期刊
JOURNAL OF PEDIATRIC SURGERY
卷 47, 期 3, 页码 477-484出版社
W B SAUNDERS CO-ELSEVIER INC
DOI: 10.1016/j.jpedsurg.2011.09.042
关键词
Mucopolysaccharidosis I; Hurler; Hurler-Scheie; Scheie; Surgery; Mortality; Anesthetic complications
Background/Purpose: Mucopolysaccharidosis I (MPS I) is a rare lysosomal storage disorder caused by deficiency of alpha-L-iduronidase, which results in progressive multisystemic disease. Patients with MPS I often require multiple common and uncommon surgeries and are at risk for surgical and anesthetic complications because of respiratory and cardiac disease. Surgery often precedes diagnosis; thus, surgeons and anesthesiologists may be unaware of potential risks. Methods: We analyzed data from the MPS I Registry, a voluntary observational database, for deaths occurring within 1 month of a surgical procedure among the 932 patients enrolled as of July 2010. Results: Among the 196 deceased patients, 186 reported 1 surgery or more, and 32 had 1 surgery or more within 1 month of death, including 20 who had 1 surgery or more within 10 days of death. Surgeries before death included hernia repair, central line placement, spinal surgery, tracheostomy, and ventriculo-peritoneal shunt. Most patients (28/32) had severe MPS I (Hurler), and 20 of 32 patients (all Hurler) died at 3 years or younger. In 6 of 32 patients, surgery was directly noted in the cause of death, including 4 patients with an attenuated form of MPS I. Conclusions: Patients with mucopolysaccharidosis have a high postoperative mortality because of underlying respiratory and cardiac diseases. (C) 2012 Elsevier Inc. All rights reserved.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据