DIABETES INSIPIDUS IN INFANCY RESISTANT TO PITRESSIN

[摘要] The present authors have presented the report of a 6½ month old Negro male who, shortly after birth, developed polyuria, polydipsia, fever, vomiting, dehydration, constipation and failure to gain weight.Investigations ruled out systemic infections as well as the common inflammatory and congenital renal diseases.Under "control" conditions (average fluid intake) there was a persistence of the fever, vomiting and constipation; the values for nonprotein nitrogen, serum sodium and chloride were elevated above normal. The polyuria, which was characterized by low specific gravity, rose to a value of 1.011 during periods of marked dehydration.During the "hydration test," a progressive increase of fluid intake above 2000 cc. daily resulted in normal temperature, absence of vomiting, increase of weight, and normal values for nonprotein nitrogen, serum sodium and chloride.There was no antidiuretic response either to intravenous infusion of hypertonic saline (Carter-Robbins' test) or to 0.1 unit pitressin intravenously. A normal control showed prompt antidiuresis during and following the infusion of hypertonic saline.Intramuscular administration of pitressin to the point of toxicity (4 units) had no effect on the volume or specific gravity of the urine.It is postulated that this syndrome of diabetes insipidus in infancy, resistant to pitressin, is the result of a failure of end-organ response in the kidney.

[学科分类] 儿科学