Late Referral for Biliary Atresia— Missed Opportunities for Effective Surgery

Mieli‐Vergani, Giorgina; Portman, B.; Howard, E. R.; Mowat, A P

doi:10.1016/s0140-6736(89)90012-3

“…Neste estudo, a taxa de sobrevida de 4 anos e a taxa de sobrevida de 4 anos com fígado nativo estiveram inversamente correlacionadas à idade da criança no momento da portoenterostomia, reforçando o efeito negativo da idade avançada no prognóstico pós-operatório 8,26,29 . Sabe-se que a idade no momento da portoenterostomia influencia na sobrevida com fígado nativo e, segundo Serinet et al 30 , seu impacto permanece até a adolescência, pois, se todos os pacientes com AB fossem submetidos a portoenterostomia antes de 46 dias de vida, 5,7% de todos os TxH realizados na França em pacientes menores de 16 anos poderiam ser evitados.…”

Section: Discussionunclassified

“…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 . O diagnóstico precoce da AB e o tratamento cirúrgico (portoenterostomia), que restabelece o fluxo biliar, realizados preferencialmente antes dos 60 dias de vida, são fundamentais, uma vez que o diagnóstico tardio relaciona-se à necessidade de TxH ou morte nos primeiros 3 anos de vida [8][9][10] . O prognóstico dos pacientes com AB tem melhorado nas últimas décadas, atingindo sobrevida de 90% dos casos 10 , devido à realização da portoenterostomia em idade adequada e do TxH seguido de imunossupressão.…”

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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“…The success of portoenterostomy has been linked to an earlier age at surgery, 10 the absence of Fig. 1.…”

Section: Discussionmentioning

confidence: 99%

Randomized, double-blind, placebo-controlled trial of corticosteroids after Kasai portoenterostomy for biliary atresia

Davenport

¹

,

Stringer

²

,

Tizzard

³

et al. 2007

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The objective of this study was to evaluate adjuvant corticosteroids after Kasai portoenterostomy for biliary atresia. The study consisted of a prospective, 2-center, double-blind, randomized, placebo-controlled trial of post-Kasai portoenterostomy corticosteroids (oral prednisolone: 2 mg/kg/day from day 7 to day 21 and 1 mg/kg/day from day 22 to day 28). The data were compared with 2 or Mann-Whitney tests, as appropriate. Seventy-one postoperative infants with type 3 biliary atresia were randomized to receive either oral prednisolone (n ‫؍‬ 36) or a placebo (n ‫؍‬ 37). At 1 month, the median bilirubin level was lower in the steroid group (66 versus 92 mol/L, P ‫؍‬ 0.06), but no difference was evident at 6 (P ‫؍‬ 0.56) or 12 (P ‫؍‬ 0.3) months. The proportion of infants with a normal bilirubin level (<20 mol/L) at 6 (47% versus 49%, P ‫؍‬ 0.89) and 12 months (50% versus 40%, P ‫؍‬ 0.35) was not significantly different. The need for transplantation by 6 (12% versus 13%, P ‫؍‬ 0.99) and 12 months (26% versus 35%, P ‫؍‬ 0.47) was not significantly different. The steroid effect was more pronounced in younger infants (less than 70 days at Kasai portoenterostomy, n ‫؍‬ 51), with a reduced bilirubin level at 1 month (64 versus 117 mol/L, P ‫؍‬ 0.01) and with a greater proportion with a normal bilirubin level at 12 months (54% versus 37%, P ‫؍‬ 0.22). Conclusion: There was a beneficial effect on the rate of reduction of bilirubin in the early postoperative period (specifically in infants less than 70 days old at surgery), but this steroid regimen did not reduce the need for liver transplantation.

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“…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. 8,26,29 Age at portoenterostomy is known to affect native liver survival. According to Serinet et al, 30 France (25.0% in 5 years).…”

Section: Place Of Originmentioning

confidence: 99%

“…[8][9][10] Prognosis of patients with BA has improved in the last decades, reaching 90% of cases, 10 due to timely performance of portoenterostomy and LTx followed by adequate immunosuppression. More than 50% of patients who undergo portoenterostomy become jaundice-free and, among them, several patients reach adolescence without LTx.…”

mentioning

confidence: 99%

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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Late Referral for Biliary Atresia— Missed Opportunities for Effective Surgery

Cited by 242 publications

References 8 publications

Biliary atresia: the Brazilian experience

Biliary atresia: the Brazilian experience

Randomized, double-blind, placebo-controlled trial of corticosteroids after Kasai portoenterostomy for biliary atresia

Atresia biliar: a experiência Brasileira

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