Genetic and environmental factors in the aetiology of familial paroxysmal polyserositis. An analysis of 150 cases from Lebanon.
To assess the hypothesis that factors other than a single gene are involved in the aetiology of familial paroxysmal polyserositis (FPP) clinical and genetic data on 150 patients have been analyzed. The finding of a significantly lower number of observed-affected compared to the expected in the Lenz-Hogben method of analysis is not in favour for an autosomal recessive mode of inheritance. The absence of a significant difference in the proportion of affected offspring in families where one of the parents has the disease, compared to the families where the parents are normal, is further not consistent with an autosomal dominant mode of inheritance. The observation in the present study of the occurrence of the more severe forms of the disease in the familial cases compared to the non-familial, isolated cases, make a polygenic type of inheritance or an autosomal inheritance where the penetrance of the disease is influenced by extraneous factors, more likely models for the aetiology of FPP. The possible role of an atopic model for the aetiology of FPP is discussed.
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D004781:Environmental Exposure; D010505:Familial Mediterranean Fever / Q000209:etiology / Q000235:genetics*; D005260:Female; D005799:Genes, Dominant; D005808:Genes, Recessive; D006801:Humans; D006969:Hypersensitivity, Immediate / Q000150:complications; D007861:Lebanon; D008297:Male; D008957:Models, Genetic; D010641:Phenotype