loss may be sudden, profound and permanent. There is no e?ective treatment once visual loss has occurred. SENILE MACULAR DEGENERATION (‘Senile’ indicates age of onset and has no bearing on mental state.) This is the leading cause of blindness in the elderly in the western world. The average age of onset is 65 years. Patients initially notice a disturbance of their vision which gradually progresses over months or years. They lose the ability to recognise ?ne detail; for example, they cannot read ?ne print, sew, or recognise people’s faces. They always retain the ability to recognise large objects such as doors and chairs, and are therefore able to get around and about reasonably well. There is no e?ective treatment in the majority of cases. RETINITIS PIGMENTOSAA group of rare, inherited diseases characterised by the development of night blindness and tunnel vision. Symptoms start in childhood and are progressive. Many patients retain good visual acuity, although their peripheral vision is limited. One of the characteristic ?ndings on examination is collections of pigment in the retina which have a characteristic shape and are therefore known as ‘bone spicules’. There is no e?ective treatment. RETINAL DETACHMENTusually occurs due to the development of a hole in the retina. Holes can occur as a result of degeneration of the retina, traction on the retina by the vitreous, or injury. Fluid from the vitreous passes through the hole causing a split within the retina; the inner part of the retina becomes detached from the outer part, the latter remaining in contact with the choroid. Detached retina loses its ability to detect light, with consequent impairment of vision. Retinal detachments are more common in the short-sighted, in the elderly or following cataract extraction. Symptoms include spots before the eyes (?oaters), ?ashing lights and a shadow over the eye with progressive loss of vision. Treatment by laser is very e?ective if caught early, at the stage when a hole has developed in the retina but before the retina has become detached. The edges of the hole can be ‘spot welded’ to the underlying choroid. Once a detachment has occurred, laser therapy cannot be used; the retina has to be repositioned. This is usually done by indenting the wall of the eye from the outside to meet the retina, then making the retina stick to the wall of the eye by inducing in?ammation in the wall (by freezing it). The outcome of surgery depends largely on the extent of the detachment and its duration. Complicated forms of detachment can occur due to diabetic eye disease, injury or tumour. Each requires a specialised form of treatment.

Scleritis In?ammation of the sclera (see EYE). This can be localised or di?use, can affect the anterior or the posterior sclera, and can affect one or both eyes. The affected eye is usually red and painful. Scleritis can lead to thinning and even perforation of the sclera, sometimes with little sign of in?ammation. Posterior scleritis in particular may cause impaired vision and require emergency treatment. There is often no apparent cause, but there are some associated conditions – for example, RHEUMATOID ARTHRITIS, GOUT, and an autoimmune disease affecting the nasal passages and lungs called Wegener’s granulomatosis. Treatment depends on severity but may involve NON-STEROIDAL ANTI-INFLAMMATORY DRUGS (NSAIDS), topical CORTICOSTEROIDS or systemic immunosuppressive drugs.

Stye Infection of a lash follicle. This presents as a painful small red lump at the lid margin. It often resolves spontaneously but may require antibiotic treatment if it persists or recurs.

Sub-conjunctival haemorrhage Haemorrhage between the conjunctiva and the underlying episclera. It is painless. There is usually no apparent cause and it resolves spontaneously.

Trichiasis Inward misdirection of the lashes. Trichiasis occurs due to in?ammation of or trauma to the lid margin. Treatment involves removal of the patient’s lashes. Regrowth may be prevented by electrolysis, by CRYOTHERAPY to the lid margin, or by surgery.

For the subject of arti?cial eyes, see under PROSTHESIS; also GLAUCOMA, SQUINT and UVEITIS.... eye, disorders of

Fluocinolone is one of the CORTICOSTEROIDS and is applied to the skin as a cream, lotion or ointment. It is more potent than hydrocortisone. It must not be given by mouth.... fluocinolone

An aerosol corticosteroid drug used in the prevention and treatment of attacks of ASTHMA. Inhaled corticosteroids have few or no systemic side-effects unless given in excessive dosage.... fluticasone

One of the two main groups of CORTICOSTEROIDS. CORTISOL, CORTISONE and corticosterone are part of this group and are essential for the body to utilise CARBOHYDRATE, FAT and PROTEIN – in particular, when the body is reacting to stress. Glucocorticoids occur naturally but can be synthesised, and they have strong anti-in?ammatory properties, being used to treat conditions in which in?ammation is a part.... glucocorticoids

These can be acquired or congenital. The acquired type presents as a red PAPULE which bleeds easily; treatment is normally by cautery. A ‘strawberry NAEVUS’ is a ‘capillarycavernous’ haemangioma appearing at or soon after birth, which may grow to a large size. Treatment is not usually required, as most of them fade – although this may take a few years. Where a haemangioma is dis?guring or interfering with vision or breathing, treatment is necessary: this may be by laser, by using CORTICOSTEROIDS or INTERFERON treatment, or by surgery.... haemangioma

A collection of conditions in which the body’s immune system (see IMMUNITY) attacks its own tissues, identifying them as foreign substances. Genetic factors may play a part in this abnormal function, but the causes are not clear. The disorder may affect one organ (organ-speci?c) or type of cell, or several (non-organspeci?c). Among the autoimmune disorders are ADDISON’S DISEASE; autoimmune haemolytic anaemia and pernicious anaemia (see under ANAEMIA); autoimmune chronic active HEPATITIS; DIABETES MELLITUS; MYASTHENIA GRAVIS; RHEUMATOID ARTHRITIS; and SYSTEMIC LUPUS ERYTHEMATOSUS (SLE).

Treatment Any major de?ciencies, such as thyroxin or insulin lack, should be corrected. The activity of the immune system should then be reduced. CORTICOSTEROIDS and, in more severe cases, strong immunosuppressant drugs – AZATHIOPRINE, CYCLOPHOSPHAMIDE or METHOTREXATE – should be administered. Treatment is di?cult because of the need to control the autoimmune condition without damaging the body’s ability to combat other diseases.... autoimmune disorders

Candidosis (moniliasis) is an infection with the yeast, Candida albicans. It is encouraged by pregnancy, DIABETES MELLITUS, prolonged wide-spectrum ANTIBIOTICS or CORTICOSTEROIDS therapy, and is also seen in debilitated infants, the elderly and immunocompromised patients, e.g. those with AIDS/HIV. It may cause white patches in the mouth or vulvovaginal area (thrush) and a red vesicular and scaly rash in the ?nger clefts, beneath the breasts or in the groin or anogenital folds. Fingernail-fold infection causes chronic PARONYCHIA with secondary nail DYSTROPHY and may complicate RAYNAUD’S DISEASE. CLOTRIMAZOLE and similar ‘azoles’ as creams, oral gels or vaginal pessaries are rapidly e?ective, but severe systemic infections require oral itraconazole or even intravenous AMPHOTERICIN B.... candida

A chronic in?ammatory bowel disease which has a protracted, relapsing and remitting course. An autoimmune condition, it may last for several years. There are many similarities with ULCERATIVE COLITIS; sometimes it can be hard to di?erentiate between the two conditions. A crucial di?erence is that ulcerative colitis is con?ned to the colon (see INTESTINE), whereas Crohn’s disease can affect any part of the gastrointestinal tract, including the mouth and anus. The sites most commonly affected in Crohn’s disease (in order of frequency) are terminal ILEUM and right side of colon, just the colon, just the ileum and ?nally the ileum and JEJUNUM. The whole wall of the affected bowel is oedamatous (see OEDEMA) and thickened, with deep ulcers a characteristic feature. Ulcers may even penetrate the bowel wall, with abscesses and ?stulas developing. Another unusual feature is the presence in the affected bowel lining of islands of normal tissue.

Crohn’s disease is rare in the developing world, but in the western world the incidence is increasing and is now 6–7 per 100,000 population. Around 80,000 people in the UK have the disorder with more than 4,000 new cases occurring annually. Commonly Crohn’s disease starts in young adults, but a second incidence surge occurs in people over 70 years of age. Both genetic and environmental factors are implicated in the disease – for example, if one identical twin develops the disease, the second twin stands a high chance of being affected; and 10 per cent of sufferers have a close relative with in?ammatory bowel disease. Among environmental factors are low-residue, high-re?ned-sugar diets, and smoking.

Symptoms and signs of Crohn’s disease depend on the site affected but include abdominal pain, diarrhoea (sometimes bloody), ANOREXIA, weight loss, lethargy, malaise, ANAEMIA, and sore tongue and lips. An abdominal mass may be present. Complications can be severe, including life-threatening in?ammation of the colon (which may cause TOXAEMIA), perforation of the colon and the development of ?stulae between the bowel and other organs in the abdomen or pelvis. If Crohn’s disease persists for a decade or more there is an increased risk of the victim developing colon cancer. Extensive investigations are usually necessary to diagnose the disease; these include blood tests, bacteriological studies, ENDOSCOPY and biopsy, and barium X-ray examinations.

Treatment As with ulcerative colitis, treatment is aimed primarily at controlling symptoms. Physicians, surgeons, radiologists and dietitians usually adopt a team approach, while counsellors and patient support groups are valuable adjuncts in a disease that is typically lifelong. Drug treatment is aimed at settling the acute phase and preventing relapses. CORTICOSTEROIDS, given locally to the affected gut or orally, are used initially and the effects must be carefully monitored. If steroids do not work, the immunosuppressant agent AZATHIOPRINE should be considered. Antidiarrhoeal drugs may occasionally be helpful but should not be taken during an acute phase. The anti-in?ammatory drug SULFASALAZINE can be bene?cial in mild colitis. A new generation of genetically engineered anti-in?ammatory drugs is now available, and these selective immunosuppressants may prove of value in the treatment of Crohn’s disease.

Diet is important and professional guidance is advisable. Some patients respond to milk- or wheat-free diets, but the best course for most patients is to eat a well-balanced diet, avoiding items that the sufferer knows from experience are poorly tolerated. Of those patients with extensive disease, as many as 80 per cent may require surgery to alleviate symptoms: a section of affected gut may be removed or, as a lifesaving measure, a bowel perforation dealt with.

(See APPENDIX 2: ADDRESSES: SOURCES OF INFORMATION, ADVICE, SUPPORT AND SELFHELP – Colitis; Crohn’s disease.)... crohn’s disease

Dioscorea spp.

Dioscoreaceae

The growing need for steroidal drugs and the high cost of obtaining them from animal sources led to a widespread search for plant sources of steroidal sapogenins, which ultimately led to the most promising one. It is the largest genus of the family constituted by 600 species of predominantly twining herbs. Among the twining species, some species twine clockwise while others anti-clockwise (Miege, 1958). All the species are dioceous and rhizomatous. According to Coursey (1967), this genus is named in honour of the Greek physician Pedenios Dioscorides, the author of the classical Materia Medica Libri Quinque. Some of the species like D. alata and D. esculenta have been under cultivation for a long time for their edible tubers. There are about 15 species of this genus containing diosgenin. Some of them are the following (Chopra et al, 1980).

D. floribunda Mart. & Gal.

D. composita Hemsl; syn. D. macrostachya Benth.

D. deltoidea Wall. ex Griseb; syn. D. nepalensis Sweet ex Bernardi.

D. aculeata Linn. syn. D. esculenta

D. alata Linn. syn. D. atropurpurea Roxb.

D. Globosa Roxb; D. purpurea Roxb; D. rubella Roxb.

D. bulbifera Linn. syn. D. crispata Roxb.

D. pulchella Roxb.; D. sativa Thunb. Non Linn.

D. versicolor Buch. Ham. Ex Wall.

D. daemona Roxb. syn. D. hispida Dennst.

D. oppositifolia Linn.

D. pentaphylla Linn. syn. D. jacquemontii Hook. f.

D. triphylla Linn.

D. prazeri Prain & Burkil syn. D. clarkei Prain & Burkill

D. deltoidea Wall. var. sikkimensis Prain

D. sikkimensis Prain & Burkill

Among the above said species, D. floribunda, D. composita and D. deltoidea are widely grown for diosgenin production.

