Quigley R
Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas, Texas, USA.
J Nephrol. 2006 Mar-Apr;19 Suppl 9:S41-5.
The proximal tubule reabsorbs approximately 80% of the filtered load of bicarbonate. Defects in the process of bicarbonate reabsorption result in loss of bicarbonate and proximal renal tubular acidosis. Global proximal tubule dysfunction is known as the Fanconi's syndrome. Both isolated proximal renal tubular acidosis and the Fanconi's syndrome can result from inherited defects or can be acquired. This review will discuss the mechanisms that cause defects in proximal tubule bicarbonate transport as well as the common causes of isolated proximal renal tubular acidosis and the Fanconi syndrome.
近端小管重吸收约80%经滤过的碳酸氢盐。碳酸氢盐重吸收过程中的缺陷会导致碳酸氢盐丢失和近端肾小管性酸中毒。全身性近端小管功能障碍称为范科尼综合征。孤立性近端肾小管性酸中毒和范科尼综合征都可能由遗传缺陷引起，也可能是后天获得的。本文将讨论导致近端小管碳酸氢盐转运缺陷的机制，以及孤立性近端肾小管性酸中毒和范科尼综合征的常见病因。
