Biliary Atresia: The Canadian Experience

Schreiber, Richard A.; Barker, Colin; Roberts, Eve A.; Martin, Steven R.; Álvarez, Fernando; Smith, Lesley; Butzner, J. Decker; Wrobel, Iwona; Mack, David; Moroz, Stanley P.; Rashid, Mohsin; Persad, Rabin; Lévesque, Dominique; Brill, Herbert; Bruce, Garth; Critch, Jeff

doi:10.1016/j.jpeds.2007.05.051

“…In developed countries, age at portoenterostomy is around 60 days. 13,14,28 The 4-year survival rate among Brazilian patients who underwent portoenterostomy (73.4%) was similar to that observed in other centers, such as in Canada (79.0%) 5 and France (75.3%). 13 The 4-year native liver survival rate of…”

Section: Survival Ratessupporting

confidence: 81%

“…Brazilian patients (36.8%) was similar to that of patients from Canada (36.0%) 5 and Switzerland (37.4%), 15 but lower than that observed in the United Kingdom (51.0%) 14 and Japan (63% in 5 years). 28 In this study, 4-year survival rate and 4-year native liver survival rate were both inversely correlated with age at portoenterostomy, reinforcing the detrimental effect of age on the postoperative prognosis.…”

Section: Place Of Originsupporting

confidence: 52%

“…34 In this study, 46.6% of patients underwent LTx, a low rate in comparison with other countries, such as Switzerland (64.6%) 15 and Canada (60.0%). 5 The low Figure 1 shows that performance of LTx reduced mortality rates among Brazilian children with BA, with a post-transplant survival reaching 88.3%, a rate similar to that observed in Canada, 5 USA, 12 and United Kingdom. 35 Another factor that may negatively impact overall survival rates, in addition to availability of LTx, is late referral of patients with BA.…”

Section: Place Of Originmentioning

confidence: 62%

“…For those who present complications such as portal hypertension and cirrhosis, LTx provides a good quality of life. 11 The experience of several countries regarding BA has been described, 5,[12][13][14][15][16][17] but there are only a few isolated reports on the experience with BA in Brazil. 18,19 This study aimed to evaluate clinical, epidemiological and prognostic characteristics of Brazilian children with BA.…”

mentioning

confidence: 99%

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Atresia biliar: a experiência Brasileira

Carvalho¹,

Santos

²

,

Silveira

³

et al. 2010

J. Pediatr. (Rio J.)

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Objective: To evaluate epidemiological, clinical and prognostic characteristics of children with biliary atresia.Methods: Data regarding portoenterostomy, liver transplantation (LTx), age at last follow-up and survival were collected from the records of patients followed up in six Brazilian centers and compared regarding decades of surgery.Results: Of 513 patients, 76.4% underwent portoenterostomy [age: 60-94.7 (82.6±32.8) days] and 46.6% underwent LTx. In 69% of cases, LTx followed portoenterostomy, whereas in 31% of cases LTx was performed as the primary surgery. Patients from the Northeast region underwent portoenterostomy later than infants from Southern (p = 0.008) and Southeastern (p = 0.0012) Brazil, although even in the latter two regions age at portoenterostomy was higher than desirable. Over the decades, LTx was increasingly performed. Overall survival was 67.6%. Survival increased over the decades (1980s vs. 1990s, p = 0.002; 1980s vs. 2000s, p < 0.001; 1990s vs. 2000s, p < 0.001). The 4-year post-portoenterostomy survival, with or without LTx, was 73.4%, inversely correlated with age at portoenterostomy (80, 77.7, 60.5% for ≤ 60, 61-90, > 90 days, respectively). Higher survival rates were observed among transplanted patients (88.3%). The 4-year native liver survival was 36.8%, inversely correlated with age at portoenterostomy (54, 33.3, 26.6% for ≤ 60, 61-90, > 90 days, respectively). Conclusions:This multicenter study showed that late referral for biliary atresia is still a problem in Brazil, affecting patient survival. Strategies to enhance earlier referral are currently being developed aiming to decrease the need for liver transplantation in the first years of life.

