Batlle D, Itsarayoungyuen K, Arruda J A, Kurtzman N A
Am J Med. 1982 Feb;72(2):188-92. doi: 10.1016/0002-9343(82)90809-9.
This report describes the occurrence of hyperkalemic hyperchloremic metabolic acidosis in six patients with sickle cell hemoglobinopathies. Three patients had sickle cell anemia, two had sickle cell trait and one had S-C disease. In all patients, decreased renal potassium excretion was demonstrated by the finding of a fractional potassium excretion lower than that of control subjects with comparable glomerular filtration rates. Two patterns of impaired urinary acidification were discerned. Four patients had a urinary pH above 5.5 in the presence of systemic acidosis and, thus, were classified a having distal renal tubular acidosis. The remaining two patients had very low rates of ammonium excretion despite intact capacity to lower urinary pH below 5.5 during systemic acidosis; this pattern was ascribed to selective aldosterone deficiency. Sickle cell hemoglobinopathies should be included in the differential diagnosis of hyperkalemic hyperchloremic metabolic acidosis.
本报告描述了6例镰状细胞血红蛋白病患者发生高钾性高氯性代谢性酸中毒的情况。3例患者患有镰状细胞贫血，2例有镰状细胞性状，1例患有S-C病。在所有患者中，通过发现钾排泄分数低于肾小球滤过率相当的对照受试者，证实肾钾排泄减少。发现了两种尿酸化受损模式。4例患者在全身性酸中毒时尿pH值高于5.5，因此被归类为远端肾小管酸中毒。其余2例患者尽管在全身性酸中毒期间有能力将尿pH值降至5.5以下，但铵排泄率极低；这种模式归因于选择性醛固酮缺乏。镰状细胞血红蛋白病应列入高钾性高氯性代谢性酸中毒的鉴别诊断中。
