Idiopathic Thrombocytopenic Purpura (itp): From 1 Different Sources
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.
No known cause.... idiopathic
A skin rash caused by bleeding into the skin from capillary blood vessels. The discrete purple spots of the rash are called purpuric spots or, if very small, petechiae. The disorder may be caused by capillary defects (nonthrombocytopenic purpura) or be due to a de?ciency of PLATELETS in the blood (thrombocytopenic purpura). Most worryingly, the rash may be due to a fulminant form of meningococcal SEPTICAEMIA called purpura fulminans. (See also HENOCH-SCHÖNLEIN PURPURA; IDIOPATHIC THROMBOCYTOPENIC PURPURA (ITP); THROMBOCYTOPENIA.).... purpura
See HENOCH-SCHÖNLEIN PURPURA.... anaphylactoid purpura
This is an in?ammatory condition of the small blood vessels, the cause of which is not known but may be an allergic response to food or drugs. Most common among young children, the in?ammation causes blood to leak into joints, kidneys, intestine and skin. The child presents with a purpuric rash and stomach pains which may come and go for weeks. Paracetamol alone is often su?cient to alleviate the condition, but severely ill patients may need corticosteroid drugs. All sufferers need follow-up for 12 months to ensure that they have not developed kidney disease.... henoch-schönlein purpura
See BELL’S PALSY.... idiopathic facial nerve palsy
Previously called juvenile rheumatoid arthritis and juvenile chronic arthritis, this is a set of related conditions of unknown cause affecting children. Characteristically, the synovial membrane of a joint or joints becomes in?amed and swollen for at leat six weeks (and often very much longer – even years). About 1 in 10,000 children develop it each year, many of whom have certain HLA genetic markers, thought to be important in determining who gets the illness. In?ammatory CYTOKINES play a big part.
Clinical features There are various types. The oligoarthritic type involves 1–4 joints (usually knee or ankle) which become hot, swollen and painful. One complication is an in?ammation of the eyes – UVEITIS. The condition often ‘burns out’, but may reappear at any time, even years later.
The polyarthritic type is more like RHEUMATOID ARTHRITIS in adults, and the child may have persistent symptoms leading to major joint deformity and crippling.
The systemic type, previously called Still’s disease, presents with a high fever and rash, enlarged liver, spleen and lymph nodes, and arthritis – although the latter may be mild. In some children the illness becomes recurrent; in others it dies down only to return as polyarthritis.
Complications These include uveitis, which can lead to loss of vision; a failure to thrive; osteoporosis (see under BONE, DISORDERS OF); joint deformity; and psychosocial diffculties.
Treatment This includes ANTIPYRETICS and ANALGESICS, including NON-STEROIDAL ANTIINFLAMMATORY DRUGS (NSAIDS), intra-articular steroid injections, anti-tumour necrosis factor drugs and steroids.
Physiotherapy is vital, and children may need to wear splints or other orthotic devices to alleviate deformity and pain. Orthopaedic operative procedures may be necessary.... juvenile idiopathic arthritis (jia)
See THROMBOCYTOPENIA.... thrombocytopenic
(ITP) An autoimmune disorder in which platelets are destroyed, leading to bleeding beneath the skin (see purpura).... idiopathic thrombocytopaenic purpura
see persistent idiopathic facial pain.... chronic idiopathic facial pain
(Schönlein–Henoch purpura, anaphylactoid purpura) a common, and frequently recurrent, form of *purpura found especially (but not exclusively) in young children. It is characterized by red weals and a purple rash on the buttocks and lower legs due to bleeding into the skin from inflamed capillaries, together with arthritis, gastrointestinal symptoms, and (in some cases) nephritis. Glucocorticoids are often used for treatment. [E. H. Henoch (1820–1910), German paediatrician; J. L. Schönlein (1793–1864), German physician]... henoch–schönlein purpura
(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
(atypical facial pain, chronic idiopathic facial pain) a chronic pain in the face that has no known cause and does not fit the classic presentation of other cranial neuralgias. It may be stress-related, and in some cases appears to be associated with defective metabolism of *tyramine. Treatment may involve the use of antidepressants.... persistent idiopathic facial pain
(benign intracranial hypertension, pseudotumour cerebri) a syndrome of raised pressure within the skull in the absence of a clear structural cause, such as a tumour. Although the cause is not certain, proposed mechanisms include impaired reabsorption of cerebrospinal fluid or venous outflow from the brain. The symptoms include headache, vomiting, double vision, and *papilloedema. The diagnosis is made by finding a high opening pressure at *lumbar puncture in the absence of a causative structural abnormality on brain imaging. It can improve spontaneously but drug therapy or neurosurgical treatment may be required to protect the patient’s vision.... idiopathic intracranial hypertension
(IPF) a serious interstitial lung disease, formerly called cryptogenic fibrosing alveolitis (see alveolitis). It is characterized by progressive fibrous scarring of the lung and increased numbers of inflammatory cells in the alveoli and surrounding tissues. The disease is usually diagnosed on clinical grounds on a basis of worsening breathlessness, inspiratory crackles at the lung bases on auscultation, clubbing of the fingers or toes, bilateral radiographic shadowing predominantly in the lower zones of the chest X-ray, subpleural *honeycomb change on CT scanning of the chest, and restrictive lung function on spirometry. It is also called usual interstitial pneumonia (UIP; see interstitial pneumonia), a term used by lung pathologists for the most common cellular pattern seen on biopsy. Treatment includes *pirfenidone, corticosteroids, and immunosuppressants.... idiopathic pulmonary fibrosis
(JIA, Still’s disease) any one of a group of conditions characterized by inflammation of the joints lasting longer than 6 weeks and occurring before the age of 16. The causes are unknown but immunological and infective mechanisms are suspected. JIA can affect either four or fewer joints (pauciarticular JIA) or more than four (polyarticular JIA). There are two recognized types of pauciarticular JIA: type 1, which generally affects girls below the age of four; and type 2 (juvenile-onset spondylarthropathy), which generally affects boys over the age of nine. There are also two types of polyarticular JIA, depending on the presence or absence of a particular antibody in the blood. There is a great range of severity of these diseases. Treatment consists of pain management and prevention of subsequent deformity or limitation of movement (e.g. contractures). Long-term joint damage is prevented by use of medications similar to those used in rheumatoid arthritis.... juvenile idiopathic arthritis
see Gardner–Diamond syndrome.... psychogenic purpura
see Henoch–Schönlein purpura.... schönlein–henoch purpura
(TTP) a rare disorder of coagulation caused by deficiency or inhibition of *ADAMTS13, a protein that is responsible for breaking down von Willebrand factor (see von Willebrand’s disease). This results in haemolytic *anaemia, *thrombocytopenia, and fluctuating neurological abnormalities. It is treated by *plasmapheresis.... thrombotic thrombocytopenic purpura
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