IN THE BEDSIDE TEACHING of medical students and housestaff, one always finds most stimulating the challenge of the unusual problems which first demand clinical control of potentially lethal conditions and, because of unusual interest or importance, will then prompt investigation into their causes. The following cases describe such an experience.
Report of Cases
On Dec 23, 1962, a 7-month-old female baby weighing 8 kg was admitted to the St. Louis Children's Hospital with what clinically suggested very severe ketosis and acidosis, such as might be expected in diabetes. Acetone could be detected on the breath, and nitroprusside and ferric chloride tests of the urine indicated heavy ketonuria. The serum CO2 content was but 4.5 mEq/liter. The urine was free of sugar, however, and also of salicylate, and the blood sugar was quite normal—82 mg/100 ml. If not diabetes or salicylate intoxication, what then was the cause of