1. D. floribunda Mart. & Gal D. floribunda Mart. & Gal. is an introduction from central America and had wide adaptation as it is successfully grown in Karnataka, Assam, Meghalaya, Andaman and Goa. The vines are glabrous and left twining. The alternate leaves are borne on slender stems and have broadly ovate or triangular ovate, shallowly cordate, coriaceous lamina with 9 nerves. The petioles are 5-7cm long, thick and firm. Variegation in leaves occurs in varying degrees. The male flowers are solitary and rarely in pairs. Female flowers have divericate stigma which is bifid at apex. The capsule is obovate and seed is winged all round. The tubers are thick with yellow coloured flesh, branched and growing upto a depth of 30cm (Chadha et al, 1995).

2. D. composita Hemsl.

D. composita Hemsl. according to Knuth (1965) has the valid botanical name as D. macrostachya Benth. However, D. composita is widely used in published literature. It is a Central American introduction into Goa, Jammu, Bangalore, Anaimalai Hills of Tami Nadu and Darjeeling in W. Bengal. The vines are right twinning and nearly glabrous. The alternate leaves have long petioles, membraneous or coriaceous lamina measuring upto 20x18cm, abruptly acute or cuspidate-acuminate, shallowly or deeply cordate, 7-9 nerved. The fasciculate-glomerate inflorescence is single or branched with 2 or 3 sessile male flowers having fertile stamens. Male fascicle is 15-30cm long. The female flowers have bifid stigma. Tubers are large, white and deep-rooted (upto 45cm) (Chadha et al, 1995).

3. D. deltoidea Wall. ex. Griseb.

D. deltoidea Wall. ex. Griseb. is distributed throughout the Himalayas at altitudes of 1000-3000m extending over the states of Jammu-Kashmir, H. P, U. P, Sikkim and further into parts of W. Bengal. The glabrous and left twining stem bears alternate petiolate leaves. The petioles are 5-12 cm long. The lamina is 5-15cm long and 4-12cm wide widely cordate. The flowers are borne on axillary spikes, male spikes 8-40cm long and stamens 6. Female spikes are 15cm long, 3. 5cm broad and 4-6 seeded. Seeds are winged all round. Rhizomes are lodged in soil, superficial, horizontal, tuberous, digitate and chestnut brown in colour (Chadha et al, 1995). D. deltoidea tuber grows parallel to ground covered by small scale leaves and is described as rhizome. The tubers are morphologically cauline in structure with a ring of vascular bundles in young tubers which appear scattered in mature tubers (Purnima and Srivastava, 1988). Visible buds are present unlike in D. floribunda and D. composita where the buds are confined to the crown position (Selvaraj et al, 1972).

Importance of Diosgenin: Diosgenin is the most important sapogenin used as a starting material for synthesis of a number of steroidal drugs. For commercial purposes, its -isomer, yamogenin is also taken as diosgenin while analysing the sample for processing. Various steroidal drugs derived from diosgenin by artificial synthesis include corticosteroids, sex hormones, anabolic steroids and oral contraceptives. Corticosteroids are the most important group of steroidal drugs synthesized from diosgenin. First group of corticosteroids regulates carbohydrate and protein metabolism. The second group consists of aldosterone, which controls balance of potassium, sodium and water in the human body. The glucocorticoids in the form of cortisone and hydrocortisone are used orally, intramuscularly or topically for treatment of rheumatoid arthritis, rheumatic fever, other collegen diseases, ulcerative colitis, certain cases of asthma and a number of allergic diseases affecting skin, eye and the ear. These are also used for treatment of gout and a variety of inflammations of skin, eye and ear and as replacement therapy in Addison’s diseases. The minerato corticoides, desoxycorticosterone or desoxycortone are used in restoring kidney functions in cases of cortical deficiency and Addison’s disease.

Both male and female sex hormones are also synthesized from disosgenin. The main male sex hormone (androgen) which is produced from disogenin is testosterone. The main female sex hormones produced are oestrogen and progesterone. Recently oestrogen has also been used in cosmetic lotions and creams to improve the tone and colour of skin. One of the main uses of progesterone during recent years has been as antifertility agent for oral contraceptives. These artificial steroids have increased oral activity and fewer side effects, as they can be used in reduced doses. Oral contraceptives are also used for animals like pigs, cows and sheep to control fertility and to give birth at a prescribed period in a group of animals at the same time. These compounds are also used to reduce the interval between the lactation periods to have more milk and meat production. Anti-fertility compounds are also used as a pest-control measure for decreasing the multiplication of pests like rodents, pigeons and sea gulls (Husain et al, 1979).

Although yam tubers contain a variety of chemical substances including carbohydrates, proteins, alkaloids and tannins, the most important constituents of these yams are a group of saponins which yield sapogenins on hydrolysis. The most imp ortant sapogenin found in Dioscorea are diosgenin, yamogenin and pannogenin. Diosgenin is a steroid drug precursor. The diogenin content varies from 2-7% depending on the age of the tubers. Saponins including 5 spirastanol glucoside and 2 furostanol glucoside, 4 new steroid saponins, floribunda saponins C, D, E and F. Strain of A and B are obtained from D. floribunda (Husain et al, 1979). Rhizomes of D. deltoidea are a rich source of diosgenin and its glycoside. Epismilagenin and smilagenone have been isolated from D. deltoidea and D. prazeri (Chakravarti et al, 1960; 1962). An alkaloid dioscorine has been known to occur in D. hispida (Bhide et al,1978). Saponin of D. prazeri produced a fall of blood pressure when given intravenously and saponin of D. deltoidea has no effect on blood pressure (Chakravarti et al,1963). Deltonin, a steroidal glycoside, isolated from rhizomes of D. deltoidea showed contraceptive activity (Biokova et al, 1990).

Agrotechnology: Dioscorea species prefer a tropical climate without extremity in temperature. It is adapted to moderate to heavy rainfall area. Dioscorea plants can be grown in a variety of soils, but light soil is good, as harvesting of tubers is easier in such soils. The ideal soil pH is 5.5-6.5 but tolerates fairly wide variation in soil pH. Dioscorea can be propagated by tuber pieces, single node stem cuttings or seed. Commercial planting is normally established by tuber pieces only. Propagation through seed progeny is variable and it may take longer time to obtain tuber yields. IIHR, Bangalore has released two improved varieties, FB(c) -1, a vigorously growing strain relatively free from diseases and Arka Upkar, a high yielding clone. Three types of tuber pieces can be distinguished for propagation purpose, viz. (1) crown (2) median and (3) tip, of which crowns produce new shoots within 30 days and are therefore preferred. Dipping of tuber pieces for 5 minutes in 0.3% solution of Benlate followed by dusting the cut ends with 0.3% Benlate in talcum powder in mo ist sand beds effectively checks the tuber rot. The treatment is very essential for obtaining uniform stand of the crop. The best time of planting is the end of April so that new sprouts will grow vigorously during the rainy season commencing in June in India. Land is to be prepared thoroughly until a fine tilth is obtained. Deep furrows are made at 60cm distance with the help of a plough. The stored tuber pieces which are ready for planting is to be planted in furrows with 30cm between the plants for one year crop and 45cm between the plants for 2 year crop at about 0.5 cm below soil level. The new sprouts are to be staked immediately. After sprouting is complete, the plants are to be earthed up. Soil from the ridges may be used for earthing up so that the original furrows will become ridges and vice versa. Dioscorea requires high organic matter for good tuber formation. Besides a basal doze of 18-20t of FYM/ha, a complete fertilizer dose of 300kg N, 150kg P2O5 and K2O each are to be applied per hectare. P and K are to be applied in two equal doses one after the establishment of the crop during May-June and the other during vigorous growth period of the crop (August- September). Irrigation may be given at weekly intervals in the initial stage and afterwards at about 10 days interval. Dioscorea vines need support for their optimum growth and hence the vines are to be trailed over pandal system or trellis. Periodic hand weeding is essential for the first few months. Intercropping with legumes has been found to smother weeds and provide extra income. The major pests of Dioscorea are the aphids and red spider mites. Aphids occur more commonly on young seedlings and vines. Young leaves and vine tips eventually die if aphids are not controlled. Red spider mites attack the underside of the leaves at the base near the petiole. Severe infestations result in necrotic areas, which are often attacked by fungi. Both aphids and spider mites can be very easily controlled by Kelthane. No serious disease is reported to infect this crop. The tubers grow to about 25-30 cm depth and hence harvesting is to be done by manual labour. The best season for harvesting is Feb-March, coinciding with the dry period. On an average 50-60t/ha of fresh tubers can be obtained in 2 years duration. Diosgenin content tends to increase with age, 2.5% in first year and 3-3.5% in the second year. Hence, 2 year crop is economical (Kumar et al, 1997).... medicinal yams

A mineralcorticoid drug (see CORTICOSTEROIDS) with an action comparable to that of PREDNISOLONE, but e?ective at a somewhat lower dose.... methylprednisolone

See CORTICOSTEROIDS.... mineralcorticoid

A common form of irritant contact DERMATITIS in the nappy area in babies under one year old. Wetting of the skin by urine, abrasion, and chemical changes due to faecal contamination all play a part. Good hygiene and use of disposable absorbent nappies have much reduced its incidence. An ointment containing a barrier, such as titanium dioxide, may help; other medications such as mild CORTICOSTEROIDS or antibiotics should be used very cautiously and only under the guidance of a doctor, as harmful effects may result – especially from overuse.... nappy rash

A rare in?ammation of the skin which causes universal itching. The skin is red, thickened and scaly. It is also called generalised exfoliative dermatitis (see SKIN, DISEASES OF). It may complicate chronic eczema (see DERMATITIS) or PSORIASIS, particularly in men, in the second half of life. It may also result from HYPERSENSITIVITY to a drug, such as gold injections used in RHEUMATOID ARTHRITIS. Rarely, it may be a manifestation of T-cell LYMPHOMA.

Universal in?ammation of the skin may cause heart failure, particularly in elderly people with pre-existing heart disease. It may lead to HYPOTHERMIA due to excessive heat loss from the skin and protein de?ciency caused by the shedding of large quantities of skin scales containing keratin. Rarely, these complications can be fatal.

Treatment depends on the cause, but in eczematous erythroderma, oral CORTICOSTEROIDS (PREDNISOLONE) in full dosage may be needed.... erythroderma

Victims of eye injuries are advised to seek prompt medical advice if the injury is at all serious or does not resolve with simple ?rst-aid measures – for example, by washing out a foreign body using an eye bath.

Blunt injuries These may cause haemorrhage inside the eye, cataract, retinal detachment or even rupture of the eye (see also EYE, DISORDERS OF). Injuries from large blunt objects – for example, a squash ball – may also cause a ‘blow-out fracture’ of the orbital ?oor resulting in double vision. Surgical treatment may be required depending on the patient’s speci?c problems.

Chemical burns Most chemical splashes cause conjunctivitis and super?cial keratitis in the victim (see EYE, DISORDERS OF); both conditions are self-limiting. Alkalis are, however, more likely to penetrate deeper into the eye and cause permanent damage, particularly to the cornea. Prompt irrigation is important. Further treatment may involve testing the pH of the tears, topical antibiotics and CORTICOSTEROIDS, and vitamin C (drops or tablets – see APPENDIX 5: VITAMINS), depending on the nature of the injury.

