Sep 1, 1976

Renal acidification in sickle-cell disease

The Journal of Laboratory and Clinical Medicine
J R OsterC A Vaamonde

Abstract

Renal acidification was evaluated in patients with sickle-cell disease (HvSS) with both oral NH4CI and NaHC03 and the results were compared to those of subjects with sickle-cell trait (HbAS) and controls. The pH of arterial blood was normal in HbSS subjects but their PC02 and [HC03] were lower than those of controls. In response to NH4CI, six of 20 HbSS subjects had an abnormal minimal urine pH (greater than 5.3) and the entire HbSS group had a higher mean value than did either controls or HbAS subjects. Since none of the six HbSS subjects had evidence of proximal tubular abnormalities, it was concluded that they exhibited the syndrome of incomplete distal renal tubular acidosis. Only one of the six HbSS volunteers with an abnormal response to NH4CI and two of seven with a normal response increased their urinary PC02 normally after bicarbonate loading. PAH clearance was significantly higher and inulin clearance tended to be higher in HbSS subjects than in either controls or HbAS subjects. Maximal concentrating ability was decreased in both sickle-cell groups but more so in HbSS. No adverse effects occurred and no appearance or increase in per cent of sickled cells resulted from short-duration NH4CI acid-loading. No differences ...Continue Reading

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Mentioned in this Paper

Trichohepatoenteric Syndrome
Kidney Function Tests
Renal Tubular Acidosis
Renal Tubular Acidosis, Type II
Distal Renal Tubular Acidosis
Drepanocyte (Cell)
Urine pH Measurement
Response to Acid
Carbonic Acid Ions
Acidification - ActCode

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