Analysis of factors affecting development of carpal tunnel syndrome in patients with Hurler syndrome after hematopoietic cell transplantation
Analysis of factors affecting development of carpal tunnel syndrome in patients with Hurler syndrome after hematopoietic cell transplantation
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DOI：
10.1038/sj.bmt.1705586
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发表时间：
2007-03-01
影响因子：
4.8
通讯作者：
Orchard, P. J.
中科院分区：
文献类型：
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作者：
Khanna, G.;Van Heest, A. E.;Orchard, P. J.
患有Hurler综合征（IH型粘多糖样沉积症（MPSIH））的儿童具有骨骼、关节和软组织异常，这些异常可能在造血干细胞移植（HSCT）后持续存在或进展。我们报告了43例HSCT后MPSIH儿童发生腕管综合征（CTS）的单中心经验。23名儿童（59%）在HSCT后发生了CTS; 39名酶活性正常或杂合范围的儿童中有19名发生了CTS（49%），而所有4名酶活性低或不存在的儿童在HSCT后发生了CTS。19例相关供者骨髓受体中有14例，接受无关供者移植的19例中有8例，5例无关脐带血受体中有1例发生CTS。手术松解时的平均年龄为4.8岁。随着HSCT年龄的每年增加，风险增加55%。年龄和HSCT后酶活性是CTS发生的重要因素。在2岁之前进行移植可使发生CTS的风险降低46%;较高的酶活性可使发生CTS的风险降低78%。然而，移植MPSIH的儿童仍有发展CTS的风险，应通过神经传导速度测试进行持续监测。
Children with Hurler syndrome (mucopolysaccharidosis type IH (MPSIH)) have skeletal, joint and soft tissue abnormalities that may persist or progress after hematopoietic stem cell transplantation (HSCT). We report our single center experience with development of carpal tunnel syndrome (CTS) in 43 children with MPSIH after HSCT. Twenty-three children (59%) developed CTS following HSCT; 19 of the 39 children with enzyme activity in the normal or heterozygous range developed CTS (49%), whereas all four children with low heterozygous or absent enzyme activity developed CTS after HSCT. Fourteen of 19 related donor marrow recipients, eight of 19 of those receiving an unrelated donor graft and one of five unrelated cord blood recipients developed CTS. The mean age at surgical release was 4.8 years. With each year increase in age at HSCT, there was a 55% increased risk. Age and enzyme activity after HSCT were significant factors in the development of CTS. Transplantation by 2 years of age reduced the risk of developing CTS by 46%; higher enzyme activity led to a 78% reduction in the risk of developing CTS. However, children transplanted for MPSIH remain at risk for the development of CTS, and should be monitored on an ongoing basis by nerve conduction velocity testing.