The Mauriac syndrome.
An insulin-dependent diabetic was diagnosed at the age of 7 years. After two years of satisfactory control she began to have several bouts of hospitalization with hyperglycaemic ketoacidosis, and developed tender hepatomegaly, which persisted to age 11 years. With restabilisation of her diabetes, the liver regressed and she continued to maintain good health for another 1 1/2 years when she died suddenly while asleep. Post-mortem examination by the coroner revealed ascites in the abdomen, hepatomegaly and fatty metamorphosis of the liver. Her diabetes control required up to 2.3 i.u. insulin per kg body weight per day plus a 1,900 calorie diet. Her growth was well below the tenth percentile, weight for height (Harvard charts). This clinical picture of high insulin dosage, hepatomegaly, unstable diabetes and growth failure approximates to the Mauriac syndrome.
Morrison
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McKenzie
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D002648:Child; D003922:Diabetes Mellitus, Type 1 / Q000150:complications* / Q000188:drug therapy; D005260:Female; D006130:Growth Disorders / Q000209:etiology*; D006529:Hepatomegaly / Q000209:etiology*; D006801:Humans; D007328:Insulin / Q000627:therapeutic use
D007328:Insulin