The outcome of the older (≥100 days) infant with biliary atresia

Davenport, Mark; Puricelli, Valeria; Farrant, P.; Hadžić, Nedim; Mieli‐Vergani, Giorgina; Portmann, B; Howard, E. R.

doi:10.1016/j.jpedsurg.2003.12.014

“…No presente estudo, a sobrevida de 4 anos com fígado nativo dos pacientes operados mais tardiamente, depois de 90 dias, foi de 26,6%, semelhante à observada no Canadá (23,0% em 4 anos) 5 e na França (25,0% em 5 anos) 31 . Além disso, no Reino Unido, a taxa de sobrevida de 5 anos com fígado nativo dos pacientes operados após 100 dias atingiu 45,0% 32 . Esses dados sugerem que a portoenterostomia deve ser considerada mesmo em crianças em torno de 90 dias de vida, desde que o paciente não apresente doença hepática descompensada e complicações da hipertensão portal.…”

Section: Discussionunclassified

Biliary atresia: the Brazilian experience

Carvalho¹,

Santos²,

Silveira³

et al. 2010

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 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...

show abstract

“…Literature has identified these possible factors for the delay at referral: lack of knowledge of the importance of an early diagnosis; incorrect handling by the pediatrician, that takes the jaundice as of little importance given the good nutritional state of patients; repeated pediatric consultations to assess the so-called physiological jaundice or breast milk jaundice; erroneous assessment of serum bilirubin levels; family's naive interpretation of jaundice as a physiological event and use of home remedies as therapeutic measures in jaundice, therefore retarding the consultation with a physician; and, ultimately, a delay caused by the difficulties in accessing medical assistance (30) . Davenport et al (9) , when analyzing patients at King's College Hospital, observed that the main motives for late diagnosis and treatment were failure of parents in searching medical evaluation for the persistent jaundice, delay in transference or a wrong diagnosis by the general physician, and a liver biopsy that did not suggest BA initially.…”

Section: Evaluation Of Patients After Surgery Up To 120 Days Of Agementioning

confidence: 99%

BILIARY ATRESIA: evaluation on two distinct periods at a reference pediatric service

Queiroz

¹

,

Ferreira

²

,

Fagundes

³

et al. 2014

Arq. Gastroenterol.

View full text Add to dashboard Cite

-Context -Biliary atresia is a progressive, idiopathic, fibro-obliterative disease of the extrahepatic biliary tree that presents with biliary obstruction exclusively in the neonatal period. Objective -To assess the differences regarding age at referral, age at surgery, duration of propaedeutics and waiting time for surgery between two groups of infants in different periods. MethodsRetrospective study of infants diagnosed with biliary atresia on two periods: 1983-1993 and 1998-2011. Results -Biliary atresia was diagnosed in 129 infants, being 48 in casuistic I and 81 in casuistic II. The median age at admission was 94 and 60 days, respectively (P = 0.0001). On evaluating patients who had undergone portoenterostomy before 120 days of age, no difference was observed regarding the duration of propaedeutics or waiting time for surgery (P = 0.15), but difference was found when comparing the age at surgery (P = 0.002). Among those infants with no biliary flow and without liver transplantation or death after 18 post-operative months, the estimated probability of survival was 44.6% and 38.7% in casuistics I and II, respectively. In casuistic I, all infants who showed biliary flow were alive during the observation period and, in casuistic II, 80.3% were alive after 7 years of follow-up. Conclusions -Even though patients were admitted and treated earlier, it is clear that surgery could be done sooner. Delay in referral and timely propaedeutics were the main contributors.

show abstract

“…31 Furthermore, in the United Kingdom, the 5-year native liver survival rate of patients operated on after 100 days reached 45.0%. 32 Such data suggest that portoenterostomy should be attempted even in children around 90 days of life, provided they present neither decompensated liver disease nor complications of portal hypertension.…”

Section: Place Of Originmentioning

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.

show abstract

The outcome of the older (≥100 days) infant with biliary atresia

Cited by 127 publications

References 31 publications

Biliary atresia: the Brazilian experience

Biliary atresia: the Brazilian experience

BILIARY ATRESIA: evaluation on two distinct periods at a reference pediatric service

Atresia biliar: a experiência Brasileira

Contact Info

Product

Resources

About