Corneal abrasion Loss of corneal epithelium (outermost layer). Almost any sort of injury to the eye may cause this. The affected eye is usually very painful. In the absence of other problems, the epithelium heals rapidly: small defects may close within 24 hours. Treatment conventionally consists of antibiotic ointment and sometimes a pad over the injured eye.

Foreign bodies Most foreign bodies which hit the eye are small and are found in the conjunctival sac or on the cornea; most are super?cial and can be easily removed. A few foreign bodies penetrate deeper and may cause infection, cataract, retinal detachment or haemorrhage within the eye. The foreign body is usually removed and the damage repaired; nevertheless the victim’s sight may have been permanently damaged. Particularly dangerous activities include hammering or chiselling on metal or stone; people carrying out these activities (and others, such as hedge-cutting and grass-strimming) should wear protective goggles.... eye injuries

Hydrocortisone has the chemical formula, 17hydroxycorticosterone. It is closely allied to CORTISONE both in its structure (cortisone is an oxidation product of hydrocortisone) and in its action. Available in tablet, topical or injection form, hydrocortisone is used in adrenocortical insu?ciency, for the suppression of local and systemic in?ammatory and allergic disorders, and in the treatment of shock. Its mineralocorticoid effects – control of salt and water balance – mean that the drug should not be used long term except as replacement therapy in the treatment of ADDISON’S DISEASE or following adrenalectomy when hydrocortisone should be given with the mineral corticoid

?udrocortisone (see ADRENAL GLANDS; CORTICOSTEROIDS).... hydrocortisone

In?ammation of the optic nerve (see EYE) which may result in sudden loss of part of a person’s vision. It is usually accompanied by pain and tenderness on touch. The cause is uncertain, although in some cases it may be a prcursor of MULTIPLE SCLEROSIS (MS): CORTICOSTEROIDS may help by improving the loss of visual acuity, but seems not to check the long-term in?ammatory activity.... optic neuritis

Also known as jumper’s knee. In?ammation of the tendon of the extensor muscle of the thigh, in which the PATELLA or knee-cap is secured. Usually the result of injury or excessive use or stress – for example, in athletic training – symptoms include pain, tenderness and sometimes restricted movement of the parent muscle. Treatment may include NON-STEROIDAL ANTIINFLAMMATORY DRUGS (NSAIDS), ULTRASOUND treatment and PHYSIOTHERAPY, and, if persistent, injection of a corticosteroid drug (see CORTICOSTEROIDS) around the tendon.... patellar tendinitis

In?ammation of the LIVER which damages liver cells and may ultimately kill them. Acute injury of the liver is usually followed by complete recovery, but prolonged in?ammation after injury may result in FIBROSIS and CIRRHOSIS. Excluding trauma, hepatitis has several causes:

Viral infections by any of hepatitis A, B, C, D, or E viruses and also CYTOMEGALOVIRUS (CMV), EPSTEIN BARR VIRUS, and HERPES SIMPLEX.

Autoimmune disorders such as autoimmune chronic hepatitis, toxins, alcohol and certain drugs – ISONIAZID, RIFAMPICIN, HALOTHANE and CHLORPROMAZINE.

WILSON’S DISEASE.

Acute viral hepatitis causes damage throughout the liver and in severe infections may destroy whole lobules (see below).

Chronic hepatitis is typi?ed by an invasion of the portal tract by white blood cells (mild hepatitis). If these mononuclear in?ammatory cells invade the body (parenchyma) of the liver tissue, ?brosis and then chronic disease or cirrhosis can develop. Cirrhosis may develop at any age and commonly results in prolonged ill health. It is an important cause of premature death, with excessive alcohol consumption commonly the triggering factor. Sometimes, cirrhosis may be asymptomatic, but common symptoms are weakness, tiredness, poor appetite, weight loss, nausea, vomiting, abdominal discomfort and production of abnormal amounts of wind. Initially, the liver may enlarge, but later it becomes hard and shrunken, though rarely causing pain. Skin pigmentation may occur along with jaundice, the result of failure to excrete the liver product BILIRUBIN. Routine liver-function tests on blood are used to help diagnose the disease and to monitor its progress. Spider telangiectasia (caused by damage to blood vessels – see TELANGIECTASIS) usually develop, and these are a signi?cant pointer to liver disease. ENDOCRINE changes occur, especially in men, who lose their typical hair distribution and suffer from atrophy of their testicles. Bruising and nosebleeds occur increasingly as the cirrhosis worsens, and portal hypertension (high pressure of venous blood circulation through the liver) develops due to abnormal vascular resistance. ASCITES and HEPATIC ENCEPHALOPATHY are indications of advanced cirrhosis.

Treatment of cirrhosis is to tackle the underlying cause, to maintain the patient’s nutrition (advising him or her to avoid alcohol), and to treat any complications. The disorder can also be treated by liver transplantation; indeed, 75 per cent of liver transplants are done for cirrhosis. The overall prognosis of cirrhosis, however, is not good, especially as many patients attend for medical care late in the course of the disease. Overall, only 25 per cent of patients live for ?ve years after diagnosis, though patients who have a liver transplant and survive for a year (80 per cent do) have a good prognosis.

Autoimmune hepatitis is a type that most commonly occurs in women between 20 and 40 years of age. The cause is unknown and it has been suggested that the disease has several immunological subtypes. Symptoms are similar to other viral hepatitis infections, with painful joints and AMENORRHOEA as additional symptoms. Jaundice and signs of chronic liver disease usually occur. Treatment with CORTICOSTEROIDS is life-saving in autoimmune hepatitis, and maintenance treatment may be needed for two years or more. Remissions and exacerbations are typical, and most patients eventually develop cirrhosis, with 50 per cent of victims dying of liver failure if not treated. This ?gure falls to 10 per cent in treated patients.

Viral hepatitis The ?ve hepatic viruses (A to E) all cause acute primary liver disease, though each belongs to a separate group of viruses.

•Hepatitis A virus (HAV) is an ENTEROVIRUS

which is very infectious, spreading by faecal contamination from patients suffering from (or incubating) the infection; victims excrete viruses into the faeces for around ?ve weeks during incubation and development of the disease. Overcrowding and poor sanitation help to spread hepatitis A, which fortunately usually causes only mild disease.

Hepatitis B (HBV) is caused by a hepadna virus, and humans are the only reservoir of infection, with blood the main agent for transferring it. Transfusions of infected blood or blood products, and injections using contaminated needles (common among habitual drug abusers), are common modes of transfer. Tattooing and ACUPUNCTURE may spread hepatitis B unless high standards of sterilisation are maintained. Sexual intercourse, particularly between male homosexuals, is a signi?cant infection route.

Hepatitis C (HCV) is a ?avivirus whose source of infection is usually via blood contacts. E?ective screening of blood donors and heat treatment of blood factors should prevent the spread of this infection, which becomes chronic in about 75 per cent of those infected, lasting for life. Although most carriers do not suffer an acute illness, they must practise life-long preventive measures.

Hepatitis D (HDV) cannot survive independently, needing HBV to replicate, so its sources and methods of spread are similar to the B virus. HDV can infect people at the same time as HBV, but it is capable of superinfecting those who are already chronic carriers of the B virus. Acute and chronic infection of HDV can occur, depending on individual circumstances, and parenteral drug abuse spreads the infection. The disease occurs worldwide, being endemic in Africa, South America and the Mediterranean littoral.

Hepatitis E virus (HEV) is excreted in the stools, spreading via the faeco-oral route. It causes large epidemics of water-borne hepatitis and ?ourishes wherever there is poor sanitation. It resembles acute HAV infection and the patient usually recovers. HEV does not cause chronic infection. The clinical characteristics of the ?ve hepatic

viruses are broadly similar. The initial symptoms last for up to two weeks (comprising temperature, headache and malaise), and JAUNDICE then develops, with anorexia, nausea, vomiting and diarrhoea common manifestations. Upper abdominal pain and a tender enlarged liver margin, accompanied by enlarged cervical lymph glands, are usual.

As well as blood tests to assess liver function, there are speci?c virological tests to identify the ?ve infective agents, and these are important contributions to diagnosis. However, there is no speci?c treatment of any of these infections. The more seriously ill patients may require hospital care, mainly to enable doctors to spot at an early stage those developing acute liver failure. If vomiting is a problem, intravenous ?uid and glucose can be given. Therapeutic drugs – especially sedatives and hypnotics – should be avoided, and alcohol must not be taken during the acute phase. Interferon is the only licensed drug for the treatment of chronic hepatitis B, but this is used with care.

Otherwise-?t patients under 40 with acute viral hepatitis have a mortality rate of around

0.5 per cent; for those over 60, this ?gure is around 3 per cent. Up to 95 per cent of adults with acute HBV infection recover fully but the rest may develop life-long chronic hepatitis, particularly those who are immunode?cient (see IMMUNODEFICIENCY).

Infection is best prevented by good living conditions. HVA and HVB can be prevented by active immunisation with vaccines. There is no vaccine available for viruses C, D and E, although HDV is e?ectively prevented by immunisation against HBV. At-risk groups who should be vaccinated against HBV include:

Parenteral drug abusers.

Close contacts of infected individuals such as regular sexual partners and infants of infected mothers.

Men who have sex with men.

Patients undergoing regular haemodialysis.

Selected health professionals, including laboratory sta? dealing with blood samples and products.... hepatitis

Means high BLOOD PRESSURE (raised pressure of the circulating blood), but since there is a wide range of ‘normal’ blood pressure in the population, a precise level of pressure above which an individual is deemed hypertensive is arbitrary. (A healthy young adult would be expected to have a systolic pressure of around 120 mm Hg and a diastolic of 80 mm Hg, recorded as 120/80.) Hypertension is not a disease as such but a quantitative deviation from the norm. A person with a pressure higher than the average for his or her age group is usually symptomless – although sometimes such people may develop headaches. The identi?cation of people with hypertension is important because it is a signal that they will be more likely to have a STROKE or myocardial infarct (coronary thrombosis or heart attack) than someone whose pressure is in the ‘normal’ range. Preventive steps can then be taken to lessen the likelihood of their developing these potentially life-threatening conditions.

Blood pressure is measured using two values. The systolic pressure – the greater of the two – represents the pressure when blood is pumped from the left VENTRICLE of the heart into the AORTA. The diastolic pressure is the measurement when both ventricles relax between beats. The pressures are measured in millimetres (mm) of mercury (Hg). Despite the grey area between normal and raised blood pressure, the World Health Organisation (WHO) has de?ned hypertension as a blood pressure consistently greater than 160 mm Hg (systolic) and 95 mm Hg (diastolic). Young children have readings well below these, but blood pressure rises with age and a healthy person may well live symptom free with a systolic pressure above the WHO ?gure. A useful working de?nition of hypertension is the ?gure at which the bene?ts of treating the condition outweigh the risks and costs of the treatment.

Between 10 and 20 per cent of the adult population in the UK has hypertension, with more men than women affected. Incidence is highest in the middle-aged and elderly. Because most people with hypertension are symptomless, the condition is often ?rst identi?ed during a routine medical examination, otherwise a diagnosis is usually made when complications occur. Many people’s blood pressure rises when they are anxious or after exercise, so if someone’s pressure is above normal at the ?rst testing, it should be taken again after, say, 10 minutes’ rest, by which time the reading should have settled to the person’s regular level. BP measurements should then be taken on two subsequent occasions. If the pressure is still high, the cause needs to be determined: this is done using a combination of personal and family histories (hypertension can run in families), a physical examination and investigations, including an ECG and blood tests for renal disease.

Over 90 per cent of hypertensive people have no immediately identi?able cause for their condition. They are described as having essential hypertension. In those patients with an identi?able cause, the hypertension is described as secondary. Among the causes of secondary hypertension are:

Lifestyle factors such as smoking, alcohol, stress, excessive dietary salt and obesity.

