Gauri L A, Chajjar K S, Saxena H C
Department of Medicine, S.P. Medical College, Bikaner.
J Assoc Physicians India. 1990 Feb;38(2):151-3.
Fifteen patients of acute intermittent porphyria (AIP) were detected out of 2500 persons of Maheshwari community surveyed. These 15 patients belonged to 11 families. The prevalence of AIP in Maheshwari population came out to be 1:640 which is considerably higher than the figures reported from many other areas of our country. AIP was more common in females than males, the ratio being 9:6. Maximum number of cases (6) were encountered in the age group of 11-20 years. AIP was observed to be inherited as an autosomal dominant disease in 3 families. Spontaneous mutation of gene may be considered to be the mode of inheritance in the remaining 8 families. The disease was found to be more common in Daga and Tapadia subcastes. There were 5 cases belonging to Daga subcaste and 3 belonging to Tapadia subcaste.
在接受调查的2500名马赫什瓦里社区居民中,检测出15例急性间歇性卟啉病(AIP)患者。这15名患者分属11个家庭。马赫什瓦里人群中AIP的患病率为1:640,这一数字远高于我国其他许多地区报告的数据。AIP在女性中比男性更常见,男女比例为9:6。11至20岁年龄组的病例数最多(6例)。在3个家庭中,AIP被观察到呈常染色体显性疾病遗传。其余8个家庭的遗传方式可能被认为是基因自发突变。该疾病在达加和塔帕迪亚亚种姓中更为常见。有5例属于达加亚种姓,3例属于塔帕迪亚亚种姓。