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“…A AB se manifesta nas primeiras semanas de vida e apresenta uma distribuição universal, com incidência variável nas diferentes regiões do mundo [2][3][4][5] . A etiologia da AB ainda não foi completamente elucidada e vários mecanismos têm sido propostos para explicar a colangiopatia progressiva observada nesta entidade 6,7 .…”

Section: Introductionunclassified

Biliary atresia: the Brazilian experience

Carvalho¹,

Santos²,

Silveira³

et al. 2010

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Objective: To evaluate epidemiological, clinical and prognostic characteristics of children with biliary atresia.Methods: Data regarding portoenterostomy, liver transplantation (LTx), age at last follow-up and survival were collected from the records of patients followed up in six Brazilian centers and compared regarding decades of surgery.Results: Of 513 patients, 76.4% underwent portoenterostomy [age: 60-94.7 (82.6±32.8) days] and 46.6% underwent LTx. In 69% of cases, LTx followed portoenterostomy, whereas in 31% of cases LTx was performed as the primary surgery. Patients from the Northeast region underwent portoenterostomy later than infants from Southern (p = 0.008) and Southeastern (p = 0.0012) Brazil, although even in the latter two regions age at portoenterostomy was higher than desirable. Over the decades, LTx was increasingly performed. Overall survival was 67.6%. Survival increased over the decades (1980s vs. 1990s, p = 0.002; 1980s vs. 2000s, p < 0.001; 1990s vs. 2000s, p < 0.001). The 4-year postportoenterostomy survival, with or without LTx, was 73.4%, inversely correlated with age at portoenterostomy (80, 77.7, 60.5% for ≤ 60, 61-90, > 90 days, respectively). Higher survival rates were observed among transplanted patients (88.3%). The 4-year native liver survival was 36.8%, inversely correlated with age at portoenterostomy (54, 33.3, 26.6% for ≤ 60, 61-90, > 90 days, respectively). Conclusions:This multicenter study showed that late referral for biliary atresia is still a problem in Brazil, affecting patient survival. Strategies to enhance earlier referral are currently being developed aiming to decrease the need for liver transplantation in the first years of life. J Pediatr (Rio J). 2010;86(6):473-479:Biliary atresia, portoenterostomy, hepatic, surgery, diagnosis, differential, prognosis. ResumoObjetivo: Avaliar as características epidemiológicas, clínicas e prognósticas de crianças com atresia biliar. Os pacientes da região Nordeste foram submetidos a portoenterostomia mais tardiamente do que as crianças das regiões Sul (p = 0,008) e Sudeste (p = 0,0012), embora, mesmo nas duas últimas regiões, a idade no momento da portoenterostomia tenha sido superior ao desejável. Ao longo das décadas, houve aumento progressivo do número de TxH realizados. A sobrevida global foi de 67,6%. A sobrevida aumentou nas últimas décadas (anos 1980 versus 90, p = 0,002; anos 1980 versus 2000, p < 0,001; anos 1990 versus 2000, p < 0,001). A sobrevida de 4 anos pós-portoenterostomia, com ou sem TxH, foi de 73,4%, inversamente correlacionada à idade no momento da portoenterostomia (80, 77,7, 60,5% para ≤ 60, 61-90, > 90 dias, respectivamente). Os pacientes transplantados apresentaram taxas de sobrevida mais elevadas (88,3%). A sobrevida de 4 anos com fígado nativo foi de 36,8%, inversamente correlacionada à idade no momento da portoenterostomia (54, 33,3, 26,6% para ≤ 60, 61-90, > 90 dias, respectivamente). Método Conclusões:Este estudo multicêntrico demonstrou que o encaminhamento tardio das crianças portad...

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Diagnostic Yield of Newborn Screening for Biliary Atresia Using Direct or Conjugated Bilirubin Measurements

Harpavat

¹

,

Garcia‐Prats

²

,

Anaya

³

et al. 2020

JAMA

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Treating biliary atresia in newborns earlier can delay or prevent the need for liver transplant; however, treatment typically occurs later because biliary atresia is difficult to detect during its early stages.OBJECTIVE To determine the diagnostic yield of newborn screening for biliary atresia with direct or conjugated bilirubin measurements and to evaluate the association of screening implementation with clinical outcomes. DESIGN, SETTING, AND PARTICIPANTSA cross-sectional screening study of 124 385 infants born at 14 Texas hospitals between January 2015 and June 2018; and a pre-post study of 43 infants who underwent the Kasai portoenterostomy as treatment for biliary atresia at the region's largest pediatric hepatology center before (

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Biliary Atresia: The Canadian Experience

Cited by 228 publications

References 27 publications

Atresia biliar: a experiência Brasileira

Atresia biliar: a experiência Brasileira

Biliary atresia: the Brazilian experience

Diagnostic Yield of Newborn Screening for Biliary Atresia Using Direct or Conjugated Bilirubin Measurements

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