| Mauriac Syndrome in a Patient with Type I Diabetes Mellitus |
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Ji Yeoun Kang, Pil Soon Yang, Hyung Su Kim, Ok Young Kim, Chul Hoi Koo, Wha Mo Lee |
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Department of Pediatrics, Pusan Medical Center, Pusan, Korea |
| 제 I형 당뇨병에 발생한 Mauriac 증후군 1례 |
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강지연, 양필순, 김형수, 김옥영, 구철회, 이화모 |
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부산의료원 소아과 |
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| Abstract |
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Mauriac syndrome consists of a triad of poorly controlled diabetes, profound growth retardation and hepatomegaly. We experienced a case of Mauriac syndrome in an 18-year-old girl who had poorly controlled diabetes mellitus, short stature, hepatomegaly and central obesity. Also at the time of examination, she had complications of diabetic cataract and nephropathy. Fourteen years prior to admission, she was diagnosed as diabetes mellitus at a hospital. Thereafter, she had been managed with irregular insulin injection. On physical examination at admission, her height was 135cm(<3 percentile) and her weight was 39kg(<3 percetile). She was short and obese. The liver was 5 cm palpable below the right subcostal margin. Her sexual maturation was Tanner stage I. On ophthalmologic examination, the cataracts were observed on both eyes and diabetic retinopathy was absent. Diabetic nephropathy could not be confirmed by kidney biopsy due to her mother`s refusal. We studied the hormonal, radiographic and histological abnormalities. The hormonal study was normal and the bone age was by delayed as much as 10 years. The liver biopsy revealed glycogen accumulation in hepatocyte. She was consistent with Mauriac syndrome.
She was managed by strict diabetic control with insulin therapy, diabetic diet and intensive education. She was discharged with well controlled blood glucose. Five months later, growth acceleration and sexual maturation have not been observed, but hepatomegaly subsided. |
| Key Words:
Mauriac syndrome |
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