Outcome of biliary atresia in Malaysia: A single‐centre study

Lee, Way Seah; Chai, Pei Fan; Lim, Kean-Seng; Lim, Li-Han; Looi, Lai‐Meng; Ramanujam, T. M.

doi:10.1111/j.1440-1754.2009.01490.x

Cited by 21 publications

(30 citation statements)

References 25 publications

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“…However, this advantage could not be extrapolated to our jaundice clearance rate. Although it is comparable with some previous reports, it is lower than the high‐volume western centres and other Asian countries such as Taiwan (61%) and Japan (62%) . This absence of correlation between age and outcome has also been reported by other investigators.…”

Section: Discussionsupporting

confidence: 90%

Seventeen years of Kasai portoenterostomy for biliary atresia in a single Southeast Asian paediatric centre

Chiang

Lee

Krishnaswamy

et al. 2016

J Paediatrics Child Health

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Section: Discussionsupporting

confidence: 90%

Seventeen years of Kasai portoenterostomy for biliary atresia in a single Southeast Asian paediatric centre

Chiang

Lee

Krishnaswamy

et al. 2016

J Paediatrics Child Health

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“…Desse modo, no Brasil, o encaminhamento tardio dos pacientes com AB para portoenterostomia continua sendo um problema a ser resolvido em nível nacional, independentemente da região ou da categoria da cidade, seja capital ou cidade do interior. 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

Biliary atresia: the Brazilian experience

Carvalho¹,

Santos²,

Silveira³

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 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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“…All cases of BA were confirmed with operative cholangiogram, or demonstration of atretic gallbladder and/or extrahepatic biliary tree intra-operatively [13,14] . Diagnosis of other causes of NC were made according to standard and acceptable clinical practices and have been described previously [1,15] . The histology review process was conducted in December 2007, more than two years after the final patient in the study cohort was recruited to allow a firm clinical diagnosis to be made in all patients.…”

Section: The Patientsmentioning

confidence: 99%

Usefulness of a scoring system in the interpretation ofhistology in neonatal cholestasis

Lee¹,

Looi²

2009

WJG

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AIM:To ascertain the usefulness of a histological scoring system devised to assist in the interpretation of liver histology in neonatal cholestasis (NC). METHODS:Liver biopsy specimens obtained from infants with NC referred to a tertiary pediatric unit in Malaysia were prospectively studied. The first author, blinded to the final diagnosis, devised the histological diagnosis based on a 7-feature (portal ductal proliferation, bile plugs in portal ductules, portoportal bridging, lymphocytic infiltration in portal region, multinucleated hepatocytes, neutrophilic infiltration, hepatocellular swelling), 15-point (0 to 15) scoring system. The author classified the histological diagnosis as either biliary atresia (BA) or neonatal hepatitis (NH, all other diagnoses), and subsequently compared the author's diagnosis with the final diagnosis. RESULTS:Eighty-four biopsy specimens obtained from 78 patients were reviewed. Without the scoring system, BA was correctly diagnosed by the author histologically in 30 cases, labelled as NH in 3. For other diagnoses, BA was excluded correctly in 33 cases and mislabeled as BA in 2 cases. The overall sensitivity for BA was 91%, specificity 86% and accuracy 88%. With the scoring system, a score of ≥ 7 had the best diagnostic utility to differentiate BA from other intrahepatic cholestasis histologically (sensitivity 88%, specificity 94%, accuracy 92%). Four patients with a score < 7 had BA, and 3 patients with a score ≥ 7 had NH. CONCLUSION:A 7-feature, 15-point histological scoring system had good diagnostic accuracy in the interpretation of liver histology in neonatal cholestasis.

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Outcome of biliary atresia in Malaysia: A single‐centre study

Cited by 21 publications

References 25 publications

Seventeen years of Kasai portoenterostomy for biliary atresia in a single Southeast Asian paediatric centre

Seventeen years of Kasai portoenterostomy for biliary atresia in a single Southeast Asian paediatric centre

Biliary atresia: the Brazilian experience

Usefulness of a scoring system in the interpretation ofhistology in neonatal cholestasis

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