Neumann L, Pelz J, Kunze J
Institute of Human Genetics and University Children's Hospital, Charité Campus Virchow-Klinikum, Humboldt University of Berlin, Germany.
Am J Med Genet. 1998 Jul 24;78(4):366-70. doi: 10.1002/(sici)1096-8628(19980724)78:4<366::aid-ajmg12>3.0.co;2-g.
Published cases of familial unilateral terminal transverse defects are scarce. We report on a morphologically similar defect of the hand in a father and his daughter. The hand anomaly is similar in both, but on the opposite side. Thalidomide was taken in the sensitive period of the pregnancy by the father's mother. To our knowledge this is the second description of unilateral terminal aphalangia in successive generations. In order to evaluate the possible genetic basis we analyze epidemiological studies in respect to the recurrence risk of cases with isolated limb reduction defects. We compare reports of familial occurrence concerning the degree of relationship as well as the pattern of malformation. The latter seems to be an important aspect from an evolutionary and a developmental viewpoint. For our observation an autosomal dominant transmission is the most likely although multifactorial determination cannot be excluded.
已发表的家族性单侧末端横断缺损病例很少。我们报告了一位父亲和他女儿手部形态相似的缺损情况。两人手部异常相似,但位于相反侧。孩子父亲的母亲在孕期敏感期服用了沙利度胺。据我们所知,这是连续两代人中单侧末端无指(趾)畸形的第二例报道。为了评估可能的遗传基础,我们分析了关于孤立性肢体短小缺损病例复发风险的流行病学研究。我们比较了家族性发病报告中亲属关系程度以及畸形模式。从进化和发育的角度来看,后者似乎是一个重要方面。就我们的观察而言,常染色体显性遗传传递最有可能,尽管不能排除多因素决定。