Mendonça Marta, Pinheiro Adriana, Castro Isabel
Serviço de Pediatria, Hospital do Divino Espírito Santo, Ponta Delgada, Açores, Portugal.
Acta Med Port. 2011 Dec;24 Suppl 3:671-4. Epub 2011 Dec 31.
The Bartter syndrome is a rare hereditary salt-wasting tubulopathy, characterized by metabolic alkalosis, hypokalemia, hyperreninemia and hyperaldosteronemia of varying severity. Indomethacin and high doses of oral potassium have been until now the therapeutic strategies used, with high risk of gastrointestinal injury. Since April 2009, aliskiren--renin inhibitor--has been used in individual cases of Bartter syndrome in adults, by ignoring its use in pediatrics. The authors present the case of an eight year old child with Bartter syndrome, treated with oral potassium chloride and oral indomethacin, whom has been diagnosed a giant gastric ulcer. To enable the reduction of the amount of potassium administered, we chose to start the aliskiren.
巴特综合征是一种罕见的遗传性失盐性肾小管病,其特征为不同程度的代谢性碱中毒、低钾血症、高肾素血症和高醛固酮血症。到目前为止,吲哚美辛和高剂量口服钾一直是所采用的治疗策略,但存在较高的胃肠道损伤风险。自2009年4月以来,阿利吉仑(一种肾素抑制剂)已被用于成人巴特综合征的个别病例,而忽略了其在儿科的应用。作者报告了一例8岁巴特综合征患儿的病例,该患儿接受口服氯化钾和口服吲哚美辛治疗,被诊断为巨大胃溃疡。为了能够减少钾的给药量,我们选择开始使用阿利吉仑。