白塞病患者家系的遗传分析：数据与常染色体隐性遗传不符。
Genetic analysis of families of patients with Behçet's syndrome: data incompatible with autosomal recessive inheritance.
作者信息
Bird Stewart J A
出版信息
Ann Rheum Dis. 1986 Apr;45(4):265-8. doi: 10.1136/ard.45.4.265.
Abstract
Data on families with Behçet's syndrome were analysed to test a hypothesis of autosomal recessive inheritance. Fifteen families from the UK and nine from Turkey were included. There were 27 individuals with Behçet's syndrome according to the Japanese criteria and 119 unaffected individuals. There were no affected parents in the families. The 'goodness of fit' test of Elandt-Johnson was applied to the distribution of affected individuals in these families, and the data were found to be incompatible with autosomal recessive inheritance. HLA associations were also examined in patients and relatives, and this led to re-examination of previously published data on HLA associations with Behçet's syndrome.
摘要
对患有白塞病的家庭数据进行了分析，以检验常染色体隐性遗传的假设。研究纳入了来自英国的15个家庭和来自土耳其的9个家庭。根据日本标准，有27名个体患有白塞病，119名个体未受影响。这些家庭中没有患病的父母。将伊兰特 - 约翰逊的“拟合优度”检验应用于这些家庭中患病个体的分布情况，发现数据与常染色体隐性遗传不相符。还对患者及其亲属进行了HLA关联检查，这导致对先前发表的关于HLA与白塞病关联的数据进行重新审视。
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