Hyperkalemic hyperchloremic metabolic acidosis in sickle cell hemoglobinopathies

Daniel Batlle*, Kriengsak Itsarayoungyuen, Jose A L Arruda, Neil A. Kurtzman

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

85 Scopus citations


This report describes the occurrence of hyperkalemic hyperchloremic metabolic acidosis in six patients with sickle cell hemoglobinopathies. Three patients had sickle cell anemia, two had sickle cell trait and one had S-C disease. In all patients, decreased renal potassium excretion was demonstrated by the finding of a fractional potassium excretion lower than that of control subjects with comparable glomerular filtration rates. Two patterns of impaired urinary acidification were discerned. Four patients had a urinary pH above 5.5 in the presence of systemic acidosis and, thus, were classified as having distal renal tubular acidosis. The remaining two patients had very low rates of ammonium excretion despite intact capacity to lower urinary pH below 5.5 during systemic acidosis; this pattern was ascribed to selective aldosterone deficiency. Sickle cell hemoglobinopathies should be included in the differential diagnosis of hyperkalemic hyperchloremic metabolic acidosis.

Original languageEnglish (US)
Pages (from-to)188-192
Number of pages5
JournalThe American journal of medicine
Issue number2
StatePublished - Feb 1982

ASJC Scopus subject areas

  • General Medicine


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