haemochromatosis


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he·mo·chro·ma·to·sis

(hē'mō-krō-mă-tō'sis)
A disorder of iron metabolism characterized by excessive absorption of ingested iron, saturation of iron-binding protein, and deposition of hemosiderin in tissue, particularly in the liver, pancreas, and skin; cirrhosis, diabetes mellitus (bronze diabetes), bronze pigmentation of the skin, and, eventually heart failure may occur; also can result from administration of large amounts of iron orally, by injection, or in forms of blood transfusion therapy.
Synonym(s): haemochromatosis.
[hemo- + G. chrōma, color, + -osis, condition]

haemochromatosis

A rare genetic disease featuring abnormal absorption and retention of iron. Total body iron may rise from the normal 4 or 5 g to as much as 60 g and the stored iron may cause CIRRHOSIS of the liver, DIABETES, IMPOTENCE, HEART FAILURE and bronzing of the skin. Treatment is by regular weekly bleeding until the levels of serum iron reach normal. The drug desferrioxamine is also useful.

he·mo·chro·ma·to·sis

(hē'mō-krō-mă-tō'sis) [MIM*235200]
Disorder of iron metabolism characterized by excessive absorption of ingested iron, saturation of iron-binding protein, and deposition of hemosiderin in tissue, particularly in the liver, pancreas, and skin; cirrhosis, diabetes (bronze diabetes), bronze pigmentation of the skin, and, eventually heart failure may occur; can also result from administration of large amounts of iron orally, by injection, or in forms of blood transfusion therapy.
Synonym(s): haemochromatosis.
[hemo- + G. chrōma, color, + -osis, condition]
References in periodicals archive ?
There's currently no cure for haemochromatosis, but there are treatments that can reduce the amount of iron in the body.
Krabbe, "Hereditary haemochromatosis: a case of iron accumulation in the basal ganglia associated with a parkinsonian syndrome," Journal of Neurology, Neurosurgery and Psychiatry, vol.
Feingold, "Idiopathic haemochromatosis: an autosomal recessive disease," Clinical Genetics, vol.
Dooley EASL clinical practice guidelines for HFE haemochromatosis. European Association for the Study of the Liver.
Excessive alcohol intake, old age, fatty liver, drugs, metabolic disorders such as diabetes and certain other diseases such as hepatitis B virus, hepatitis C virus, biliary diseases or haemochromatosis, may cause hepatic fibrosis.
Rheumatic manifestationsof haemochromatosis. Baillieres Clin Rheumatol 1991; 5:351-65.
Liver histology can show steatosis, portal and lobular inflammation, hepatic granulomas, centrilobular necrosis, microabscesses, haemochromatosis, cholestasis, nonspecific reactive changes, and infrequent cirrhosis [4].
Proton pump inhibitors suppress absorption of dietary non-haem iron in hereditary haemochromatosis. Gut 2007;56:1291-1295.
Low molecular weight iron complexes and oxygen radical reactions in idiopathic haemochromatosis. Clin Sci 1985; 68:463-67.
Familial haemochromatosis, as the prototype, may have a prolonged subclinical phase before presenting with dermatological, hepatic, pancreatic, cardiac, musculoskeletal or endocrine symptoms and signs.
[3] Kulaksiz, Gehrke, Janetzko et al., "Pro-hepcidin: expression and cell specific localisation in the liver and its regulation in hereditary haemochromatosis, chronic renal insufficiency, and renal anaemia," Gut, vol.
Hereditary haemochromatosis is a genetic multi-organ disorder associated with a polymorphism in the haemochromatosis gene (HFE) (Pietrangelo 2010).