***TASK*** the task is to summarize an input biomedical literature in six sentences 
    ***INPUT*** the input is a biomedical literature 
    ***OUTPUT*** the output is the summary of an input biomedical literature in six sentences 
    ***DOCUMENTATION*** 
    ***EXAMPLES*** 
    Input: Abstract Purpose We aim ed to evaluate early response to two different corticosteroids doses after Kasai portoenterostomy for biliary atresia ( BA ) . Methods A prospect i ve , r and omized trial was performed in infants from the nationwide BA registry with type 3 BA . Sixty-nine infants were r and omized to receive either 4 mg/kg/day ( group A , n = 35 ) or 2 mg/kg/day prednisolone ( group B , n = 34 ) . The corticosteroids were started on postoperative day 7 , and the dose was tapered toward day 30 . Results of liver function tests on days 31 and 60 were compared between the groups . Results Mean bilirubin , AST , ALT , and GGT levels did not significantly differ between the groups . However , the levels of total and direct bilirubin of infants < 70 days old at surgery significantly differed between the groups . Four patients from group A and five from group B , dropped out of the study . Complications during the first month after PE were comparable between the groups . Conclusions An initial 4 mg/kg/day dose did not significantly improve liver function , except that bilirubin levels were lower in the subgroup of infants < 70 days old at surgery . There were no significant complications with either dose of corticosteroids Objective : To identify the epidemiological characteristics of infants with biliary atresia in Engl and and Wales , since central isation of its management in 1999 . Methods : The care of infants with biliary atresia ( BA ) in Engl and and Wales is central ised to only three centres . All infants ( treated from January 1999 to December 2006 ) were identified from a prospect i ve national data base ; demographic details were ascertained from medical records and compared between two groups based on presumed aetiology ( isolated biliary atresia ( IBA ) and developmental biliary atresia ( DBA ) ( for example , syndromic infants , biliary atresia splenic malformation , cystic biliary atresia ) ) . Results : There were 302 ( 133 male ( 44 % ) ) infants with BA that could be divided into IBA ( n = 219 , 73 % ) and DBA ( n = 76 , 25 % ) . The overall incidence was 0.58/10 000 ( 1 in 17 049 ) live births with marked regional differences along a north-west/south-east axis varying from 0.38 ( north-west Engl and ) to 0.78 ( south-east Engl and ) /10 000 live births ( OR 2.05 ( 95 % CI 1.26–3.41 ) ; p = 0.002 ) . The commonest month of birth was September with December being the least common , although there was no evidence for significant seasonal variation ( p = 0.2 ) . Infants with DBA were more likely to be female ( p<0.001 ) , of white background ( p = 0.01 ) , first-born ( p = 0.04 ) and to be formula-fed ( p = 0.07 ) . Infants of south Asian origin came to surgery at an older age ( 59 ( IQ 45–75 ) versus 52 ( IQ 42–65 ) days ; p = 0.03 ) . Conclusions : There is a remarkable variation of incidence of biliary atresia within Engl and and Wales , some of which may have been caused by factors related to a different aetiological and racial background OBJECTIVE : Postoperative adjuvant steroid treatment is reported to improve jaundice-free survival in biliary atresia ( BA ) patients and to reduce the need for early liver transplantation . However , evidence of all retrospective studies is very limited , although high-dose corticosteroids were favored . The aim of this dosage finding study was to test the most promising corticosteroid protocol in a smaller but representative series , in order to optimize the setting s of upcoming prospect i ve and long-term multicenter studies . METHODS : Our prospect i ve single-center and open-labeled pilot study on high-dose steroids included 49 consecutive BA patients . Basic data of the study group were not different from 29 controls . In the study group , 20 consecutive patients were treated after the Kasai with methylprednisolone ( 10 mg/kg day 1 to 5 and 1 mg/kg day 6 to 28 ) . RESULTS : Overall survival with native liver was 63 % after 6 months and 31 % after 2 yr , with no statistical difference between the study and control groups . After 2 yr , 27 % of all patients were still jaundice-free . With regard to predictive parameters , we found , 6 months after the Kasai , bilirubin < 20 μmol/L as highly