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分期单心室姑息治疗与纯合子镰状细胞病

Staged Single Ventricle Palliation and Homozygous Sickle Cell Disease.

作者信息

Maddali Madan Mohan, Junghare Minakshi Sandip, Nishant Arora Ram, Kandachar Pranav Subbaraya, Valliattu Johan

机构信息

Department of Cardiac Anesthesia, National Heart Center, Royal Hospital, Muscat, Oman.

Department of Cardiothoracic Surgery, National Heart Center, Royal Hospital, Muscat, Oman.

出版信息

J Card Surg. 2016 Apr;31(4):237-41. doi: 10.1111/jocs.12721. Epub 2016 Feb 24.

DOI:10.1111/jocs.12721
PMID:26917191
Abstract

Hypoxemia is a well-known trigger for precipitating a sickling crisis in patients with sickle cell disease. Patients undergoing staged single ventricle palliation have hypoxemia during the initial stages of the Fontan pathway. The successful completion of staged single ventricle palliation in a child with a combination of homozygous sickle cell disease and a single ventricle physiology that tolerate prolonged hypoxemia during earlier stages of Fontan pathway is described.

摘要

低氧血症是镰状细胞病患者引发镰状细胞危象的一个众所周知的诱因。接受分期单心室姑息治疗的患者在Fontan手术路径的初始阶段会出现低氧血症。本文描述了一名患有纯合镰状细胞病且具有单心室生理特征的儿童成功完成分期单心室姑息治疗的案例，该患儿在Fontan手术路径的早期阶段能够耐受长时间的低氧血症。

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