Diseases of the KIDNEYS.

Pregnancy (ECLAMPSIA).

Various ENDOCRINE disorders – for example, PHAEOCHROMOCYTOMA, CUSHING’S DISEASE, ACROMEGALY, thyrotoxicosis (see under THYROID GLAND, DISEASES OF).

COARCTATION OF THE AORTA.

Drugs – for example, oestrogen-containing oral contraceptives (see under CONTRACEPTION), ANABOLIC STEROIDS, CORTICOSTEROIDS, NON-STEROIDAL ANTIINFLAMMATORY DRUGS (NSAIDS).

Treatment People with severe hypertension may need prompt admission to hospital for urgent investigation and treatment. Those with a mild to moderate rise in blood pressure for which no cause is identi?able should be advised to change their lifestyle: smokers should stop the habit, and those with high alcohol consumption should greatly reduce or stop their drinking. Obese people should reduce their food consumption, especially of animal fats, and take more exercise. Everyone with hypertension should follow a low-salt diet and take regular exercise. Patients should also be taught how to relax, which helps to reduce blood pressure and, if they have a stressful life, working patterns should be modi?ed if possible. If these lifestyle changes do not reduce a person’s blood pressure su?ciently, drugs to achieve this will be needed. A wide range of anti-hypertensive drugs are available on prescription.

A ?rst-line treatment is one of the THIAZIDES, e?ective at a low dosage and especially useful in the elderly. Beta blockers (see BETAADRENOCEPTOR-BLOCKING DRUGS), such as oxprenolol, acebutol or atenolol, are also ?rst-line treatments. ACE inhibitors (see ANGIOTENSIN-CONVERTING ENZYME (ACE) INHIBITORS) and CALCIUM-CHANNEL BLOCKERS can be used if the ?rst-line choices are not e?ective. The drug treatment of hypertension is complex, and sometimes various drugs or combinations of drugs have to be tried to ?nd what regimen is e?ective and suits the patient. Mild to moderate hypertension can usually be treated in general practice, but patients who do not respond or have complications will normally require specialist advice. Patients on anti-hypertensive treatments require regular monitoring, and, as treatment may be necessary for several years, particular attention should be paid to identifying sideeffects. Nevertheless, e?ective treatment of hypertension does enable affected individuals to live longer and more comfortable lives than would otherwise be the case. Older people with moderately raised blood pressure are often able to live with the condition, and treatment with anti-hypertensive drugs may produce symptoms of HYPOTENSION.

In summary, hypertension is a complex disorder, with di?erent patients responding di?erently to treatment. So the condition sometimes requires careful assessment before the most e?ective therapy for a particular individual is identi?ed, and continued monitoring of patients with the disorder is advisable.

Complications Untreated hypertension may eventually result in serious complications. People with high blood pressure have blood vessels with thickened, less ?exible walls, a narrowed LUMEN and convoluted shape. Sometimes arteries become rigid. ANEURYSM may develop and widespread ATHEROMA (fat deposits) is apparent in the arterial linings. Such changes adversely affect the blood supply to body tissues and organs and so damage their functioning. Patients suffer STROKE (haemorrhage from or thrombosis in the arteries of the BRAIN) and heart attacks (coronary thrombosis

– see HEART, DISEASES OF). Those with hypertension may suffer damage to the retina of the EYE and to the OPTIC DISC. Indeed, the diagnosis of hypertension is sometimes made during a routine eye test, when the doctor or optician notices changes in the retinal arteries or optic disc. Kidney function is often affected, with patients excreting protein and excessive salt in their urine. Occasionally someone with persistent hypertension may suffer an acceleration of damage to the blood vessels – a condition described as ‘malignant’ hypertension, and one requiring urgent hospital treatment.

Hypertension is a potentially dangerous disease because it develops into a cycle of self-perpetuating damage. Faulty blood vessels lead to high blood pressure which in turn aggravates the damage in the vessels and thus in the tissues and organs they supply with blood; this further raises the affected individual’s blood pressure and the pathological cycle continues.... hypertension

Sometimes described as thrombocytopenia, this is an autoimmune disorder in which blood PLATELETS are destroyed. This disturbs the blood’s coagulative properties (see COAGULATION) and spontaneous bleeding (PURPURA) occurs into the skin. The disease may be acute in children but most recover without treatment. Adults may develop a more serious, chronic variety which requires treatment with CORTICOSTEROIDS and sometimes SPLENECTOMY. Should the disease persist despite these treatments, intravenous immunoglobulin or immunosuppressive drugs (see IMMUNOSUPPRESSION) are worth trying. Should the bleeding be or become life-threatening, concentrates of platelets should be administered.... idiopathic thrombocytopenic purpura (itp)

The term given to suppression of harmful immune responses (see IMMUNITY), the most obvious application being the prevention of organ rejection by people who receive kidney, heart or bone-marrow transplants (see TRANSPLANTATION). Immunosuppression is also used in certain diseases in a way that is non-speci?c – that is, it inhibits the entire immune system, not just harmful reactions. CORTICOSTEROIDS are the commonest dugs used in this way, as are METHOTREXATE and AZATHIOPRINE. Tacrolimus, a macrolide (see MACROLIDES) IMMUNOSUPPRESSANT, is used not only for engrafted patients but also in treating eczema (see DERMATITIS).

There has been a rapid introduction in recent years of monoclonal antibodies which prevent T-cells from proliferating. They can be recognised by the su?x ‘mab’ (standing for monoclonal antibody) and include rituximab and alemtuzumab. In?iximab, used in CROHN’S DISEASE and RHEUMATOID ARTHRITIS, inhibits tumour necrosis factor alpha.... immunosuppression

Substances that can be inhaled into the body through the lungs. They may be delivered in traditional form dissolved in hot water and inhaled in the steam, or as an aerosol – a suspension of very small liquid or solid particles in the air. The latter are now usually delivered by devices in which the aerosol is kept under pressure in a small hand-held cylinder and delivered in required doses by a release mechanism.

Aerosols Asthmatic patients (see ASTHMA) ?nd aerosol devices to be of value in controlling their attacks. They provide an e?ective and convenient way of applying drugs directly to the bronchi, thus reducing the risks of unwanted effects accompanying SYSTEMIC therapy. BRONCHODILATOR aerosols contain either a beta-sympathomimetic agent or ipratropium bromide, which is an ANTICHOLINERGIC drug.

ISOPRENALINE was the ?rst compound to be widely used as an aerosol. It did however stimulate beta1 receptors in the heart as well as beta2 receptors in the bronchi, and so produced palpitations and even dangerous cardiac arrhythmias. Newer beta-adrenoceptor agonists are speci?c for the beta2 receptors and thus have a greater safety margin. They include SALBUTAMOL, TERBUTALINE, rimiterol, fenoterol and reproterol. Unwanted effects such as palpitations, tremor and restlessness are uncommon with these, more speci?c preparations. In patients who get insu?cient relief from the beta-adrenoreceptor agonist, the drug ipratropium bromide is worth adding. Salmeterol is a longer-acting choice for twice-daily administration: it is not intended for the relief of acute attacks, for which shorter-acting beta2 stimulants such as salbutamol should be used. Salmeterol should be added to existing corticosteroid therapy (see CORTICOSTEROIDS), rather than replacing it.

Patients must be taught carefully and observed while using their inhalers. It is important for them to realise that if the aerosol no longer gives more than slight transient relief, they should not increase the dose but seek medical help.... inhalants

This corticosteroid drug has a similar level of glucocorticoid activity as PREDNISOLONE and is converted to prednisolone in the liver. Though prednisone is still in use, prednisolone is the most commonly used oral corticosteroid for long-term anti-in?ammatory treatment. (See CORTICOSTEROIDS; GLUCOCORTICOIDS.)... prednisone

A short-acting selective beta2-adrenoceptor stimulant delivered via a metered-dose aerosol inhaler, a powder inhaler or through a nebuliser to control symptoms of ASTHMA. If stimulant inhalation is needed more than twice a day to control asthma attacks, prophylactic treatment should be considered including, in severe cases, oral CORTICOSTEROIDS. Salbutamol relaxes the muscles which cause bronchial spasms in the lungs – the prime symptom of asthma. There are other similar preparations such as terbutaline.... salbutamol

The group name for compounds that resemble CHOLESTEROL chemically. The group includes the sex hormones, the hormones of the adrenal cortex, and bile acids. They have a powerful in?uence on the normal functioning of the body, and natural and synthetic steroids are used in the treatment of many disorders. (See CORTICOSTEROIDS; ENDOCRINE GLANDS.)... steroid

‘Rheumatism’ is the colloquial term for nonspeci?c musculoskeletal symptoms arising in the joints, ligaments, tendons and muscles. ‘Arthritis’ describes a pathological musculoskeletal disorder. Most common are sprains of ligaments, strains of tendons and muscles,

BURSITIS, TENDINITIS and non-speci?c back pain (see BACKACHE).

Osteoarthritis (OA) rarely starts before 40, but by the age of 80 affects 80 per cent of the population. There are structural and functional changes in the articular cartilage, as well as changes in the collagenous matrix of tendons and ligaments. OA is not purely ‘wear and tear’; various sub-groups have a genetic component. Early OA may be precipitated by localised alteration in anatomy, such as a fracture or infection of a joint. Reactive new bone growth typically occurs, causing sclerosis (hardening) beneath the joint, and osteophytes – outgrowths of bone – are characteristic at the margins of the joint. The most common sites are the ?rst metatarsal (great toe), spinal facet joints, the knee, the base of the thumb and the terminal ?nger joints (Heberden’s nodes).

OA has a slow but variable course, with periods of pain and low-grade in?ammation. Acute in?ammation, common in the knee, may result from release of pyrophosphate crystals, causing pseudo-gout.

Urate gout results from crystallisation of URIC ACID in joints, against a background of hyperuricaemia. This high concentration of uric acid in the blood may result from genetic and environmental factors, such as excess dietary purines, alcohol or diuretic drugs.

In?ammatory arthritis is less common than OA, but potentially much more serious. Several types exist, including: SPONDYLARTHRITIS This affects younger men, chie?y involving spinal and leg joints. This may lead to in?ammation and eventual ossi?cation of the enthesis – that is, where the ligaments and tendons are inserted into the bone around joints. This may be associated with disorders in other parts of the body: skin in?ammation (PSORIASIS), bowel and genito-urinary in?ammation, sometimes resulting in infection of the organs (such as dysentery). The syndromes most clearly delineated are ankylosing spondylitis (see SPINE AND SPINAL CORD, DISEASES AND INJURIES OF), psoriatic or colitic spondylitis, and REITER’S SYNDROME. The diagnosis is made clinically and radiologically; no association has been found with autoantibodies (see AUTOANTIBODY). A particularly clear gene locus, HLA B27, has been identi?ed in ankylosing spondylitis. Psoriasis can be associated with a characteristic peripheral arthritis.

Systemic autoimmune rheumatic diseases (see AUTOIMMUNE DISORDERS). RHEUMATOID ARTHRITIS (RA) – see also main entry. The most common of these diseases. Acute in?ammation causes lymphoid synovitis, leading to erosion of the cartilage, associated joints and soft tissues. Fibrosis follows, causing deformity. Autoantibodies are common, particularly Rheumatoid Factor. A common complication of RA is Sjögren’s syndrome, when in?ammation of the mucosal glands may result in a dry mouth and eyes. SYSTEMIC LUPUS ERYTHEMATOSUS (SLE) and various overlap syndromes occur, such as systemic sclerosis and dermatomyositis. Autoantibodies against nuclear proteins such as DNA lead to deposits of immune complexes and VASCULITIS in various tissues, such as kidney, brain, skin and lungs. This may lead to various symptoms, and sometimes even to organ failure.

