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患有中枢神经系统肿瘤的儿童及其家庭中的神经纤维瘤病和其他疾病。

Neurofibromatosis and other disorders among children with CNS tumors and their families.

作者信息

Baptiste M, Nasca P, Metzger B, Field N, MacCubbin P, Greenwald P, Armbrustmacher V, Waldman J, Carlton K

机构信息

Division of Epidemiology, New York State Department of Health, Albany 12237.

出版信息

Neurology. 1989 Apr;39(4):487-92. doi: 10.1212/wnl.39.4.487.

DOI:10.1212/wnl.39.4.487
PMID:2494566
Abstract

We conducted a population-based case-control study with 338 patients, less than 15 years of age, diagnosed with a primary tumor of the central nervous system from January 1968 through December 1977 in 53 New York State counties. The study also included 676 controls selected from the birth certificate files of the New York State Department of Health. We collected information on neurofibromatosis and congenital anomalies in study subjects, their siblings and parents by telephone interview with the mother of each case and control. We obtained supplemental information on neurofibromatosis in the patients and their families from hospital medical records. This study confirmed the strong association of neurofibromatosis with risk of CNS tumors. Thirteen cases and no controls had neurofibromatosis. Two fathers and 3 mothers of cases had neurofibromatosis. Five cases had siblings with neurofibromatosis. None of the first-degree relatives of controls had neurofibromatosis. We observed a relative risk of 4.49 for history of seizures. Seizures are often among the presenting symptoms for CNS tumors. We observed no difference between cases and controls in the occurrence of congenital anomalies. There was a nonsignificant excess of congenital anomalies among siblings of cases compared with controls. This decreased to 1.13 when adjusted for number of siblings.

摘要

我们进行了一项基于人群的病例对照研究,研究对象为1968年1月至1977年12月期间在纽约州53个县诊断出患有中枢神经系统原发性肿瘤、年龄小于15岁的338名患者。该研究还包括从纽约州卫生部出生证明档案中选取的676名对照。我们通过电话采访每个病例和对照的母亲,收集了研究对象、他们的兄弟姐妹和父母的神经纤维瘤病及先天性异常信息。我们从医院病历中获取了患者及其家庭中神经纤维瘤病的补充信息。这项研究证实了神经纤维瘤病与中枢神经系统肿瘤风险之间的强关联。13例病例患有神经纤维瘤病,对照中无一人患有。病例组中有2名父亲和3名母亲患有神经纤维瘤病。5例病例的兄弟姐妹患有神经纤维瘤病。对照组的一级亲属中无人患有神经纤维瘤病。我们观察到癫痫病史的相对风险为4.49。癫痫常常是中枢神经系统肿瘤的首发症状之一。我们观察到病例组和对照组在先天性异常的发生率上没有差异。与对照组相比,病例组兄弟姐妹的先天性异常发生率有不显著的增加。在对兄弟姐妹数量进行调整后,这一比例降至1.13。

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