Pseudohypoaldosteronism due to sweat gland dysfunction

Pediatr Res. 1976 Jul;10(7):677-82. doi: 10.1203/00006450-197607000-00009.

Abstract

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.

Publication types

  • Case Reports

MeSH terms

  • Aldosterone / metabolism*
  • Child, Preschool
  • Dehydration / etiology
  • Female
  • Humans
  • Hyperkalemia / etiology
  • Hyponatremia / etiology
  • Kidney / metabolism
  • Potassium / metabolism
  • Sodium / metabolism
  • Sweat / analysis
  • Sweat Gland Diseases / metabolism*

Substances

  • Aldosterone
  • Sodium
  • Potassium