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A 14-year-old boy presented with Type I diabetes mellitus and subsequently developed pancreatic exocrine insufficiency and systemic sclerosis (SSc). His diabetes had been diagnosed when he was about 5 years old, after the onset of ketoacidosis. Insulin treatment was provided from then until the time he was treated in our department. Exocrine pancreatic insufficiency, which occurred at age 9, was treated with pancreatic extracts. Cystic fibrosis was excluded after a chloride sweat test. The diagnosis of SSc was confirmed at age 14 on the basis of skin sclerosis, sclerodactyly and oesophageal and pulmonary involvement and then at age 18 by the occurrence of Raynaud's disease. Thus, this patient demonstrated a rare and previously unreported association of Type I diabetes mellitus and systemic scleroderma. Limited joint mobility and skin abnormalities are frequent in childhood diabetes mellitus but should not be misdiagnosed as systemic scleroderma.

50 children with a strong clinical suspicion of gastroesophageal reflux and 10 control patients were evaluated with gastroesophageal scintiscans. 500 muCi à 1 mCi of Tc sulfur colloid mixed the patients' routine milk or formula feeding was administered and serial images of the abdomen and thorax were obtained. A positive scintiscan was found in 35 patients (sensitivity 70%) and none of the controls (specificity 100%). A comparison of findings in 34 patients referred for both radiographic and radionuclide studies showed that barium studies were positive in 38% and radionuclide in 64.7%. We found this examination to be more sensitive that the standard barium radiography particularly in patients with respiratory symptoms. We concluded that the GE scintiscan is complementary to barium studies in the diagnosis of GE reflux. Pulmonary aspiration of gastric contents was detected in only a case of the 35 patients with documented GE reflux. A T 1/2 emptying gastric longer than 90 minutes (linear calculation) or 105 minutes (exponential calculation) is an indirect GE reflux test. This procedure is simple, safe more physiologic than other available examinations.

A case of oxalate lithiasis associated with Abetalipoproteinemia is reported. The excessive absorption of dietary oxalate in this patient with fat malabsorption occurs because unabsorbed fatty acids in the bowel lumen combine with calcium ions which would otherwise impair oxalate absorption by forming insoluble calcium oxalate crystals. The medical management include dietary fat restriction and supplements of the fat soluble vitamins A, E and K. The prognostic is poor particularly in the neurological and retinal component of this disease.