Department of Pediatric Nephrology, University Children's Hospital, Skopje, Macedonia.
World J Pediatr. 2011 Feb;7(1):86-8. doi: 10.1007/s12519-010-0021-6. Epub 2010 Feb 1.
antenatal variant of Bartter syndrome is characterized by a history of polyhydramnios, premature birth, metabolic alkalosis, hypokalemia, polyuria and renal salt wasting. In this report we present a premature female baby with antenatal Barter syndrome who had three episodes of urinary tract infection (UTI), without evidence for congenital anomaly of the kidneys or urinary tract.
antenatal Bartter syndrome was diagnosed according to the standard criteria. Ultrasound scan and voiding cystourethrography were performed to exclude congenital anomaly of the kidneys and urinary tract.
the baby presented with early hyperkalemia and acidosis. The typical biochemical features of the Bartter syndrome were observed in the second month. Despite appropriate treatment she had persistent hypercalciuria. The clinical course was complicated with recurrent episodes of febrile UTIs. Urinary tract system imaging did not demonstrate congenital anomalies. She finally died of severe dehydration, acidosis and renal failure.
since no congenital anomaly of the kidneys or urinary tract was demonstrated in our patient, we believe that severe, persistent hypercalciuria is the most important risk factor for development of recurrent UTIs.
产前变异型巴特综合征的特征是羊水过多、早产、代谢性碱中毒、低钾血症、多尿和肾盐丢失的病史。本报告介绍了一例产前巴特综合征的早产女性婴儿,她有 3 次尿路感染(UTI)发作,无肾脏或尿路先天畸形的证据。
根据标准标准诊断产前巴特综合征。进行超声扫描和排尿性膀胱尿道造影术以排除肾脏和尿路的先天畸形。
婴儿表现出早期高钾血症和酸中毒。第二个月观察到巴特综合征的典型生化特征。尽管进行了适当的治疗,但她仍持续高钙尿症。临床病程复杂,伴有复发性发热性 UTI 发作。尿路系统成像未显示先天畸形。她最终死于严重脱水、酸中毒和肾衰竭。
由于我们的患者未显示肾脏或尿路的先天畸形,我们认为严重、持续的高钙尿症是反复尿路感染发展的最重要危险因素。