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Proximal Renal Tubular Acidosis in Methylmalonic Acidemia

 

作者: WolffJ. A.,   StromC.,   GriswoldW.,   SweetmanF.,   KulovichS.,   ProdanosC.,   NyhanW. L.,  

 

期刊: Journal of Neurogenetics  (Taylor Available online 1985)
卷期: Volume 2, issue 1  

页码: 31-39

 

ISSN:0167-7063

 

年代: 1985

 

DOI:10.3109/01677068509100141

 

出版商: Taylor&Francis

 

关键词: Key words:methylmalonic academia;proximal renal tubular acidosis;urinary pH;renal bicarbonate threshold

 

数据来源: Taylor

 

摘要:

A patient with methylmalonic acidemia was found to have a persistent hyperchloremic acidosis. Investigation documented the presence of a proximal renal tubular acidosis. Between 14 and 18 months of age the urinary pH was as high as 8.0 when the serum bicarbonate was 17 mEq/liter and the threshold for bicarbonate was at 16-17 mEq/liter. When restudied at 33 months of age, the threshold had risen to 20 mEq/liter, but this was still abnormal and supplemental treatment was required to keep the serum concentration of bicarbonate above 20 mEq/liter. It is postulated that organic acid metabolites which accumulate in this and related disorders may interfere with renal tubular function as has been shown for maleic acid in experimental animals.

 

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