Congenital nephrogenic diabetes insipidus in a baby girl.

A 6-week-old girl with fever, hypernatraemia, dehydration, and polyuria failed to concentrate urine in response to exogenous vasopressin administration. There was no family history of nephrogenic diabetes insipidus. When she was 15 months old, the infusion of vasopressin did not produce an increase in urinary cyclic-AMP.

Acute renal failure in newborn infants.

The clinical course and follow-up of 14 neonates who developed acute renal failure are reported. Renal failure in these patients was secondary to major perinatal disorders, e.g., hyaline membrane disease, pneumonia, hemorrhage, or sepsis. Thirteen patients had hypoxia and nine were in shock when renal failure developed. Five patients died during the acute stage of renal failure. Of nine survivors, five patients sustained residual renal damage.

Neonatal renal papillary and cortical necrosis.

Renal papillary and cortical necrosis (RPCN) in newborn infants has been generally fatal. This report describes three survivors of neonatal RPCN who have been followed up from 23 to 57 months. The cause of RPCN was hemorrhage and asphyxia neonatorum in two patients and hemorrhage and shock in one. The diagnosis was confirmed in one patient by an excretory urogram at 3 weeks of age and in another by renal biopsy at 5 weeks of age. In the third, diagnosis was made retrospectively. Findings from subsequent examination showed that creatinine clearance was normal in one patient, slightly reduced in another, and moderately reduced in the third. Maximum ability to concentrate urine was decreased in all. Follow-up excretory urograms in all three patients showed dilated bizarre-appearing calices and renal cortical atrophy. These radiologic findings are similar to those found in segmental hypoplasia or chronic (atrophic) pyelonephritis.

Unusual manifestations of Henoch-Schönlein syndrome.

Henoch-Schönlein syndrome represents a diagnostic challenge when the abdominal and/or joint manifestations precede the cutaneous lesions. Laparotomy is often performed unnecessarily. We describe a patient whose abdominal symptoms antedated the appearance of cutaneous lesions by three months. Gastrointestinal blood loss, thrombocytosis, hypoproteinemia, and the roentgenographic appearance of the small intestine suggested the correct diagnosis. His course was further complicated by hypertension associated with elevated plasma renin levels without evidence of nephritis. Joint manifestations were mild and transient as were testicular pain and swelling.

Pseudohypoaldosteronism due to sweat gland dysfunction.

Pseudohypoaldosteronism is an uncommon disorder characterized by urinary sodium wasting and is attributed to a defect in distal renal tubular sodium handling with failure to respond to endogenous aldosterone. Sweat electrolyte values in other reported patients, when measured, have been normal. A 3.5-year-old girl developed repeated episodes of dehydration, hyponatremia, and hyperkalemia during the first 19 months of life. Serum sodium was as low as 113 mEq/liter and potassium as high as 11.1 mEq/liter. Her plasma and urinary aldosterone levels were persistently elevated (Figs. 1-4). Unlike patients with classic pseudohypoaldosteronism she demonstrated no urinary sodium wasting (Figs. 2 and 3). During episodes of hyponatremia and reduced sodium intake her urinary sodium was less than 5 mEq/liter. In addition, her sweat sodium concentration was consistently above 125 mEq/liter and salivary sodium concentration above 58 mEq/liter. Her chest x-ray, 72-hr fecal fat excretion, serum and urinary pancreatic amylase (amy-2) were normal, providing no evidence for cystic fibrosis. It is proposed that this patient represents a new variant of pseudohypoaldosteronism with excessive loss of sodium from the sweat and salivary glands instead of the kidneys.

Extracapillary proliferative glomerulonephritis in children.

The clinical course and renal pathology of 17 children with acute extracapillary proliferative glomerulonephritis is reported. Patients with systemic diseases associated with renal involvement were excluded. The onset followed streptococcal infection in ten; of these, four have died, one has been transplanted, and the remaining five have completely healed. The mean follow-up of the latter five patients was 32 months (range, 18 to 57 months). Of the other seven patients without evidence of precedeing streptococcal infection, two have died, two have been transplanted, and the remaining three all have hypertension, proteinuria, and reduced creatinine clearance. The mean follow-up of the latter three patients was 29 months (range, 14 to 38 months). The initial renal histopathologic changes and their progress in later renal biopsies is described. The role of various therapeutic agents is discussed. The prognosis in acute extracapillary proliferative glomerulonephritis following streptococcal infection appears to be better than in ones without preceding streptococcal infection.