Time-Space Distribution of Extrahepatic Biliary Atresia in The Netherlands and West Germany

Houwen, Rhj; Kerremans, I.; Steensel-Moll, H A van; Romunde, L. K. J. Van; Bijleveld, Cma; Schweizer, Paul

doi:10.1055/s-2008-1043419

Cited by 32 publications

(47 citation statements)

References 3 publications

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“…Incidence of BA atresia in Croatia (1:23,600) is lower than incidence reported in other European studies (it varies between 1:14,000 and 1:20,000) but still belongs to the confidence interval of Swiss and Dutch studies [4,9,11,14,24]. It is reasonable to hypothesize that unfortunate circumstance in the first years of our survey which caused great migrations of people to Western countries must be taken in account when interpreting our data.…”

Section: Discussioncontrasting

confidence: 70%

Biliary atresia: the Croatian experience 1992–2006

et al. 2010

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The objective of this study was to determine the outcomes of Croatian children with biliary atresia. Health records of infants born in Croatia between January 1, 1992 and December 31, 2006 who were diagnosed with biliary atresia and treated at a single university center were reviewed. Survival rates were calculated with the Kaplan-Meier method. Twenty-nine patients with biliary atresia were identified. Incidence was one in 23,600 live births (95% confidence interval 1/17,400-1/27,200). The median age at Kasai operation was 66 days (range 22-192). Median follow-up was 2.65 years (range 0.2-14.3). Overall survival rates for the patients who underwent Kasai portoenterostomy, including those six (20%) who subsequently underwent liver transplantation, were 75.6%. Five and 10-year native liver survival rates were 51.7% and 38.8%, respectively (median survival time was 7.88 years). Survival rate curves in two groups of patients according to the biliary atresia phenotype (fetal or perinatal form) were divergent. Survival was 87.7% in perinatal form and 43.8% in fetal form (Breslow chi-square 8.082, p < 0.01). Overall survival rates of patients with biliary atresia in Croatia compared unfavorably with current international standards; this could be improved with earlier referral for liver transplantation. Results of Kasai operation (native liver survival rates) compared favorably with results reported elsewhere.

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Section: Discussioncontrasting

confidence: 70%

Biliary atresia: the Croatian experience 1992–2006

et al. 2010

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“…The incidence of BA ranges from 0.5 per 10 000 live births in the Netherlands to 3.2 in French Polynesia [9,10], and in Japan, the incidence is 1.0 to 1.2 per 10 000 live births according to the registry data of Japanese Biliary Atresia Society [1]. The present population-based study of BA found an incidence of 1.1/10 000 live births in the Hyogo area.…”

Section: Discussionmentioning

confidence: 92%

Insignificant seasonal and geographical variation in incidence of biliary atresia in Japan: a regional survey of over 20 years

Wada

Muraji

Yokoi

et al. 2007

Journal of Pediatric Surgery

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“…The NS et al observed that conception during spring was more associated with the occurrence of BA than conception during winter 9 . Other groups from different countries have found no association of BA with seasonality 5,[10][11][12]19 . Different investigation methods, samples sizes, and environmental or weather conditions in different regions may explain these conflicting results.…”

Section: Discussionmentioning

confidence: 99%

“…This study showed that the conception of a patient with BA was 84% less likely if this condition was diagnosed in winter, suggesting a seasonal influence on BA etiology, although season at birth was not associated with the occurrence of BA. Seasonality suggests that environmental factors can trigger BA, but conflicting results have been described on this topic [9][10][11][17][18][19] . In Texas, a higher number of BA patients were born during summer and spring in the countryside, suggesting that the noxious agent could be an agricultural toxin 17 .…”

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confidence: 99%

Environmental Risk Factors Associated With Biliary Atresia in Rio Grande Do Sul, Brazil

Longo¹,

Ferrari²,

Hoss³

et al. 2017

Clin. Biomed. Res.

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Biliary atresia (BA) seems to be a multifactorial disorder in which environmental factors interact with the patient's genetic constitution. This study aimed to analyze information concerning environmental risk factors associated with BA in southern Brazil. A case-control study with mothers of patients with BA and mothers of patients with cystic fibrosis (CF) was conducted. Inquiry included questions related to exposition to environmental risk factors during the periconceptional and gestational (second and third trimesters) periods. Mothers of BA patients had smoked during pregnancy more frequently in comparison with the mothers of CF patients, but no significant difference was found in a multivariate analysis. There was no between group difference in terms of seasonality, but the multivariate analysis showed a significant difference within the BA group between date of conception in winter compared to other seasons. In conclusion, smoking during pregnancy seemed to increase the risk of BA while date of conception in winter decreased it.Keywords: Biliary atresia; environmental risk factor; etiology; seasonality; smoking Biliary atresia (BA) is a disease that begins exclusively in infancy and consists in the complete obstruction of extrahepatic and intrahepatic bile ducts 1,2 . Regardless of a timely surgical treatment, BA induces cirrhosis and cholangiopathy and constitutes the leading cause of pediatric liver transplantation 3,4 . The etiology of biliary obstruction and chronic cholangiopathy is not fully understood, and their association with genetic/chromosomal alterations, immune and immunogenetic abnormalities, environmental factors and maternal diseases, such as gestational diabetes, has been proposed 1,[5][6][7] . An immune disorder induced by viral infection seems to be part of BA etiology 8 . Distinct variants of the disease, such as BA splenic malformation (BASM), isolated BA, cystic BA, and cytomegalovirus IgM-positive BA, suggest distinct patterns of acquisition 3,7 . BA seems to be a multifactorial disorder in which environmental factors interact with the patient's genetic constitution. Environmental risk factors associated with the development of BA have been seldom investigated 5,[9][10][11][12] . In the present study, we collected and analyzed information concerning family history and environmental risk factors in the gestational and periconceptional periods associated with the occurrence of BA in the state of Rio Grande do Sul, southern Brazil, aiming to elucidate the etiology of this disease. METHODSThis was a case-control study in which the evaluated sample included mothers of patients with the isolated form of BA. The investigation was conducted during the first weeks after birth or at different points during outpatient follow-up. Mothers of infants, children and adolescents (up to 18 years old) St. Hill), and use of medications, condoms and skin creams. Additional topics were parents' age, pregnancy planning, season at conception and at birth, and diagnosis of infections. To control a...

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Time-Space Distribution of Extrahepatic Biliary Atresia in The Netherlands and West Germany

Cited by 32 publications

References 3 publications

Biliary atresia: the Croatian experience 1992–2006

Biliary atresia: the Croatian experience 1992–2006

Insignificant seasonal and geographical variation in incidence of biliary atresia in Japan: a regional survey of over 20 years

Environmental Risk Factors Associated With Biliary Atresia in Rio Grande Do Sul, Brazil

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