Infective arthritis includes: SEPTIC ARTHRITIS An uncommon but potentially fatal disease if not diagnosed and treated early with approriate antibiotics. Common causes are TUBERCLE bacilli and staphylococci (see STAPHYLOCOCCUS). Particularly at risk are the elderly and the immunologically vulnerable, such as those under treatment for cancer, or on CORTICOSTEROIDS or IMMUNOSUPPRESSANT drugs. RHEUMATIC FEVER Now rare in western countries. Resulting from an immunological reaction to a streptococcal infection, it is characterised by migratory arthritis, rash and cardiac involvement.

Other infections which may be associated with arthritis include rubella (German measles), parvovirus and LYME DISEASE.

Treatment Septic arthritis is the only type that can be cured using antibiotics, while the principles of treatment for the others are similar: to reduce risk factors (such as hyperuricaemia); to suppress in?ammation; to improve function with physiotherapy; and, in the event of joint failure, to perform surgical arthroplasty. NON-STEROIDAL ANTI-INFLAMMATORY DRUGS (NSAIDS) include aspirin, paracetamol and many recently developed ones, such as the proprionic acid derivatives IBUPROFEN and naproxen, along with other drugs that have similar properties such as PIROXICAM. They all carry a risk of toxicity, such as renal dysfunction, or gastrointestinal irritation with haemorrhage. Stronger suppression of in?ammation requires corticosteroids and CYTOTOXIC drugs such as azathioprine or cyclophosphamide. Recent research promises more speci?c and less toxic anti-in?ammatory drugs, such as the monoclonal antibodies like in?iximab. An important treatment for some osteoarthritic joints is surgical replacement of the joints.... joints, diseases of

Stretch-marks seen in the skin, common in adolescent boys and girls owing to stretching of the skin by rapid growth (striae distensae). In boys, striae occur around the shoulders and thighs; in girls the breasts and hips are affected. In both sexes horizontal striae on the back may be mistaken for signs of trauma. Striae are common in women in late pregnancy, especially on the lower abdomen (striae gravidarum). Injudicious prolonged use of potent topical CORTICOSTEROIDS can induce striae, particularly about the groins, inner thighs or armpits. Prolonged high-dose oral STEROID therapy may cause widespread striae.... striae

An IMMUNOSUPPRESSANT drug used for primary immunosuppression in recipients of kidney or liver transplants (see TRANSPLANTATION) where the natural rejection process has been resistant to conventional immunosuppression regimens such as CORTICOSTEROIDS, AZATHIOPRINE and CICLOSPORIN A. It is also used, with caution, in some severe cases of eczema (see DERMATITIS).... tacrolimus

One of the CORTICOSTEROIDS with a potency equivalent to that of PREDNISONE, but less likely to cause retention of sodium. It is used for the suppression of in?ammatory and allergic disorders, and is used particularly for treating the skin and joints by local injection.... triamcinolone

Drugs that are used to treat or prevent allergic reactions (see allergy). There are several groups, including corticosteroids, antihistamines, leukotriene receptor antagonists, and sodium cromoglicate.... antiallergy drugs

Various tonics and remedies traditionally prescribed to stimulate the appetite.

None are proven to be effective.

Some drugs such as corticosteroids may stimulate the appetite when used to treat unrelated disorders.... appetite stimulants

Also known as croup. An acute infection of the respiratory tract in infants and young children, usually caused by parain?uenza virus. The onset is variable but the croupy cough and stridulous breathing usually occur a few days after the onset of a viral upper-respiratory-tract infection. A harsh barking cough is typical of the condition. The majority of affected children can be treated with HUMIDIFICATION and a single dose of inhaled corticosteroid (budesonide – see CORTICOSTEROIDS) or a single day’s treatment with oral prednisolone. Severe croup can cause serious breathing problems when the child should be referred for urgent specialist assessment, and hospitalisation is preferable in all cases. Rarely, some form of intervention is necessary and this will either be in the form of endotracheal intubation or of a tracheostomy.... laryngo-tracheo-bronchitis

Also known as Hansen’s disease, this is a chronic bacterial infection caused by Mycobacterium leprae affecting the skin, mucous membranes, and nerves. Infection is now almost con?ned to tropical and subtropical countries – mostly in Africa and India. There are two distinct (polarised) clinical forms: tuberculoid and lepromatous. The former usually takes a benign course and frequently burns out, whereas the latter is relentlessly progressive; between these two polar forms lies an intermediate/dimorphous group. Susceptibility may be increased by malnutrition. Nasal secretions (especially in lepromatous disease) are teeming with M. leprae and constitute the main source of infection; however, living in close proximity to an infected individual seems necessary for someone to contract the disease. M. leprae can also be transmitted in breast milk from an infected mother.

Only a small minority of those exposed to M. leprae develop the disease. The incubation period is 3–5 years or longer. The major clinical manifestations involve skin and nerves: the former range from depigmented, often anaesthetic areas, to massive nodules; nerve involvement ranges from localised nerve swelling(s) to extensive areas of anaesthesia. Advanced nerve destruction gives rise to severe deformities: foot-drop, wrist-drop, claw-foot, extensive ulceration of the extremities with loss of ?ngers and toes, and bone changes. Eye involvement can produce blindness. Laryngeal lesions produce hoarseness and more serious sequelae. The diagnosis is essentially a clinical one; however, skin-smears, histological features and the lepromin skin-test help to con?rm the diagnosis and enable the form of disease to be graded.

Although the World Health Organisation had originally hoped to eliminate leprosy worldwide by 2000, that has proved an unrealistic target. The reason is an absence of basic information. Doctors are unable to diagnose the disorder before a patient starts to show symptoms; meanwhile he or she may have already passed on the infection. Doctors do not know exactly how transmission occurs or how it infects humans – nor do they know at what point a carrier of the bacterium may infect others. The incidence of new infections is still more than 650,000 cases a year or about 4.5 cases per 10,000 people in those countries worst affected by the disease.

Treatment Introduction of the sulphone compound, dapsone, revolutionised management of the disease. More recently, rifampicin and clofazimine have been added as ?rst-line drugs for treatment. Second-line drugs include minocycline, o?oxacin and clarithromycin; a number of regimens incorporating several of these compounds (multi-drug regimens – introduced in 1982) are now widely used. A three-drug regime is recommended for multi-bacillary leprosy and a two-drug one for parcibacillary leprosy. Dapsone resistance is a major problem worldwide, but occurs less commonly when multi-drug regimens are used. Older compounds – ethionamide and prothionamide

– are no longer used because they are severely toxic to the liver. Corticosteroids are sometimes required in patients with ‘reversal reaction’. Supportive therapy includes physiotherapy; both plastic and orthopaedic surgery may be necessary in advanced stages of the disease. Improvement in socio-economic conditions, and widespread use of BCG vaccination are of value as preventive strategies. Early diagnosis and prompt institution of chemotherapy should prevent long-term complications.... leprosy

Lichen, or licheni?cation, is a term used to describe a thickening of chronically in?amed skin to give a tree-bark-like appearance.

Lichen simplex (neurodermatitis) is a form of eczema (see DERMATITIS) perpetuated by constant rubbing of the affected skin. Typically, well-de?ned plaques occur on one or both sides of the nape of the neck, on the ulnar forearm near the elbow, or on the sides of the calves. It is often associated with emotional stress.

Lichen planus is a less common in?ammation of the skin characterised by small, shiny, ?at-topped violaceous papules which may coalesce to form large plaques. Itching can be intense. Typically seen on the ?exor aspects of the wrists, the lower back and on the legs below the knees, it may also affect the mucous membranes of the mouth and lips. The cause is unknown. While in some patients the disorder appears to be nervous or emotional in origin, it can be caused by certain drugs such as CHLOROQUINE. Severe cases may require oral CORTICOSTEROIDS to control the eruption.... lichen

A device used for administering a drug in powder or vapour form, used mainly in the treatment of various respiratory disorders, including asthma and chronic bronchitis. Metered-dose inhalers deliver a precise dose when the inhaler is pressed. Drugs taken by inhalation include bronchodilators and corticosteroids.... inhaler

A group of antiallergy drugs, such as montelukast and zafirlukast, used to prevent symptoms of mild to moderate asthma. The drugs work by blocking the effects of leukotrienes – naturally occurring substances released in the lungs during an allergic reaction.

Because they are not bronchodilator drugs, and will not relieve an existing attack, they are usually used with bronchodilators and inhaled corticosteroids to reduce the frequency of attacks.

Side effects include gastrointestinal disturbances and headache.

Skin and hypersensitivity reactions may also occur.... leukotriene receptor antagonists

Pain in the front and sides of the lower leg that develops or worsens during exercise. There may also be tenderness and oedema in the affected area. Shin splints is a common problem in runners. It may be caused by various disorders, such as compartment syndrome, tendinitis, myositis, or periostitis.

In most cases, the pain disappears after 1 or 2 weeks of rest. However, if it is severe or recurrent, a course of nonsteroidal anti-inflammatory drugs or corticosteroids may be needed. Rarely, surgery is performed to alleviate excessive pressure in a muscle. Some people benefit from physiotherapy.... shin splints

Leukaemia is an umbrella term for several malignant disorders of white blood cells in which they proliferate in a disorganised manner. The disease is also characterised by enlargement of the SPLEEN, changes in the BONE MARROW, and by enlargement of the LYMPH glands all over the body. The condition may be either acute or chronic.

According to the type of cells that predominate, leukaemia may be classi?ed as acute or chronic lymphoblastic leukaemia or myeloid leukaemia. Acute lymphoblastic leukaemia (ALL) is mostly a disease of childhood and is rare after the age of 25. Acute myeloid leukaemia is most common in children and young adults, but may occur at any age. Chronic lymphatic leukaemia occurs at any age between 35 and 80, most commonly in the 60s, and is twice as common in men as in women. Chronic myeloid leukaemia is rare before the age of 25, and most common between the ages of 30 and 65; men and women are equally affected. Around 2,500 patients with acute leukaemia are diagnosed in the United Kingdom, with a similar number annually diagnosed with chronic leukaemia.

Cause Both types of acute leukaemia seem to arise from a MUTATION in a single white cell. The genetically changed cell then goes through an uncontrolled succession of divisions resulting in many millions of abnormal white cells in the blood, bone marrow and other tissues. Possible causes are virus infection, chemical exposure, radiation and genetic background. The cause of chronic lymphocytic leukaemia is not known; the chronic myeloid version may have a genetic background.

Symptoms In acute cases the patient is pale due to anaemia, may have a purpuric rash due to lack of platelets, and may have enlarged lymphatic glands and spleen. The temperature is raised, and the condition may be mistaken for an acute infection (or may ?rst become apparent because the patient develops a severe infection due to a lack of normal white blood cells).

In the chronic type of the disease the onset is gradual, and the ?rst symptoms which occasion discomfort are either swelling of the abdomen and shortness of breath, due to painless enlargement of the spleen; or the enlargement of glands in the neck, armpits and elsewhere; or the pallor, palpitation, and other symptoms of anaemia which often accompany leukaemia. Occasional bleeding from the nose, stomach, gums or bowels may occur, and may be severe. Generally, there is a slight fever.

When the blood is examined microscopically, not only is there an enormous increase in the number of white cells, which may be multiplied 30- or 60-fold, but various immature forms are also found. In the lymphatic form of the disease, most white cells resemble lymphocytes, which, in healthy blood, are present only in small numbers. In the myeloid form, myelocytes, or large immature cells from the bone marrow, which are never present in healthy blood, appear in large numbers, and there may also be large numbers of immature, nucleated erythrocytes.

