A Case of Mauriac's syndrome

ABSTRACT

Mauriac's syndrome was described in the 1920s, as a triad of poorly controlled insulin dependent diabetes mellitus, profound growth retardation, and hepatomgaly. Following the wide availability of insulin and intensification of diabetic control, this entity has become quite rare. A 9-year-old female child was transferred to pediatric OPD of SNUCH because of hyperglycemia, short stature, and visual disturbance. Five years prior to admission, she was diagnosed as diabetes mellitus at hospital due to polyuria, enuresis and polydipsia. However, she had been managed with irregular insulin injection and 1u of NPH once a day because of poor economic state and poor diabetic education. Two years ago, her mother noticed she had grown little and she had complainted poor vision. Since 1 year prior to admission, abdominal pain, vomiting, and diarrhea were developed twice, but subsided spontaneously without specific medication. On physical examination at admission, her height was 102 cm(< 3 percentile) and her weight was 16 kg(3-10 percentile). She was short and obese. The liver was 3FB palpable below the right subcostal margin. Limitation of motion of MP and PIP joints of left middle finger and right fourth finger were observed. On ophthalmologic examination, the cataracts were observed on both eyes and diabetic retinopathy was absent. Diabetic nephropathy was confirmed by kidney biopsy due to proteinuria. The bone age was delayed as 6-year. She was consistent with Mauriac's syndrome. During admission, she and her mother recieved diabetic education, and she was managed by strict diabetic control with human insulin. 4 months after, at discharge, her growth accelerations of height and weight were observed. Limited joint mobility and cataract were improved. Proteinuria disappeared after persantin and captopril medications.