Rodriguez-Soriano J, Vallo A, Chouza M, Castillo G
Acta Paediatr Scand. 1975 Jul;64(4):671-4. doi: 10.1111/j.1651-2227.1975.tb03902.x.
A 9-year-old girl presented with tetralogy of Fallot and moderate metabolic acidosis. Despite a Blalock's fistula there was evidence of chronic hypoxia with cyanosis, clubbing of fingers and toes and very elevated blood hematocrit values. Renal acidification and bicarbonate titration demonstrated the existence of proximal renal tubular acidosis: renal bicarbonate threshold was low (18 mmoles/1) and normal urinary acidificaiton was present at subthreshold serum bicarbonate levels. Following corrective heart surgery, blood acid-base values and renal reabsorption of bicarbonate became normal. A causal relationship between extracellular fluid volume expansion dependent on the high hematocrit and proximal renal tubular acidosis is suggested.
一名9岁女孩患有法洛四联症并伴有中度代谢性酸中毒。尽管做了锁骨下动脉 - 肺动脉吻合术，但仍有慢性缺氧的证据，表现为发绀、手指和脚趾杵状指以及血细胞比容值极高。肾脏酸化和碳酸氢盐滴定显示存在近端肾小管酸中毒：肾脏碳酸氢盐阈值低（18毫摩尔/升），在低于阈值的血清碳酸氢盐水平时存在正常的尿液酸化。心脏矫正手术后，血液酸碱值和肾脏对碳酸氢盐的重吸收恢复正常。提示依赖于高血细胞比容的细胞外液容量扩张与近端肾小管酸中毒之间存在因果关系。
