Biliary Atresia: Swiss National Study, 1994–2004

Wildhaber, Barbara E.; Majno, Pietro; Mayr, Johannes A.; Zachariou, Zacharias; Hohlfeld, Judith; Schwoebel, Marcus G.; Kistler, Walter; Meuli, Martin; Coultre, C. Le; Mentha, Gilles; Belli, Dominique Charles; Chardot, Christophe

doi:10.1097/mpg.0b013e3181633562

“…A taxa de sobrevida de 4 anos com fígado nativo de pacientes brasileiros (36,8%) foi semelhante à dos pacientes canadenses (36,0%) 5 e suíços (37,4%) 15 , porém menor do que a observada no Reino Unido (51,0%) 14 e no Japão (63% em 5 anos) 28 .…”

Section: Discussionunclassified

“…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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“…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 Originmentioning

confidence: 52%

“…13 In the long run, however, most patients with BA ultimately need a LTx. 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.…”

Section: Place Of Originmentioning

confidence: 99%

“…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%

See 1 more Smart Citation

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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