A multicenter study of the outcome of biliary atresia in the United States, 1997 to 2000

Shneider, Benjamin L.; Brown, Morton B.; Haber, Barbara; Whitington, Peter F.; Schwarz, Kathleen B.; Squires, Robert H.; Bezerra, Jorge A.; Shepherd, Ross W.; Rosenthal, Philip; Hoofnagle, Jay H.; Sokol, Ronald J.

doi:10.1016/j.jpeds.2005.12.054

“…14 In the US, recent reports indicate that 66% of children were operated before completing two months of life. 15 Median age at surgery in our series was 80.5 days, higher than both older series (61 to 69 days) 16,17 and more recent ones (54 to 68 days). 5,14,[18][19][20][21] Only the average PE age of the Thailand series (90.3±36.4 days) was similar to that found in the present study (93.7±51.3 days).…”

contrasting

confidence: 48%

“…3,5,[14][15][16][17][18][19][20][21][22] The presence of uncompensated cirrhosis or malformations in other organs which could rule out surgery. 3,22 Nine patients over three months old and with advanced hepatic failure were not submitted to PE.…”

mentioning

confidence: 99%

Atresia biliar: continuamos operando tarde

Kieling¹,

Santos²,

Vieira³

et al. 2008

J. Pediatr. (Rio J.)

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“…Para aqueles que apresentam complicações como hipertensão portal e cirrose, o TxH proporciona uma boa qualidade de vida 11 . Vários países têm descrito suas experiências em relação à AB 5,[12][13][14][15][16][17] , porém há poucos relatos isolados sobre a experiência com AB no Brasil 18,19 . O presente estudo teve como objetivo avaliar as características clínicas, epidemiológicas e prognósticas de crianças brasileiras portadoras de AB.…”

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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“…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: 54%

“…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%

Atresia biliar: a experiência Brasileira

Carvalho¹,

Santos

²

,

Silveira

³

et al. 2010

J. Pediatr. (Rio J.)

View full text Add to dashboard Cite

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 multicenter study of the outcome of biliary atresia in the United States, 1997 to 2000

Cited by 350 publications

References 17 publications

Atresia biliar: continuamos operando tarde

Atresia biliar: continuamos operando tarde

Biliary atresia: the Brazilian experience

Atresia biliar: a experiência Brasileira

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