Endocrine Abstracts

We describe the symptoms and review the laboratory findings in a case of patient with Mauriac syndrome.

A 19 years old Type 1 diabetic male with past history of multiple admissions to intensive therapy unit (ITU) with diabetic ketoacidosis (DKA) in a period of 3 years was admitted to ITU with DKA. On his examination protuberant abdomen with hepatomegaly, cushingoid feature, normal BMI and growth impairment were seen. His sexual development was normal. He had background diabetic retinopathy. On his biochemistry, plasma cholesterol and triglyceride were 35.7 and 53.8 mmol/l respectively. Other tests showed Hb 11.2 g/dl, HbA1c 10.9% and IGF-1 87 μg/l. His renal function was normal. Liver function test showed ALP 238 IU/l, ALT 239 IU/l and total bilirubin 8 μmol/l.

Mauriac syndrome may present in poorly controlled Type 1 diabetic adolescents and young adults. It is characterized by growth failure, hepatomegaly and cushingoid features. In current patient, severely impaired plasma lipid profile was observed which has not been reported in previous cases.

Although Mauriac syndrome is rare, it should be still considered in Type 1 diabetic children with growth impairment and liver disease. Optimal glycaemic control may play an important role in preventing such an occurrence.