PDF(Pubmed)
Abstract：
Protein-losing enteropathy (PLE) is a rare disorder with diverse causes, but the treatments are limited and understudied. It is often associated with significant mortality and morbidity. The survival of hypoplastic left heart syndrome (HLHS) in infants without any intervention is usually 4.5 days, and 30-day mortality is 95%. However, with surgical intervention, survival at 20 years is 80%. HLHS can lead to protein-leading enteropathy and is corrected by the three-step procedures (Norwood, Glenn, and Fontan) during infancy. We report a case of Fontan procedure postoperative HLHS associated with PLE and describe its clinical course and outcome. The main intention of reporting this case is to provide awareness among physicians while dealing with refractory cases of hypoproteinemia and appropriate management based on the literature.
摘要：
蛋白质丢失性肠病（PLE）是一种罕见的疾病，具有多种原因，但是治疗方法有限，研究不足。它通常与显著的死亡率和发病率相关。未经任何干预的婴儿左心发育不良综合征（HLHS）的生存期通常为4.5天，30天死亡率为95%。然而,通过手术干预，20年生存率为80%。HLHS可导致蛋白质主导的肠病，并通过三步程序得到纠正(Norwood，格伦,和Fontan）在婴儿期。我们报告了一例与PLE相关的HLHS术后Fontan手术，并描述了其临床过程和结果。报告此病例的主要目的是在处理难治性低蛋白血症病例和根据文献进行适当管理的同时，提高医生的认识。
