Long‐term Outcome of Children With Biliary Atresia Who Were Not Transplanted After the Kasai Operation: &gt;20‐year Experience at a Children's Hospital

Sato, Masato; Ohhama, Youkatsu; Take, Hiroshi; Kitagawa, Norihiko; Kaneko, Hironori; Mochizuki, Kiyofumi; Hatata, Tomoko

doi:10.1097/mpg.0b013e318189f2d5

“…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: 62%

“…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.…”

Section: Place Of Originmentioning

confidence: 74%

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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“…O encaminhamento precoce dos pacientes para realização de portoenterostomia em tempo adequado, continua sendo um desafio em todo o mundo [22][23][24][25][26] , embora já venha sendo observado em alguns países 27 , uma tendência de redução da idade no momento do encaminhamento ao longo dos anos. Em países desenvolvidos, a idade no momento da portoenterostomia é de aproximadamente 60 dias 13,14,28 .…”

Section: Discussionunclassified

“…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

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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“…In the hypertrophied area of the liver, the liver architecture can be near normal with bile ducts whereas the atrophic area usually shows severe fibrosis and only a few bile ducts remain [48]. Data is now available on long-term follow-up in BA patients post-KPE [49][50][51][52][53][54] (Table 3) ranging from 23-46% at 20 years [49,50,52]. Individuals surviving into adulthood with their native liver appear to have appropriate cognitive development with one study reporting that 88% were employed or attending higher education [53].…”

Section: Biliary Atresiamentioning

confidence: 99%

The management of childhood liver diseases in adulthood

Joshi

¹

,

Gupta

²

,

Samyn

³

et al. 2017

Journal of Hepatology

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SummaryAn increasing number of patients with childhood liver disease survive into adulthood. These young adults are now entering adult services and require ongoing management. Aetiologies can be divided into liver diseases that develop in young adults which present to adult hepatologists i.e., biliary atresia and Alagille syndrome or liver diseases that occur in children/adolescents and adults i.e., autoimmune hepatitis or Wilson's disease. To successfully manage these young adults, a dynamic and responsive transition service is essential. In this review, we aim to describe the successful components of a transition service highlighting the importance of self-management support and a multi-disciplinary approach. We will also review some of the liver specific aetiologies which are unique to young adults, offering an update on pathogenesis, management and outcomes. Ó

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Long‐term Outcome of Children With Biliary Atresia Who Were Not Transplanted After the Kasai Operation: >20‐year Experience at a Children's Hospital

Abstract: Although increasing numbers of patients with BA survive 20 years or more after the Kasai operation, meticulous lifelong postoperative care should be continued for the survivors because of the possibility of hepatic deterioration.

Cited by 141 publications

References 26 publications

Atresia biliar: a experiência Brasileira

Atresia biliar: a experiência Brasileira

Biliary atresia: the Brazilian experience

The management of childhood liver diseases in adulthood

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