Seamless management of biliary atresia in England and Wales (1999–2002)

Davenport, Mark; Goyet, Jean deVille de; Stringer, Mark D.; Mieli‐Vergani, Giorgina; Kelly, D A; McClean, Patricia; Spitz, L

doi:10.1016/s0140-6736(04)16045-5

Cited by 282 publications

(264 citation statements)

References 14 publications

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“…The proportions who achieved clearance of jaundice (bilirubin Ͻ 20 mol/L) at 6 and 12 months were similar to our previously reported experience (ϳ50%) 6 and were not significantly different from each other. Similarly, although there was a reduced need for transplantation at 12 months in the steroid group (26% versus 35%), this was not statistically significant.…”

Section: Discussionsupporting

confidence: 88%

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

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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Section: Discussionsupporting

confidence: 88%

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

et al. 2007

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“…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: 82%

“…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 Originsupporting

confidence: 48%

“…Experiences from other countries have proved that late referral can be changed by improving medical practices and health policies. The United Kingdom adopted a policy of centralization in 1999, limiting treatment of patients with BA to three reference centers, 14 while France, in 1997, introduced a collaborative effort among their various national centers. 13 Other countries have adopted measures to raise awareness of their population with warning signs and screening systems for BA, such as "Yellow Alert" campaigns 37 and the use of a colorimetric scale to identify acholic stools.…”

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.…”

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confidence: 99%

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Atresia biliar: a experiência Brasileira

Carvalho¹,

Santos

Silveira

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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Use of Corticosteroids After Hepatoportoenterostomy for Bile Drainage in Infants With Biliary Atresia

Bezerra

Spino

Magee

et al. 2014

JAMA

163

133

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IMPORTANCE Biliary atresia is the most common cause of end-stage liver disease in children. Controversy exists as to whether use of steroids after hepatoportoenterostomy improves clinical outcome. OBJECTIVE To determine whether the addition of high-dose corticosteroids after hepatoportoenterostomy is superior to surgery alone in improving biliary drainage and survival with the native liver. DESIGN, SETTING, AND PATIENTS The multicenter, double-blind Steroids in Biliary Atresia Randomized Trial (START) was conducted in 140 infants (mean age, 2.3 months) between September 2005 and February 2011 in the United States; follow-up ended in January 2013. INTERVENTIONS Participants were randomized to receive intravenous methylprednisolone (4 mg/kg/d for 2 weeks) and oral prednisolone (2 mg/kg/d for 2 weeks) followed by a tapering protocol for 9 weeks (n = 70) or placebo (n = 70) initiated within 72 hours of hepatoportoenterostomy. MAIN OUTCOMES AND MEASURES The primary end point (powered to detect a 25% absolute treatment difference) was the percentage of participants with a serum total bilirubin level of less than 1.5 mg/dL with his/her native liver at 6 months posthepatoportoenterostomy. Secondary outcomes included survival with native liver at 24 months of age and serious adverse events. RESULTS The proportion of participants with improved bile drainage was not statistically significantly improved by steroids at 6 months posthepatoportoenterostomy (58.6% [41/70] of steroids group vs 48.6% [34/70] of placebo group; adjusted relative risk, 1.14 [95% CI, 0.83 to 1.57]; P = .43). The adjusted absolute risk difference was 8.7% (95% CI, −10.4% to 27.7%). Transplant-free survival was 58.7% in the steroids group vs 59.4% in the placebo group (adjusted hazard ratio, 1.0 [95% CI, 0.6 to 1.8]; P = .99) at 24 months of age. The percentage of participants with serious adverse events was 81.4% [57/70] of the steroids group and 80.0% [56/70] of the placebo group (P > .99); however, participants receiving steroids had an earlier time of onset of their first serious adverse event by 30 days posthepatoportoenterostomy (37.2% [95% CI, 26.9% to 50.0%] of steroids group vs 19.0% [95% CI, 11.5% to 30.4%] of placebo group; P= .008). CONCLUSIONS AND RELEVANCE Among infants with biliary atresia who have undergone hepatoportoenterostomy, high-dose steroid therapy following surgery did not result in statistically significant treatment differences in bile drainage at 6 months, although a small clinical benefit could not be excluded. Steroid treatment was associated with earlier onset of serious adverse events in children with biliary atresia.

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Seamless management of biliary atresia in England and Wales (1999–2002)

Cited by 282 publications

References 14 publications

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

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

Atresia biliar: a experiência Brasileira

Use of Corticosteroids After Hepatoportoenterostomy for Bile Drainage in Infants With Biliary Atresia

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