Mizuochi Tatsuki, Suda Kenji, Seki Yoshitaka, Yanagi Tadahiro, Yoshimoto Hironaga, Kudo Yoshiyuki, Iemura Motofumi, Tanikawa Ken, Matsuishi Toyojiro
Department of Pediatrics and Child Health, Kurume University School of Medicine, Kurume, Japan.
Pediatr Int. 2015 Apr;57(2):e39-41. doi: 10.1111/ped.12603.
There are few reports on successful high-dose spironolactone treatment of refractory protein-losing enteropathy (PLE) caused by Fontan procedure. We report successful diuretics treatment with spironolactone and furosemide at standard dose, of refractory PLE in a patient with Noonan syndrome and repaired congenital heart disease. This is the first successful application of diuretics treatment in a patient with refractory PLE without Fontan procedure. This case illustrates that diuretics treatment can be the first-line treatment of PLE regardless of the causative physiology, and can be effective in refractory PLE with Noonan syndrome.
关于大剂量螺内酯成功治疗Fontan手术引起的难治性蛋白丢失性肠病（PLE）的报道很少。我们报告了一例努南综合征合并先天性心脏病修复术后的患者，使用标准剂量的螺内酯和呋塞米成功进行利尿剂治疗难治性PLE。这是利尿剂治疗在未行Fontan手术的难治性PLE患者中的首次成功应用。该病例表明，无论病因生理学如何，利尿剂治疗均可作为PLE的一线治疗方法，且对努南综合征所致的难治性PLE可能有效。
