Calcagno P L
Pediatr Res. 1979 Dec;13(12):1379-81. doi: 10.1203/00006450-197912000-00016.
Patients with idiopathic hypokalemic metabolic alkalosis and hyperrenienmia have been lumped under the heading of Bartter's syndrome. However, the clinical picture is not totally uniform. Recently, Gullner et al. described a familial disorder with hypokalemic metabolic alkalosis, hyperreninemia, and aldosteronism, but without juxtaglomerular hyperplasia. They suggested that this family had a condition other than Bartter's syndrome. The present report details the followup from infancy to adulthood of a patient with hypokalemic metabolic alkalosis, salt wasting, and hyperreninemia, but with normal aldosterone level and without juxtaglomerular hyperplasia. The authors suggest that this new condition be termed renal alkalosis. The studies suggest that the distal tubular reabsorptive capacity was defective in this patient.
患有特发性低钾血症性代谢性碱中毒和高肾素血症的患者被归类为巴特综合征。然而,临床表现并不完全一致。最近,古尔纳等人描述了一种家族性疾病,伴有低钾血症性代谢性碱中毒、高肾素血症和醛固酮增多症,但无肾小球旁器增生。他们认为这个家族患的是不同于巴特综合征的疾病。本报告详细介绍了一名患有低钾血症性代谢性碱中毒、盐耗竭和高肾素血症,但醛固酮水平正常且无肾小球旁器增生的患者从婴儿期到成年期的随访情况。作者建议将这种新病症称为肾性碱中毒。研究表明,该患者的远曲小管重吸收能力存在缺陷。