Abstract
A three-year-old boy presented with the transient signs of disturbed consciousness, cyanosis and arrhythmia and was found to have hyperkalemia (K+9.6), metabolic acidosis (pH 7.25, bicarbonate 17) and growth failure. Blood pressure was normal. Creatinine clearance, PSP excretion and urinary concentration were normal. Aldosterone level and plasma renin activity were also within normal ranges. Urinary acidification was normal, but the ability to excrete net acid appeared decreased. The renal threshold for bicarbonate was low. Administration of the sodium bicarbonate and acetazolamide showed poor response in reducing serum potassium, but serum potassium and bicarbonate returned to normal levels during the administration of a Thiazide diuretic. Hyperkalemia was also corrected by lasix, but serum bicarbonate remained unchanged. There were no significant weight changes during these treatments. When the diuretics were tapered or discontinued, hyperkalemia recurred while serum bicarbonate remained at normal levels. From these observations it appeared that this patient had impaired renal excretion of potassium. He has been on long-term Thiazide thereapy (1.3mg/kg) and his serum potassium and bicarbonate levels are kept within normal ranges, and he grew 9 cm in 18 months. Family studies revealed that his two-year-old sister had hyperkalemia (K+6.8) and metabolic acidosis (pH 7.29, bicarbonate 16) with her height at fifth percentile and she is being worked up. His parents and an older brother are normal.
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Litaka, K., Asakura, A., Watanabe, N. et al. CHRONIC HYPERKALEMIA, METABOLIC ACIDOSIS AND SHORT STATURE IN TWO SIBLINGS-THE SPITZER-WEINSTEIN SYNDROME. Pediatr Res 14, 977 (1980). https://doi.org/10.1203/00006450-198008000-00024
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DOI: https://doi.org/10.1203/00006450-198008000-00024