Renal proximal tubular dysfunction and paroxysmal nocturnal hemoglobinuria. Am J Med 1977 Jan;62(1):125-9
Date
01/01/1977Pubmed ID
13653DOI
10.1016/0002-9343(77)90357-6Scopus ID
2-s2.0-0017333026 (requires institutional sign-in at Scopus site) 24 CitationsAbstract
A patient with paroxysmal nocturnal hemoglobinuria, who required many blood transfusions for hemolytic episodes, had a persistent hyperchloremic metabolic acidosis. Bicarbonate infusion demonstrated a large fractional excretion of bicarbonate (28.6 per cent at a plasma bicarbonate level of 23 meq/liter) which was consistent with proximal renal tubular acidosis. Generalized aminoaciduria and decreased tubular reabsorption of phosphate were also present. Marked deposition of iron in renal proximal tubules was associated with these functional abnormalities. We believe that, as systemic acidosis can promote hemolysis in patients with paroxysmal nocturnal hemoglobinuria, hemolysis can lead, by way of iron deposition in renal tubules, to further acidosis. This cycle should be interrupted with appropriate doses of bicarbonate.
Author List
Riley AL, Ryan LM, Roth DAMESH terms used to index this publication - Major topics in bold
Acidosis, Renal TubularAdult
Blood
Hemoglobinuria, Paroxysmal
Hemolysis
Humans
Hydrogen-Ion Concentration
Male