Treatment This varies according to the type of leukaemia and to the particular condition of the patient. Excellent results are being obtained in the control of ALL using blood transfusions, CHEMOTHERAPY, RADIOTHERAPY and bone-marrow TRANSPLANTATION. In the case of acute leukaemia, the drugs now being used include MERCAPTOPURINE, METHOTREXATE and CYCLOPHOSPHAMIDE. Blood transfusion and CORTICOSTEROIDS play an important part in controlling the condition during the period before a response to chemotherapy can be expected. Chemotherapy has almost completely replaced radiotherapy in the treatment of chronic leukaemia. For the myeloid form, BUSULFAN is the most widely used drug, replaced by hydroxyurea, mercaptopurine, or one of the nitrogen mustard (see NITROGEN MUSTARDS) derivatives in the later stages of the disease. For the lymphatic form, the drugs used are CHLORAMBUCIL, CYCLOPHOSPHAMIDE, and the nitrogen mustard derivatives.

Prognosis Although there is still no guaranteed cure, the outlook in both acute and chronic leukaemia has greatly improved – particularly for the acute form of the disease. Between 70 and 80 per cent of children with acute lymphoblastic leukaemia may be cured; between 20 and 50 per cent of those with acute myeloid leukaemia now have much-improved survival rates. Prognosis of patients with chronic lymphocytic leukaemia is often good, depending on early diagnosis.... leukaemia

This is the Latin word for wolf, and a term applied to certain chronic skin diseases which can destroy skin, underlying cartilage and even bone to cause serious deformity if uncontrolled.

Lupus vulgaris is a form of TUBERCULOSIS of the skin. It typically begins in childhood and may spread slowly for decades if untreated. The face and neck are the usual sites. In untreated disease, large, well-demarcated areas may be affected with redness, scaling and thickening. If the affected skin is blanched by pressure, yellow-brown foci may be observed – the so-called apple-jelly nodules. The disease causes extensive scarring as it spreads and may destroy cartilage in its path – for example, on the nose or ear – causing gross deformity. The disease was common in the UK up to 50 years ago, but is now rare. It is treated with a combination of tuberculostatic drugs.

Lupus erythematosus is an autoimmune disease which can affect skin or internal organs.

Discoid lupus erythematosus (DLE) In this disease, only the skin is affected. Sharply de?ned red, scaly and eventually atrophic patches appear on the face, especially on the nose and cheeks. ALOPECIA with scarring is seen if the scalp is affected. The condition is aggravated by sunlight. Topical CORTICOSTEROIDS are helpful.... lupus

a small ulcer, occurring singly or in groups in the mouth as white or red spots; their cause is unknown. Treatment is with topical tetracycline or topical corticosteroids.... aphthous ulcer

(BOOP) a disease entity characterized clinically by a flulike illness with cough, fever, shortness of breath, and late inspiratory crackles; there are specific histological features and patchy infiltrates on X-ray. It is sometimes the result of a viral infection, but may follow medication with certain drugs or be associated with connective-tissue disease, such as rheumatoid arthritis. The condition usually responds to oral corticosteroids; however, if a drug is implicated, it must be withdrawn.... bronchiolitis obliterans organizing pneumonia

(eosinophilic granulomatosis with polyangiitis) a systemic autoimmune *vasculitis comprising severe asthma, allergic rhinitis, and sinusitis associated with an increased *eosinophil count in the peripheral blood and eosinophilic deposits in the small vessels of the lungs. It usually responds to oral corticosteroids. [J. Churg (1910–2005) and L. Strauss (1913–85), US pathologists]... churg–strauss syndrome

Multiple sclerosis is a progressive disease of the BRAIN and SPINAL CORD, which, although slow in its onset, in time may produce marked symptoms such as PARALYSIS and tremors (see TREMOR), and may ultimately result in a severely disabled invalid. The disorder consists of hardened patches, from the size of a pin-head to that of a pea or larger, scattered here and there irregularly through the brain and spinal cord. Each patch is made up of a mass of the CONNECTIVE TISSUE (neuroglia), which should be present only in su?cient amount to bind the nerve-cells and ?bres together. In the earliest stage, the insulating sheaths (MYELIN) of the nerve-?bres in the hardened patches break up, are absorbed, and leave the nerve-?bres bare, the connective tissue being later formed between these.

Cause Although this is one of the most common diseases of the central nervous system in Europe – there are around 50,000 affected individuals in Britain alone – the cause is still not known. The disease comes on in young people (onset being rare after the age of 40), apparently without previous illness. The ratio of women-to-men victims is 3:2. It is more common in ?rst and second children than in those later in birth order, and in small rather than big families. There may be a hereditary factor for MS, which could be an autoimmune disorder: the body’s defence system attacks the myelin in the central nervous system as if it were a ‘foreign’ tissue.

Symptoms These depend greatly upon the part of the brain and cord affected by the sclerotic patches. Temporary paralysis of a limb, or of an eye muscle, causing double vision, and tremors upon exertion, ?rst in the affected parts, and later in all parts of the body, are early symptoms. Sti?ness of the lower limbs causing the toes to catch on small irregularities in the ground and trip the person in walking, is often an annoying symptom and one of the ?rst to be noticed. Great activity is shown in the re?ex movements obtained by striking the tendons and by stroking the soles of the feet. The latter re?ex shows a characteristic sign (Babinski sign) in which the great toe bends upwards and the other toes spread apart as the sole is stroked, instead of the toes collectively bending downwards as in the normal person. Tremor of the eye movements (nystagmus) is usually found. Trembling handwriting, interference with the functions of the bladder, giddiness, and a peculiar ‘staccato’ or ‘scanning’ speech are common symptoms at a later stage. Numbness and tingling in the extremities occur commonly, particularly in the early stages of the disease. As the disease progresses, the paralyses, which were transitory at ?rst, now become con?rmed, often with great rigidity in the limbs. In many patients the disease progresses very slowly.

People with multiple sclerosis, and their relatives, can obtain help and guidance from the Multiple Sclerosis Society. Another helpful organisation is the Multiple Sclerosis Resources Centre. Those with sexual or marital problems arising out of the illness can obtain information from SPOD (Association to Aid the Sexual and Personal Relationships of People with a Disability). (See APPENDIX 2: ADDRESSES: SOURCES OF INFORMATION, ADVICE, SUPPORT AND SELFHELP.)

Treatment is di?cult, because the most that can be done is to lead a life as free from strain as possible, to check the progress of the disease. The use of INTERFERON beta seems to slow the progress of MS and this drug is licensed for use in the UK for patients with relapsing, remitting MS over two years, provided they can walk unaided – a controversial restriction on this (expensive) treatment. CORTICOSTEROIDS may be of help to some patients.

The NATIONAL INSTITUTE FOR CLINICAL EXCELLENCE (NICE) ruled in 2001 that the use of the drugs interferon beta and glatiramer acetate for patients with multiple sclerosis was not cost-e?ective but recommended that the Department of Health, the National Assembly for Wales and the drug manufacturers should consider ways of making the drugs available in a cost-e?ective way. Subsequently the government said that it would consider funding a ‘risk-sharing’ scheme in which supply of drugs to patients would be funded only if treatment trials in individuals with MS showed that they were e?ective.

The Department of Health has asked NICE to assess two CANNABIS derivatives as possible treatments for multiple sclerosis and the relief of post-operative pain. Trials of an under-thetongue spray and a tablet could, if successsful, lead to the two drugs being available around 2005.

It is important to keep the nerves and muscles functioning, and therefore the patient should remain at work as long as he or she is capable of doing it, and in any case should exercise regularly.... multiple sclerosis (ms)

n. a local anaesthetic used in combination with corticosteroids in ointments and suppositories to relieve the pain of haemorrhoids.... cinchocaine

(corticoid) n. any steroid hormone synthesized by the adrenal cortex. There are two main groups of corticosteroids. The glucocorticoids (e.g. *cortisol, *cortisone, and corticosterone) are essential for the utilization of carbohydrate, fat, and protein by the body and for a normal response to stress. Naturally occurring and synthetic glucocorticoids have very powerful anti-inflammatory effects and are used to treat conditions that involve inflammation. The mineralocorticoids (e.g. *aldosterone) are necessary for the regulation of salt and water balance.... corticosteroid

n. an agent that soothes and softens the skin. Emollients are fats and oils, such as lanolin and liquid paraffin; they are used alone as moisturizers to lessen the need for active drug therapy (such as corticosteroids for eczema) and in skin preparations as a base for a more active drug, such as an antibiotic.... emollient

(eformoterol) n. a *sympathomimetic drug (a ?2 agonist) used, with inhaled corticosteroids (see also budesonide), as a long-acting *bronchodilator to control chronic asthma and chronic obstructive pulmonary disease. Side-effects include tremor, palpitations, and headache.... formoterol

(GPA) an autoimmune disease, formerly known as Wegener’s granulomatosis, predominantly affecting the sinuses, lungs, and kidneys, and characterized by blood-vessel inflammation and the formation of necrotizing *granulomas. It is associated with the presence of antineutrophil cytoplasmic antibodies (*ANCA). Untreated the disease is usually fatal, but it can be controlled with corticosteroids, cyclophosphamide, or rituximab.... granulomatosis with polyangiitis

Mumps, also known as epidemic parotitis, is an infectious disease characterised by in?ammatory swelling of the PAROTID GLAND and other SALIVARY GLANDS – often occurring as an EPIDEMIC and affecting mostly young people. Its name comes from the old verb, ‘mump’, meaning to mope or assume a disconsolate appearance – an apt description of the victim of the disease at its height.

Causes Mumps is due to infection with a virus and is highly infectious from person to person. It is predominantly a disease of childhood and early adult life, but it can occur at any age. Epidemics usually occur in the winter and spring. It is infectious for two or three days before the swelling of the glands appears. A vaccine is now available that gives a high degree of protection against the disease, the incidence of which is falling sharply. The vaccine is combined with those for MEASLES and RUBELLA – see MMR VACCINE; IMMUNISATION.

Symptoms There is an incubation period of 2–3 weeks after infection before the glands begin to swell. The gland ?rst affected is generally the parotid, situated in front of and below the ear. The swelling usually spreads to the submaxillary and sublingual glands lying beneath the jaw. The patient is feverish and the gland is tender. The swelling disappears after about ?ve days. In 15–30 per cent of males, in?ammation of the testicles (orchitis) develops. This usually occurs during the second week of the illness, but may not occur until 2–3 weeks later; it may result in partial ATROPHY of the testicles, but practically never in INFERTILITY. In a much smaller proportion of females with mumps, in?ammation of the OVARIES or BREASTS may occur. In?ammation of the PANCREAS, accompanied by tenderness in the upper part of the abdomen and digestive disturbances, sometimes results, and MENINGITIS is also an occasional complication. The various complications are found much more often when the disease affects adults than when it occurs in childhood.

Treatment There is no speci?c treatment but ANALGESICS and plenty of ?uid should be available. The child may need to be in bed for a few days and should not return to school until the symptoms have settled. Adults with orchitis may need strong painkillers, and CORTICOSTEROIDS may be required to reduce the painful swelling.... mumps

A serious disorder in which the chief symptoms are muscular weakness and a special tendency for fatigue to come on rapidly when e?orts are made. The prevalence is around 1 in 30,000. Two-thirds of the patients are women, in whom it develops in early adult life. In men it tends to develop later in life.

