ABSTRACT
The clinical records of 237 patients with extrahepatic biliary atresia (EHBA) attendi9ng King's College Hospital, London, between March 1973 and October 1985 were analyzed in terms of familial and reproductive factors, as well as of their possible effect on patient survival. The male: female ratio was 0.93, and the ages of the patients'mothers and fathers were significantly higher than would have been expected from sibship data. Similarly, the number of first-born children having EHBA was less than expected. In a subsample of 189 mothers, the obstetrical histories of women who had children with associated EHBA (20% of the total) showed: 1) a higher frequency of illness before and during pregnancy; 2) a higher level of pharmaceutical drug consumption during pregnancy, and 3) more fetal losses, especially in the gestation immediately preceding the patient's birth. Age at death (average 15.1 ñ 13.2 monthjs) and survival rates depend critically on surgical intervention and were not related to the presence or absence of extrahepatic malformations or to the type of atresia. The present observations, taken together with those of others, indicate that problems in the reproductive process or exposure to noxious environmental agents may be etiological factors in associated EHBA
Subject(s)
Pregnancy , Biliary Atresia/etiology , Congenital Abnormalities , Environment , Risk Factors , SurvivalABSTRACT
Four pairs of discordant twins were observed in a series of 237 extrahepatic biliary atresia patients ascertained in London. The twinning prevalence (1.7%) was as expedcted considering the ethnic composition of the sample. Out of a total of 17 other twin pairs reported in the literature only one was concordant for the disease. Since only 17 instances of familial cases have been described, the conclusion is that any influence of genetic factors in this condition is likely to be manifested indirectly, possibly in the form of increased susceptibility of the biliary epithelium to infectious or toxic agents