Abstract | BACKGROUND: Urine pH during acidemia and urine PCO2 upon alkalization both may be useful to indicate H+ secretion from collecting ducts. The urine anion gap has been used to detect urinary NH4+ for differential diagnosis of hyperchloremic metabolic acidosis. We have previously demonstrated that the lack of normal H(+)-ATPase may underlie secretory defect distal renal tubular acidosis ( dRTA). In this study we evaluated the diagnostic value of the urine-blood (U-B) PCO2 in H(+)-ATPase defect dRTA, and compared it with that of urine pH and urine anion gap during acidemia. METHODS: In H(+)-ATPase defect dRTA, the diagnostic values of three urinary parameters were evaluated: (1) urine pH measured after acid (NH4Cl) loading; (2) urine-to-blood carbon dioxide tension gradient (U-B PCO2) during alkali (NaHCO3) loading; and (3) urine anion gap during acidemia. Seventeen patients were diagnosed as having H(+)-ATPase defect dRTA based on reduced urinary NH4+ and an absolute decrease in H(+)-ATPase immunostaining in intercalated cells on renal biopsy. Eight patients with non- dRTA renal disease served as control patients. RESULTS: Upon NaHCO3 loading, U-B PCO2 was < or =30 mm Hg in all 17 dRTA patients and >30 mm Hg in all 8 control patients. With NH4Cl loading, urine pH was >5.4 in 15 of 17 dRTA patients and < or =5.4 in 7 of 8 control patients, and the urine anion gap was >5 mmol/L in 13 of 17 dRTA patients and< or =5 mmol/L in 6 of 8 control patients. Therefore, the sensitivity and specificity of U-B PCO2 < or =30 mm Hg during NaHCO3 loading were both 100%, whereas those of urine pH >5.4 or urine anion gap >5 mmol/L during NH4Cl loading were below 90%. In control patients, the U-B PCO2 was found to be well correlated with the urinary NH4+ (r= 0.79, P < 0.05). CONCLUSION: The U-B PCO2 during NaHCO3 loading is an excellent diagnostic index of H(+)-ATPase defect dRTA.
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Authors | Sejoong Kim, Jay Wook Lee, Junghwan Park, Ki Young Na, Kwon Wook Joo, Curie Ahn, Suhnggwon Kim, Jung Sang Lee, Gheun-Ho Kim, Jin Kim, Jin Suk Han |
Journal | Kidney international
(Kidney Int)
Vol. 66
Issue 2
Pg. 761-7
(Aug 2004)
ISSN: 0085-2538 [Print] United States |
PMID | 15253731
(Publication Type: Clinical Trial, Journal Article, Research Support, Non-U.S. Gov't)
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Chemical References |
- Anions
- Ammonium Chloride
- Carbon Dioxide
- Sodium Bicarbonate
- Proton-Translocating ATPases
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Topics |
- Acidosis, Renal Tubular
(blood, diagnosis, pathology, urine)
- Adult
- Ammonium Chloride
- Anions
(metabolism)
- Carbon Dioxide
(blood, urine)
- Humans
- Hydrogen-Ion Concentration
- Kidney Cortex
(enzymology, pathology)
- Proton-Translocating ATPases
(deficiency, metabolism)
- Sodium Bicarbonate
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