It is a classical example of an autoimmune disease (see AUTOIMMUNITY). The body develops ANTIBODIES which interfere with the working of the nerve endings in muscle that are acted on by ACETYLCHOLINE. It is acetylcholine that transmits the nerve impulses to muscles: if this transmission cannot be e?ected, as in myasthenia gravis, then the muscles are unable to contract. Not only the voluntary muscles, but those connected with the acts of swallowing, breathing, and the like, become progressively weaker. Rest and avoidance of undue exertion are necessary, and regular doses of neostigmine bromide, or pyridostigmine, at intervals enable the muscles to be used and in some cases have a curative e?ect. These drugs act by inhibiting the action of cholinesterase – an ENZYME produced in the body which destroys any excess of acetylcholine. In this way they increase the amount of available acetylcholine which compensates for the deleterious e?ect of antibodies on the nerve endings.

The THYMUS GLAND plays the major part in the cause of myasthenia gravis, possibly by being the source of the original acetylcholine receptors to which the antibodies are being formed. Thymectomy (removal of the thymus) is often used in the management of patients with myasthenia gravis. The incidence of remission following thymectomy increases with the number of years after the operation. Complete remission or substantial improvement can be expected in 80 per cent of patients.

The other important aspect in the management of patients with myasthenia gravis is IMMUNOSUPPRESSION. Drugs are now available that suppress antibody production and so reduce the concentration of antibodies to the acetylcholine receptor. The problem is that they not only suppress abnormal antibody production, but also suppress normal antibody production. The main groups of immunosuppressive drugs used in myasthenia gravis are the CORTICOSTEROIDS and AZATHIOPRINE. Improvement following steroids may take several weeks and an initial deterioration is often found during the ?rst week or ten days of treatment. Azathioprine is also e?ective in producing clinical improvement and reducing the antibodies to acetylcholine receptors. These effects occur more slowly than with steroids, and the mean time for an azathioprine remission is nine months.

The Myasthenia Gravis Association, which provides advice and help to sufferers, was created and is supported by myasthenics, their families and friends.... myasthenia gravis

n. a substance that is produced in one part of the body (by an *endocrine gland, such as the thyroid, adrenal, or pituitary), passes into the bloodstream and is carried to other (distant) organs or tissues, where it acts to modify their structure or function. Examples of hormones are corticosteroids (from the adrenal cortex), adrenaline (from the adrenal medulla), androgens and oestrogens (from the testis and ovary, respectively), thyroid hormone (from the thyroid gland), and insulin (from the islets of Langerhans). The *pituitary gland secretes a variety of hormones, whose release is controlled by specific releasing hormones.... hormone

(ITP) an *autoimmune disease in which platelets are destroyed, leading to spontaneous bruising (see purpura). Acute ITP is a relatively mild disease of children, who usually recover without treatment. A chronic form of the disease, typically affecting adults, is more serious, requiring treatment with corticosteroids or, if there is no response, with splenectomy. If both fail, immunosuppressant drugs may be effective. Platelet concentrates are used for life-threatening bleeding.... idiopathic thrombocytopenic purpura

n. a *monoclonal antibody that inhibits the activity of *tumour necrosis factor (TNF-?; see cytokine inhibitor). It is used to treat severe Crohn’s disease and ulcerative colitis, and rheumatoid arthritis, ankylosing spondylitis, and psoriatic arthritis that have failed to respond to treatment with corticosteroids or immunosuppressants and antirheumatic drugs, respectively. Side-effects include intestinal upset, flushing, and allergic reactions.... infliximab

Certain skin diseases – particularly CHILBLAIN, ACNE, LUPUS and ERYSIPELAS – tend to affect the NOSE, and may be very annoying. Redness of the skin may be caused by poor circulation in cold weather.

Acute in?ammation is generally the result of a viral infection (see COLD, COMMON) affecting the mucous membrane and paranasal sinuses (see SINUSITIS); less commonly it results from the inhalation of irritant gases. Boils may develop just inside the entrance to the nose, causing pain; these are potentially troublesome as infection can spread to the sinuses. HAY FEVER is one distressing form of acute rhinitis.

Malformations are of various kinds. Racial and familial variations in the external nose occur and may be a reason for RHINOPLASTY. Di?erences in the size and shape of the nose occur, often forming the starting point for chronic in?ammation of the nose, perennial rhinitis (all the year round), hay fever, or ASTHMA. More commonly, obstruction results from nasal polyps or adenoids, leading to inhalation through the mouth. Adenoids are an overgrowth of glandular tissue at the back of the throat, into which the nose opens. Polyps are growths of soft jelly-like character: they arise from chronic in?ammation associated with allergic rhinitis, chronic sinusitis, asthma, and aspirin abuse. Large polyps can cause erosion of the nasal bones and should be surgically removed.

Bleeding (see HAEMORRHAGE).

Foreign bodies At ?rst these may not cause any symptoms, but in time they can cause obstruction of the affected nostril with a foul-smelling bloody discharge. The problem is common with small children who tend to push small objects into their noses. Foreign bodies require removal, sometimes in hospital. Anyone attempting to remove a foreign body should take care not to push it further into the nose.

Loss of sense of smell, or anosmia, may be temporary or permanent. Temporary anosmia is caused by conditions of the nose which are reversible, whereas permanent

anosmia is caused by conditions which destroy the OLFACTORY NERVES. Temporary conditions are those such as the common cold, or other in?ammatory conditions of the nasal mucosa or the presence of nasal polyps (see above). Permanent anosmia may follow in?uenzal NEURITIS or it may also follow injuries to the brain and fractures of the skull involving the olfactory nerves.

Injury to nose The commonest injury is a fracture of the nasal bones or displacement of the cartilage that forms the bridge of the nose. The nasal SEPTUM may also be displaced sideways by a lateral blow. Sporting activities, especially boxing and rugby football, are commonly a cause of nasal injury. If a fracture is suspected, or if there is substantial tissue swelling, an X-ray examination is necessary. Resetting a damaged bone should be done either immediately, before swelling makes surgery di?cult, or ten days or so later when the swelling has subsided. Results are usually good, ensuring a clear airway as well as a restored pro?le. It is not unusual for the cheek-bone to sustain a depressed fracture at the same time as the nose is broken. Careful assessment and prompt surgery are called for. (For more information on fractures, see under BONE, DISORDERS OF).

Rhinitis In?ammation of the MUCOUS MEMBRANE lining the nose. Symptoms include nasal discharge and obstruction, sneezing and sometimes pain in the sinuses. There are several types of rhinitis:

•Allergic – due to allergy to dust, pollen or other airborne particles. Also called hay fever, allergic rhinitis causes a runny nose, sneezing and local congestion. It affects up to 10 per cent of the population and is more common in people suffering from other allergic disorders such as asthma or eczema (see DERMATITIS). Skin tests help to identify the causative ALLERGEN which the sufferer can then try to avoid, although in the case of pollen this is di?cult. Decongestant drugs, ANTIHISTAMINE DRUGS, and CORTICOSTEROIDS may help, as can SODIUM CROMOGLYCATE inhaled regularly during the pollen season. A desensitisation course to a particular allergen sometimes provides long-term relief.

Atrophic rhinitis is caused by a deterioration in the nasal mucous membrane as a result of chronic bacterial infection, nasal surgery or AGEING. Symptoms include persistent nasal infection and discharge and loss of sense of smell. ANTIBIOTICS and, in some cases, OESTROGENS alleviate the symptoms.

Hypertrophic rhinitis results from repeated nasal infection, and is characterised by thickened nasal membranes and congestion of the nasal veins. Removal of thickened mucosa may help severe cases.

Vasomotor rhinitis occurs when the mucosa becomes oversensitive to stimuli such as pollutants, temperature changes or certain foods or medicines. It may occur as a result of emotional disturbances and is common in pregnancy.

Viral rhinitis occurs as a result of infection by the common cold virus; treatment is symptomatic. Sinusitis is sometimes a complication.... nose, disorders of

n. an overgrowth of fibrous scar tissue following trauma to the skin. It does not resolve spontaneously but may be flattened by silicone gels, applied pressure, injections of potent corticosteroids, or intralesional excision (or combinations of these). Keloid formation is particularly common at certain sites, such as the breastbone or ear lobe; surgical excision of benign (nonmalignant) lesions from such sites is therefore best avoided. See also scar.... keloid

an extremely itchy skin disease of unknown cause. Shiny flat-topped pink/purple spots may occur anywhere but are characteristically found on the inside of the wrists. It may take the form of a white lacy pattern in the mouth, which usually produces no symptoms. Trauma, such as a scratch, may induce a linear form of the disease (see Koebner phenomenon). Treatment with potent topical corticosteroids gives symptomatic relief. Tablet treatments may be necessary for generalized disease.... lichen planus

an acute form of *sarcoidosis characterized by fever, *erythema nodosum, enlarged lymph nodes near the inner border of the lungs, joint pain or inflammation, often involving the ankles, and *uveitis. Symptoms may resolve spontaneously after a few weeks or may need therapy with NSAIDs or low-dose corticosteroids. Recurrence may occur in a minority of patients. [S. Löfgren (1910–78), Swedish clinician]... löfgren’s syndrome

(MPA) an autoimmune disease characterized by inflammation of small blood vessels, leading to reduced kidney function and breathlessness. It is associated with the presence of antinuclear cytoplasmic antibodies (*ANCA) and can be treated with corticosteroids, cyclophosphamide, or rituximab.... microscopic polyangiitis

Autoimmune disease of the SKIN in which the cells of the epidermis lose their adhesion to each other, resulting in blister formation.

Pemphigus vulgaris is a serious form affecting skin and MUCOUS MEMBRANE. It affects young and middle-aged people with widespread blistering, erosion and crusting of the skin. Extensive involvement of the lips, mouth and throat interfere with nutrition. Untreated, it is eventually fatal, but the disease can now be controlled by large doses of oral CORTICOSTEROIDS and other immunosuppressive drugs. MORBIDITY from the adverse effects of steroids is a serious problem, but some patients are eventually cured.

Pemphigus foliaceus is seen in the elderly; the blistering is more super?cial in the epidermis. It may be very widespread, but is not life-threatening because mucous membranes are not affected. Topical corticosteroids will sometimes control the eruption, but in severe cases treatment is as for pemphigus vulgaris.

Pemphigoid is a variant where the blistering occurs because of separation of the epidermis and dermis. Mucosae are rarely affected and the disease affects mainly the arms and legs in the elderly. Treatment is as for pemphigus but smaller doses of corticosteroids usually su?ce.... pemphigus

An acute febrile illness, usually seen in children, which may include ARTHRALGIA, ARTHRITIS, CHOREA, carditis (see below) and rash (see ERUPTION). The illness has been shown to follow a beta-haemolytic streptococcal infection (see STREPTOCOCCUS).

Rheumatic fever is now extremely uncommon in developed countries, but remains common in developing areas. Diagnosis is based on the presence of two or more major manifestations – endocarditis (see under HEART, DISEASES OF), POLYARTHRITIS, chorea, ERYTHEMA marginatum, subcutaneous nodules – or one major and two or more minor ones – fever, arthralgia, previous attacks, raised ESR, raised white blood cell count, and ELECTROCARDIOGRAM (ECG) changes. Evidence of previous infection with streptococcus is also a criterion.

Clinical features Fever is high, with attacks of shivering or rigor. Joint pain and swelling (arthralgia) may affect the knee, ankle, wrist or shoulder and may migrate from one joint to another. TACHYCARDIA may indicate cardiac involvement. Subcutaneous nodules may occur, particularly over the back of the wrist or over the elbow or knee. Erythema marginatum is a red rash, looking like the outline of a map, characteristic of the condition.

Cardiac involvement includes PERICARDITIS, ENDOCARDITIS, and MYOCARDITIS. The main long-term complication is damage to the mitral and aortic valves (see HEART).

The chief neurological problem is chorea (St Vitus’s dance) which may develop after the acute symptoms have subsided.

