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肾移植受者包裹性腹膜硬化症 1 例报告。

Encapsulating Peritoneal Sclerosis in a kidney transplant recipient - Case Report.

机构信息

Hospital Beneficência Portuguesa de São Paulo, Equipe de Nefrologia e Transplante de Rim e Rim-Pâncreas, São Paulo, SP, Brasil.

Hospital Beneficência Portuguesa de São Paulo, Instituto de Gastrocirurgia Avançada, Programa de Cirurgia Geral e Aparelho Digestivo, São Paulo, SP, Brasil.

出版信息

J Bras Nefrol. 2020 Jul-Sep;42(3):370-374. doi: 10.1590/2175-8239-JBN-2019-0193.

Abstract

Encapsulating Peritoneal Sclerosis (EPS) is a severe and rare condition frequently associated with peritoneal dialysis, characterized by bowel obstruction, with lethal consequences in 20% of the patients. The disease presents as a mass of fibrous tissue encapsulating visceral organs that may potentially compromise digestive tract function. This report describes the case of a patient under peritoneal dialysis (PD) due to chronic kidney disease secondary to focal segmental glomerulosclerosis diagnosed with EPS. The patient had undergone two living-donor kidney transplant procedures. Surgical techniques and clinical measures employed to unravel bowel obstruction are described, which have been shown to ameliorate EPS secondary complications. Parenteral nutrition has significantly contributed to afford adequate nutrition, improving tissue healing as well as serum protein levels, vitamins and electrolytes. Therapy with tamoxifen and sodium thiosulfate effectively delayed the development of EPS.

摘要

包裹性腹膜硬化症(EPS)是一种严重且罕见的疾病,常与腹膜透析相关,其特征为肠梗阻,20%的患者因此而死亡。该疾病表现为包裹内脏器官的纤维组织肿块,可能会影响消化道功能。本报告描述了一位因局灶节段性肾小球硬化症导致慢性肾脏病而接受腹膜透析(PD)的患者,该患者被诊断为 EPS。该患者已接受了两次活体供肾移植手术。本文介绍了用于解决肠梗阻的手术技术和临床措施,这些措施已被证明可以改善 EPS 的继发性并发症。肠外营养显著有助于提供充足的营养,促进组织愈合以及提高血清蛋白、维生素和电解质水平。他莫昔芬和硫代硫酸钠治疗有效地延缓了 EPS 的发展。

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