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1. any disturbance of fat metabolism.
2. a group of conditions due to defective metabolism of fat, resulting in absence of subcutaneous fat; they may be congenital or acquired and partial or total. (See Atlas 2, Part F.)
congenital generalized lipodystrophy an autosomal recessive condition marked by the virtual absence of subcutaneous adipose tissue, large body size, splenomegaly, hirsutism, acanthosis nigricans, and reduced glucose tolerance in the presence of high insulin levels.
intestinal lipodystrophy former name for Whipple's disease.
partial lipodystrophy a condition seen mainly in females in the first decade of life, characterized by symmetrical loss of subcutaneous fat, usually beginning on the face and gradually extending to the chest, neck, back, and upper limbs; this gives the lower part of the body an apparent, and possibly real, adiposity of the buttocks and lower limbs. Some affected patients develop insulin-resistant diabetes mellitus, triglyceridemia, and renal disease.
progressive lipodystrophy progressive and symmetrical loss of subcutaneous fat from the parts above the pelvis, facial emaciation, and abnormal accumulation of fat about the thighs and buttocks.
total lipodystrophy an autosomal recessive disorder occurring mainly in females, characterized by a generalized loss of subcutaneous fat and extracutaneous adipose tissue, present at birth or appearing later in life, and associated with hepatomegaly with abdominal protuberance, hypoglycemia and insulin-resistant nonketotic diabetes, hyperlipemia, marked elevation of the basal metabolic rate, accelerated somatic growth, advanced bone age, acanthosis nigricans and hirsutism.
Whipple's diseaseIntestinal lipodystrophy, lipodystrophy GI disease A systemic malabsorption syndrome characterized by diffuse tissue infiltration with glycoprotein-rich macrophages and abundant bacillary forms Clinical ♂:♀ ratio, 10:1, accompanied by acropachyia digestiva, cutaneous hyperpigmentation, malabsorption, diarrhea, steatorrhea, arthritis, lymphadenopathy, endocarditis, fever, pleuritis CNS alterations-eg demyelination of posterior columns; bacilliform inclusions occur in the skin, nervous system, joints, heart, vessels, kidney, lung, serosal membranes, lymph nodes, spleen, liver Treatment Penicillin + streptomycin for 2 wks, then long-term tetracycline. Cf Pseudo-Whipple's disease.
Whipple's diseaseA rare MALABSORPTION disorder in which the VILLI of the intestine are stubby or absent and fatty masses occur in the bowel, the local lymph nodes and elsewhere in the body. Enormous numbers of MACROPHAGES containing foamy material are found in the intestine and elsewhere and with them many bacteria that can be identified by POLYMERASE CHAIN REACTION as Tropheryma whipplei . Symptoms are numerous and include arthritis, fever, loss of weight, abdominal pain, diarrhoea, ANAEMIA, abnormal skin colouring, PLEURISY, ENDOCARDITIS, OEDEMA and FINGER CLUBBING. The joint problems often precede the intestinal disorder by months or years. The condition responds well to very prolonged antibacterial treatment with co-trimoxazole. Also known as intestinal lipodystrophy. (George Hoyt Whipple, 1878–1976, American pathologist).
A disorder of impaired absorption of nutrients by the small intestine. Symptoms include diarrhea, abdominal pain, progressive weight loss, joint pain, swollen lymph nodes, abnormal skin pigmentation, anemia, and fever. The precise cause is unknown, but it is probably due to an unidentified bacterial infection.
Mentioned in: Small Intestine Biopsy