[脊髓脊膜膨出的临床异质性作为神经管多部位闭合的证据]

[Clinical heterogeneity of myelomeningocele as evidence of multisite closure of the neural tube].

作者信息

Rogala P, Szulc A, Pawlak A, Głowacki M

机构信息

Kliniki Ortopedii Instytutu Ortopedii i Rehabilitacji AM w Poznaniu.

出版信息

Chir Narzadow Ruchu Ortop Pol. 1995;60(2):97-105.

PMID:7671738

Abstract

Eighty-two patients treated surgically for myelomeningocele or myelodysplasia between 1945 and 1993 are presented. Family and environmental history has been studied. The presence of genetic markers (HLA B27, ABO, Rh) has been investigated. Heterogenicity of localization and type of defect in the series reviewed has been found. Associations between certain genetic features confirm multisite mechanism of closure of the neural tube. The relationship between site of neural tube defect and concurrence of developmental defects has been substantiated. A higher site often results in multiple defects.

摘要

本文介绍了1945年至1993年间接受手术治疗的82例脊髓脊膜膨出或脊髓发育异常患者。研究了家族和环境史。调查了遗传标记（HLA B27、ABO、Rh）的存在情况。在所回顾的系列病例中发现了缺陷定位和类型的异质性。某些遗传特征之间的关联证实了神经管闭合的多部位机制。神经管缺陷部位与发育缺陷并发之间的关系得到了证实。较高的部位往往导致多种缺陷。