Arnold W C, Brewster M, Byrne W J, Booth B
Int J Pediatr Nephrol. 1986 Apr-Jun;7(2):95-8.
Fanconi syndrome with proximal renal tubular acidosis is caused by a variety of anatomic, functional and metabolic disorders. We report a patient with a variant of isovaleric acidosis who developed proximal tubular acidosis. This patient was able to acidify the urine during metabolic acidosis, developed a hyperchloremic metabolic acidosis, and needed 24 mEq/kg/day of bicarbonate to maintain normal serum bicarbonate. She had a FE Bicarbonate of 12 +/- 4% during bicarbonate infusion. Isovaleric acidosis may be another toxic cause of proximal RTA.
范科尼综合征伴近端肾小管性酸中毒由多种解剖、功能和代谢紊乱引起。我们报告了一名患有异戊酸血症变异型且发生近端肾小管性酸中毒的患者。该患者在代谢性酸中毒期间能够酸化尿液，出现了高氯性代谢性酸中毒，并且需要每天24 mEq/kg的碳酸氢盐来维持正常的血清碳酸氢盐水平。在输注碳酸氢盐期间，她的碳酸氢盐排泄分数为12±4%。异戊酸血症可能是近端肾小管性酸中毒的另一个中毒原因。
