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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