Stunted growth and hepatomegaly in diabetes mellitus

Abstract

A 17-year-old girl was admitted for evaluation of short stature, delayed puberty, diabetes mellitus, and hepatomegaly. The patient was well until age 14 months when she presented with diabetic ketoacidosis. At five years of age, she was hospitalized twice for ketoacidosis with upper respiratory infections. She was noted to have hepatomegaly, but had normal liver function studies. The short stature had been recognized, but her parents were informed that subnormal growth was typical of children with diabetes. Because the family moved frequently, at least 12 different pediatricians were consulted. For the five years prior to our evaluation, the patient had been ireated With 12 units each of NPH and regular insulin daily. She and her parents claimed that mild ketonuria and increased glucosuria had been noted only over the past two months. physical examination showed (Fig. 1) a very small, younger appearing girl with a protuberant abdomen, weighing 31.5 kg with a height of 132 cm. Blood pressure measured 120/78 mm Hg; pulse rate was 84/minute. The fundi showed mild Venous dilatation but no other changes consistent with diabetic retinopathy. The thyroid, heart, and lungs were normal. The abdomen was distended by an enlarged smooth, nontender liver, 14 cm in vertical span. Pubertal development was at Tanner Stage II. Hair growth was increased on the extensor surfaces of all extremities, and the fourth and fifth fingers bilaterally had flexion contractures. Lipodystrophy was evident at the insulin injection sites. No neurologic deficit was apparent. The hemogram was normal. Glucose and ketones were present in the urine. The patient had normal serum electrolyte, creatinine, and blood urea nitrogen concentrations; blood glucose