Document Detail

Distal renal tubular acidosis and high urine carbon dioxide tension in a patient with southeast Asian ovalocytosis.
MedLine Citation:
PMID:  10352205     Owner:  NLM     Status:  MEDLINE    
Southeast Asian ovalocytosis (SAO) is the best-documented disease in which mutation in the anion exchanger-1 (AE1) causes decreased anion (chloride [Cl-]/bicarbonate [HCO3-]) transport. Because AE1 is also found in the basolateral membrane of type A intercalated cells of the kidney, distal renal tubular acidosis (dRTA) might develop if the function of AE1 is critical for the net excretion of acid. Studies were performed in a 33-year-old woman with SAO who presented with proximal muscle weakness, hypokalemia (potassium, 2.7 mmol/L), a normal anion gap type of metabolic acidosis (venous plasma pH, 7. 32; bicarbonate, 17 mmol/L; anion gap, 11 mEq/L), and a low rate of ammonium (NH4+) excretion in the face of metabolic acidosis (26 micromol/min). However, the capacity to produce NH4+ did not appear to be low because during a furosemide-induced diuresis, NH4+ excretion increased almost threefold to a near-normal value (75 micromol/L/min). Nevertheless, her minimum urine pH (6.3) did not decrease appreciably with this diuresis. The basis of the renal acidification defect was most likely a low distal H+ secretion rate, the result of an alkalinized type A intercalated cell in the distal nephron. Unexpectedly, when her urine pH increased to 7.7 after sodium bicarbonate administration, her urine minus blood carbon dioxide tension difference (U-B Pco2) was 27 mm Hg. We speculate that the increase in U-B Pco2 might arise from a misdirection of AE1 to the apical membrane of type A intercalated cells.
C Kaitwatcharachai; S Vasuvattakul; P t Yenchitsomanus; P Thuwajit; P Malasit; D Chuawatana; S Mingkum; M L Halperin; P Wilairat; S Nimmannit
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  American journal of kidney diseases : the official journal of the National Kidney Foundation     Volume:  33     ISSN:  1523-6838     ISO Abbreviation:  Am. J. Kidney Dis.     Publication Date:  1999 Jun 
Date Detail:
Created Date:  1999-06-22     Completed Date:  1999-06-22     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  8110075     Medline TA:  Am J Kidney Dis     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  1147-52     Citation Subset:  IM    
Renal and Medical Molecular Biology Units, Songklanakarin Hospital, Bangkok, Thailand.
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MeSH Terms
Acidosis, Renal Tubular / etiology*,  metabolism,  urine
Antiporters / genetics
Carbon Dioxide / urine*
Diuretics / administration & dosage
Elliptocytosis, Hereditary / complications*
Furosemide / administration & dosage
Hydrogen-Ion Concentration
Quaternary Ammonium Compounds / urine
Sodium Bicarbonate / administration & dosage
Reg. No./Substance:
0/Antiporters; 0/Diuretics; 0/Quaternary Ammonium Compounds; 124-38-9/Carbon Dioxide; 144-55-8/Sodium Bicarbonate; 54-31-9/Furosemide

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