Chronic rheumatic heart disease occurs subsequently in at least half of those who have had rheumatic fever with carditis. The heart valve usually involved is the mitral; less commonly the aortic, tricuspid and pulmonary. The lesions may take 10–20 years to develop in developed countries but sooner elsewhere. The heart valves progressively ?brose and ?brosis may also develop in the myocardium and pericardium. The outcome is either mitral stenosis or mitral regurgitation and the subsequent malfunction of this or other heart valves affected is chronic failure in the functioning of the heart. (see HEART, DISEASES OF).

Treatment Eradication of streptococcal infection is essential. Other features are treated symptomatically. PARACETAMOL may be preferred to ASPIRIN as an antipyretic in young children. One of the NON-STEROIDAL ANTIINFLAMMATORY DRUGS (NSAIDS) may bene?t the joint symptoms. CORTICOSTEROIDS may be indicated for more serious complications.

Patients who have developed cardiac-valve abnormalities require antibiotic prophylaxis during dental treatment and other procedures where bacteria may enter the bloodstream. Secondary cardiac problems may occur several decades later and require replacement of affected heart valves.... rheumatic fever

the most common form of *cutaneous T-cell lymphoma, with patches, plaques, and later nodules on the skin. It typically progresses very slowly and can be treated with topical corticosteroids, phototherapy, radiotherapy, or other agents.... mycosis fungoides

n. inflammation of the testis. This causes pain, redness, and swelling of the scrotum, and may be associated with inflammation of the epididymis (epididymo-orchitis). The condition may affect one or both testes; it is usually caused by infection spreading down the vas deferens but can develop in mumps. Mumps orchitis affecting both testes after puberty may result in reduced testicular size and abnormalities in semen analysis, although sterility is rare. Treatment of epididymo-orchitis is by local support and by administration of analgesics and antibiotics; mumps orchitis often responds to *corticosteroids.... orchitis

The following are described under their separate dictionary entries: FAECES; HAEMORRHOIDS; FISTULA; DIARRHOEA; CONSTIPATION.

Imperforate anus, or absence of the anus, may occur in newly born children, and the condition is relieved by operation.

Itching at the anal opening is common and can be troublesome. It may be due to slight abrasions, to piles, to the presence of threadworms (see ENTEROBIASIS), and/or to anal sex. The anal area should be bathed once or twice a day; clothing should be loose and smooth. Local application of soothing preparations containing mild astringents (bismuth subgallate, zinc oxide and hamamelis) and CORTICOSTEROIDS may provide symptomatic relief. Proprietary preparations contain lubricants, VASOCONSTRICTORS and mild ANTISEPTICS.

Pain on defaecation is commonly caused by a small ulcer or ?ssure, or by an engorged haemorrhoid (pile). Haemorrhoids may also cause an aching pain in the rectum. (See also PROCTALGIA.)

Abscess in the cellular tissue at the side of the rectum – known from its position as an ischio-rectal abscess – is fairly common and may produce a ?stula. Treatment is by ANTIBIOTICS and, if necessary, surgery to drain the abscess.

Prolapse or protrusion of the rectum is sometimes found in children, usually between the ages of six months and two years. This is generally a temporary disorder. Straining at defaecation by adults can cause the lining of the rectum to protrude outside the anus, resulting in discomfort, discharge and bleeding. Treatment of the underlying constipation is essential as well as local symptomatic measures (see above). Haemorrhoids sometimes prolapse. If a return to normal bowel habits with the production of soft faeces fails to restore the rectum to normal, surgery to remove the haemorrhoids may be necessary. If prolapse of the rectum recurs, despite a return to normal bowel habits, surgery may be required to rectify it.

Tumours of small size situated on the skin near the opening of the bowel, and consisting of nodules, tags of skin, or cauli?ower-like excrescences, are common, and may give rise to pain, itching and watery discharges. These are easily removed if necessary. Polypi (see POLYPUS) occasionally develop within the rectum, and may give rise to no pain, although they may cause frequent discharges of blood. Like polypi elsewhere, they may often be removed by a minor operation. (See also POLYPOSIS.)

Cancer of the rectum and colon is the commonest malignancy in the gastrointestinal tract: around 17,000 people a year die from these conditions in the United Kingdom. Rectal cancer is more common in men than in women; colonic cancer is more common in women. Rectal cancer is a disease of later life, seldom affecting young people, and its appearance is generally insidious. The tumour begins commonly in the mucous membrane, its structure resembling that of the glands with which the membrane is furnished, and it quickly in?ltrates the other coats of the intestine and then invades neighbouring organs. Secondary growths in most cases occur soon in the lymphatic glands within the abdomen and in the liver. The symptoms appear gradually and consist of diarrhoea, alternating with attacks of constipation, and, later on, discharges of blood or blood-stained ?uid from the bowels, together with weight loss and weakness. A growth can be well advanced before it causes much disturbance. Treatment is surgical and usually this consists of removal of the whole of the rectum and the distal two-thirds of the sigmoid colon, and the establishment of a COLOSTOMY. Depending upon the extent of the tumour, approximately 50 per cent of the patients who have this operation are alive and well after ?ve years. In some cases in which the growth occurs in the upper part of the rectum, it is now possible to remove the growth and preserve the anus so that the patient is saved the discomfort of having a colostomy. RADIOTHERAPY and CHEMOTHERAPY may also be necessary.... rectum, diseases of

An antibiotic derived from Streptomyces mediterranei, rifampicin is a key component of the treatment of TUBERCULOSIS. Like ISONIAZID, it should always be included unless there is a speci?c contraindication. It is also valuable in the treatment of BRUCELLOSIS, LEGIONNAIRE’S DISEASE, serious staphylococcal (see STAPHYLOCOCCUS) infections and LEPROSY. It is also given to contacts of certain forms of childhood MENINGITIS.

Rifampicin is given by mouth; during the ?rst two months it often causes transient disturbance of LIVER function, with raised concentrations of serum transaminases, but usually treatment need not be interrupted. In patients with pre-existing liver disease more severe toxicity may occur, and liver function should be carefully monitored both before starting and during rifampicin treatment. It induces hepatic enzymes which accelerate the metabolism of various drugs including ANTICOAGULANTS, SULPHONYLUREAS, PHENYTOIN SODIUM, CORTICOSTEROIDS and OESTROGENS. The e?ectiveness of oral contraceptives is reduced and alternative family-planning advice should be o?ered.

Rifampicin should be avoided during pregnancy and breast feeding, and extra caution should be applied if there is renal impairment, JAUNDICE or PORPHYRIAS. Adverse effects include gastrointestinal symptoms, in?uenza-like symptoms, collapse and SHOCK, haemolytic ANAEMIA, acute ?ushing and URTICARIA; body secretions may be coloured red.... rifampicin

(bullous pemphigoid) n. a chronic itchy blistering disorder most common in the elderly. The blisters most commonly occur on the limbs and may persist, unlike those of *pemphigus. Pemphigoid is an *autoimmune disease and responds to treatment with corticosteroids or immunosuppressant drugs. Ocular pemphigoid is a potentially blinding disease in which there is dryness, blistering, and scarring of the conjunctiva, leading to shortening of the *fornices due to adhesions (*symblepharon).... pemphigoid

n. inflammation of the lung that is confined to the walls of the air sacs (alveoli) and often caused by viruses or unknown agents. It may be acute and transient or chronic, leading to increasing respiratory disability. It does not respond to antibiotics but corticosteroids may be helpful. Compare pneumonia.... pneumonitis

n. a steroid synthesized in the adrenal glands, ovaries, and testes. Pregnenolone is an important intermediate product in steroid hormone synthesis and can – depending on the pathways followed – be converted to corticosteroids (glucocorticoids or mineralocorticoids), androgens, or oestrogens.... pregnenolone

an acute destructive ulcerating process of the skin, especially the legs. It may be associated with ulcerative *colitis or *Crohn’s disease or with *rheumatoid arthritis or other forms of arthritis affecting many joints. Treatment is with ciclosporin, high doses of corticosteroids, or other immunosuppressants.

pyogenic adj. causing the formation of pus. Pyogenic bacteria include Staphylococcus aureus, Streptococcus hemolyticus, and Neisseria gonorrhoeae.... pyoderma gangrenosum

n. (in transplantation) the destruction by immune mechanisms of a tissue grafted from another individual. Antibodies, complement, clotting factors, and platelets are involved in the failure of the graft to survive. *Allograft rejection is a vigorous response that can be modified by drugs (such as ciclosporin and corticosteroids) and antibodies against T cells; *xenograft rejection is an acute response that is at present beyond therapeutic control.... rejection

n. a *sympathomimetic drug: a long-acting beta agonist used, with inhaled corticosteroids, as a *bronchodilator to control chronic asthma and chronic obstructive pulmonary disease. Possible side-effects include palpitations, a paradoxical worsening of the condition, and a lowering of the blood potassium level.... salmeterol

1. see juvenile idiopathic arthritis. 2. (adult-onset Still’s disease) a systemic inflammatory disease characterized by a spiking fever, joint pain, and a salmon pink rash that comes and goes. Treatment includes NSAIDS, corticosteroids, or disease-modifying antirheumatic drugs. [Sir G. F. Still (1868–1941), British physician]... still’s disease

n. a medicinal preparation in solid form suitable for insertion into the rectum, vagina, or urethra. Rectal suppositories may contain simple lubricants (e.g. glycerin); drugs that act locally in the rectum or anus (e.g. corticosteroids, local anaesthetics); or drugs that are absorbed and act at other sites (e.g. analgesics, such as diclofenac). Vaginal suppositories are used in the treatment of some gynaecological disorders (see pessary). Urethral suppositories contain antibiotics, local anaesthetics (prior to a procedure), or medication used in the treatment of erectile dysfunction.... suppository

Common chronic in?ammation of the facial skin, this condition is seen in middle and late life. Redness, obvious dilatation of venules and crops of ACNE-like papules and pustules affect mainly the central forehead, cheeks, nose and chin. A keratoconjunctivitis (combined in?ammation of the cornea and conjunctiva of the EYE) may be associated. Subjects ?ush easily, especially after alcohol or hot drinks. Eventually the affected areas may become thickened and oedematous, and in men, proliferation of ?brous and sebaceous tissue may lead to gross thickening and enlargement of the nose (RHINOPHYMA).

Treatment Long-term, low-dose, oral tetracycline (see ANTIBIOTICS; TETRACYCLINES) is the treatment of choice. In mild cases, METRONIDAZOLE gel can be helpful. Potent topical CORTICOSTEROIDS are contraindicated and make rosacea worse.... rosacea

Aloe, aloe vera (Aloe vera).

Plant Part Used: Leaf, leaf gel.

Dominican Medicinal Uses: Leaf gel: applied topically for skin conditions: minor abrasions, burns, cuts, fungal infection, scrapes, sunburn, wound-healing; taken orally for common cold, flu-like symptoms, pulmonary infection.

Safety: Results of toxicity studies and published literature.

Contraindications: Internal use: pregnancy, lactation, children under 12 y, individuals with inflammatory intestinal disease.

Drug Interactions: Internal use: cardiac glycosides, antiarrhythmic drugs (potential potassium loss and intensified drug effect); thiazide diuretics, loop diuretics, licorice, corticosteroids (risk of potassium loss); antidiabetic drugs: (risk of hypoglycemia).

Clinical Data: Clinical: anesthetic, antiviral, burn-healing, wound-healing (leaf gel).

Laboratory & Preclinical Data: In vivo: antidiabetic, anti-inflammatory, antiulcer, chemomodulatory, hypothyroid, wound-healing (leaf pulp/gel).

In vitro: antileukemic, antimutagenic, antitumor, cytotoxic, enzyme inhibition (chemical constituents).

* See entry for Sábila in “Part 3: Dominican Medicinal Plant Profiles” of this book for more information, including references.... sábila