Original Article

J Korean Soc Pediatr Endocrinol. 1999; 4(1): 100-103.

A Case of Mauriac Syndrome.

Young Ho Kim, Kee Hyoung Lee, Kee Hwan Yoo, Young Sook Hong, Joo Won Lee, and Soon Kyum Kim

In 1930s, Mauriac described a syndrome in diabetic children consisting of stunted growth, hepatomegaly, and delayed puberty. This syndrome was related to poorly controlled diabetes of long duration. A 14-year-old girl, who had been diagnosed as insulin dependent diabetes mellitus three years ago but had not been well controlled for diabetes, visited to hospital because of short stature, delayed puberty, and visual disturbance. On physical exam her height was 146cm(<3 percentile) and her weight was 34kg(<3 percentile) and sexual maturation was delayed as Tanner stage I. The liver was 3FB palpable below the right subcostal margin. She had hyperglycemia and elevated plasma cortisol. She was diagnosed as Mauriac syndrome. On ophthalmologic examination, the cataracts were observed on both eyes and she had also peripheral neuropathy on lower extremities. Her blood glucose was controlled strictly by regular insulin during admission, and her weight was increased and hepatomegaly was improved at discharge, two months later. We report a case of Mauriac syndrome with a brief review and related literatures.

Keywords : Mauriac syndrome, Short stature, Hepatomegaly, Delayed puberty