Adrenal Hyperplasia, Congenital: From 1 Different Sources
An uncommon genetic disorder in which an enzyme defect blocks the production of corticosteroid hormones from the adrenal glands. Excessive amounts of androgens (male sex hormones) are produced, which can result in abnormal genital development in an affected fetus. Other effects include dehydration, weight loss, low blood pressure, and hypoglycaemia. Hyperplasia (enlargement) of the adrenal glands occurs and there is excessive skin pigmentation in skin creases and around the nipples.
In severe cases, the disorder is apparent soon after birth. In milder cases, symptoms appear later, sometimes producing premature puberty in boys and delayed menstruation, hirsutism, and potential infertility in girls.
Congenital adrenal hyperplasia is confirmed by measuring corticosteroid hormones in blood and urine. Treatment is by hormone replacement. If this is started early, normal sexual development and fertility usually follow.
Congenital deformities, diseases, etc. are those which are either present at birth, or which, being transmitted direct from the parents, show themselves some time after birth.... congenital
See ADRENOGENITAL SYNDROME and GENETIC DISORDERS.... congenital adrenal hyperplasia
Hyperplasia means an abnormal increase in the number of cells in a tissue.... hyperplasia
(BPH) A medical term for enlargement of the prostate gland (see prostate, enlarged).... benign prostatic hyperplasia
The outer covering of the two adrenal glands that lie atop each kidney. Embryonically derived from gonad tissue, they make steroid hormones that control electrolytes, the management of fuels, the rate of anabolism, the general response to stress, and maintenance of nonspecific resistance.... adrenal cortex
The inner part of the adrenals, derived embryonically from spinal nerve precursors, they secrete epinephrine, norepinephrine and dopamine; used locally as neurotransmitters, sensitive receptors can be mobilized totally by the adrenal medullas.... adrenal medulla
(BPH) The benign buildup in the prostate of “warts” or epithelial neoplasias that can block or interrupt urination, and which are usually concurrent with moderate prostate enlargement. They cause a dull ache on urination, ejaculation, and/or defecation. The diagnosis is medical, since the same subjective conditions can result from cancer of the prostate. BPH is common in men over fifty and can be the result either of diminished production of complete testosterone or poor pelvic circulation. Alcohol, coffee, speed, and antihistamines can all aggravate the problem.... benign prostatic hypertrophy, or hyperplasia
An agent which stimulates the adrenal glands thereby increasing secretion of cortisol and adrenal hormones. A herb with a mild cortico-steroid effect. Liquorice. Ginseng. Sarsaparilla. ... adrenal-activator
Cancerous or noncancerous tumours in the adrenal glands, usually causing excess secretion of hormones. Adrenal tumours are rare. Tumours of the adrenal cortex may secrete aldosterone, causing primary aldosteronism, or hydrocortisone, causing Cushing’s syndrome. Tumours of the medulla may cause excess secretion of adrenaline and noradrenaline. Two types of tumour affect the medulla: phaeochromocytoma and neuroblastoma, which affects children. These tumours cause intermittent hypertension and sweating attacks. Surgical removal of a tumour usually cures these conditions.... adrenal tumours
See hyperplasia, gingival.... gingival hyperplasia
See developmental hip dysplasia.... hip, congenital dislocation of
(CDH) herniation of the fetal abdominal organs into the fetal chest, which occurs in one in 2000–5000 live births. This leads to pulmonary *hypoplasia, which is the main cause of the associated high neonatal mortality. The risk of pulmonary hypoplasia is substantially greater where there is herniation of the liver into the thoracic cavity. CDH is commonly associated with additional structural abnormalities (cardiac, neural tube defects, and exomphalos), and the risk of chromosomal abnormality (*aneuploidy) is 10–20%. Demonstration of a fluid-filled bowel at the level of the heart on ultrasound is diagnostic.... congenital diaphragmatic hernia
(CDH) an abnormality present at birth in which the head of the femur is displaced or easily displaceable from the acetabulum (socket) of the ilium, which is poorly developed; it frequently affects both hip joints. CDH occurs in about 1.5 per 1000 live births, being more common in first-born girls, in breech deliveries, and if there is a family history of the condition. The leg is shortened and has a reduced range of movement, and the skin creases may be asymmetrical. All babies are routinely screened for CDH at birth and at developmental check-ups by gentle manipulation of the hip causing it to be reduced and dislocated with a clunk (see Barlow manoeuvre; Ortolani manoeuvre). The diagnosis is confirmed by X-ray or ultrasound scan. Treatment is with a special harness holding the hip in the correct position. If this is unsuccessful, the hip is reduced under anaesthetic and held with a plaster of Paris cast or the defect is corrected by surgery. Successful treatment of an infant can give a normal hip; if the dislocation is not detected, the hip does not develop normally and osteoarthritis develops at a young age.... congenital dislocation of the hip
see transposition of the great vessels.... congenitally corrected transposition
a hereditary condition (inherited as an autosomal *recessive) causing severe visual loss in infants. The *fundus usually appears to be normal when examined with an *ophthalmoscope, but marked abnormalities are found on the ERG (see electroretinography), usually with extinguished wave pattern. [T. Leber (1840–1917), German ophthalmologist]... leber’s congenital amaurosis
a pathological process involved in *atherosclerosis of arteries and vein grafts, and in *restenosis, that may be in response to angioplasty and stent placement. Damage to the endothelium of the artery exposes the underlying smooth muscle cells in the *media to cytokines, growth factors, and other plasma components in the circulation, which results in loss of their contractile characteristics. These abnormal muscle cells migrate to the *intima, where they proliferate and eventually form a thick layer of tissue (neointima), which occludes the artery.... neointimal hyperplasia
an increase in the thickness of the cells of the *endometrium, usually due to prolonged exposure to unopposed oestrogen, which can be endogenous, as in anovular menstrual cycles; or exogenous, deriving, for example, from *hormone replacement therapy or an oestrogen-secreting tumour. It is classified as simple, complex, or atypical. Endometrial hyperplasia most commonly presents with abnormal uterine bleeding and accounts for 15% cases of postmenopausal bleeding. It may also be asymptomatic, and in some cases regresses spontaneously without ever being detected. The presence of atypical cells may lead to *endometrial cancer. Treatment can include progestogen therapy or surgery (see endometrial ablation); hysterectomy is advised when atypical changes are present.... endometrial hyperplasia
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