A congenital renal tubular defect.

Waring, Kajdi and Tappan (1945) described six cases of severe polyuria and polydipsia which showed no response to pitressin therapy. The onset was after birth and the main features were erratic fever, constipation, vomiting and hyper-electrolytaemia without acidosis. The urine was constantly of low specific gravity, and in spite of high fluid intakes it was difficult to maintain hydration. Pitressin had no good effect in doses up to a toxic level. A similar condition in seven members ofone family was reported by Williams (1946). In at least two the condition began in infancy, with one death. Moderately impaired kidney function was found in five adult cases, but not severely enough to cause marked ill-health. Dancis, Birmingham and Leslie (1948), in presenting a further case in a child of 61 months, demonstrated that the pyrexia coincided with periods of dehydration, that diuresis of a low specific gravity urine persisted in spite of complete withdrawal of fluids, and that the patient's urine contained a normal amount of anti-diuretic substance. They inferred from this that the kidney did not respond to antidiuretic hormone. We report here a further case of this rare condition in which there was associated retardation of growth and mental development.