sensitive ( 97 % ) and specific ( 93 % ) for jaundice-free survival with native liver . CONCLUSIONS : In contrast to previous reports , this pilot study shows that high-dose steroid pulses after Kasai procedure are not effective in postoperative adjuvant therapy protocol s and should be avoided in upcoming multicenter steroid studies . Therefore , we recommend extended and r and omized multicenter studies to pre-evaluate the supposed effectiveness of alternative steroid protocol s , by comparing , 6 months after the Kasai procedure , the number of patients with normal bilirubin BACKGROUND AND AIMS Perinatal cytomegalovirus ( CMV ) infection is a possible cause or trigger of biliary atresia though clinical evidence is scant . We hypothesised that CMV IgM+ve biliary atresia is a separate clinical entity compared to CMV IgM-ve biliary atresia . METHODS Prospect i ve single-centre study . 210 infants with histologically confirmed biliary atresia were treated in our institution ( Jan. 2004 to Dec. 2011 ) ; of these 20 ( 9.5 % ) were CMV IgM+ve at presentation . We compared these with 111 infants who were CMV IgM-ve ( controls )  for clinical features , biochemistry at presentation and outcome following Kasai portoenterostomy ( KPE ) . A blinded comparison of age-matched liver histology was also performed . Data are quoted as median ( interquartile range ) . A P value ≤ 0.05 was regarded as significant . RESULTS Infants with CMV IgM+ve biliary atresia were older at Kasai portoenterostomy ( or laparotomy ) [ 70 ( 60 - 80 ) days vs. 56 (44 - 75)days ; P = 0.003 ] and were more jaundiced [ 175 ( 147 - 224 ) vs. 140 ( 121 - 181 ) μmol/L ; P = 0.002 + with higher AST*287 ( 157 - 403 ) vs. 180 ( 133 - 254 ) IU/L ; P = 0.005 ] and aspartate aminotransferase-to-platelet ratio index [ 1.1 ( 0.79 - 3.0 ) vs. 0.63 ( 0.43 - 0.95 ) ] levels . Liver histology : CMV IgM+ve biliary atresia was characterised by a greater degree of inflammation ( P < 0.0001 ) and fibrosis ( P = 0.02 ) , whereas CMV IgM-ve isolated biliary atresia had a higher degree of lobular cholestasis ( P = 0.001 ) . This effect was independent of the effects of age at KPE . OUTCOME CMV IgM+ve biliary atresia had a poorer outcome with a reduced clearance of jaundice ( 15 % vs. 52.2 % ; P = 0.002 ) , native liver survival ( P < 0.0001 ) and increased mortality ( P = 0.002 ) . CONCLUSIONS CMV IgM+ve biliary atresia is a distinct clinical and pathological entity with a diminished response to Kasai portoenterostomy 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 R and omized 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 r and omized 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 . TRIAL REGISTRATION clinical trials.gov Identifier : NCT00294684 BACKGROUND We studied the outcomes at school age in children who had participated in a double-blind , placebo-controlled trial of early postnatal dexamethasone therapy ( initiated within 12 hours after birth ) for the prevention of chronic lung disease of prematurity . METHODS Of the 262 children included in the initial study , 159 lived to school age . Of these children , 146 ( 72 in the dexamethasone group and 74 in the control group ) were included in our study . All the infants had had severe respiratory distress syndrome requiring mechanical ventilation shortly after birth . In the dexamethasone group , 0.25 mg of dexamethasone per kilogram of body weight was given intravenously every 12 hours for one week , and then the dose was tapered . We evaluated the children 's growth , neurologic and motor function , cognition , and school performance . RESULTS Children in the dexamethasone group were significantly shorter than the controls ( P=0.03 for boys , P=0.01 for girls , and P=0.03 for all children ) and had a significantly smaller head circumference ( P=0.04 ) . Children in the dexamethasone group had significantly poorer motor skills ( P<0.001 ) , motor coordination ( P<0.001 ) , and visual-motor integration ( P=0.02 ) . As compared with the controls , children in the dexamethasone group also had significantly lower full IQ scores ( mean [ + /-SD ] , 78.2+/-15.0 vs. 84.4+/-12.6 ; P=0.008 ) , verbal IQ scores ( 84.1+/-13.2 vs. 88.4+/-11.8 , P=0.04 ) , and performance IQ scores ( 76.5+/-14.6 vs. 84.5+/-12.7 , P=0. 
    Output:
    
Clearance of jaundice is related to the age at KPE in infants who receive high-dose steroids .
Native liver survival appears to be improved as a result of this .
This is the first study to show tangible longer-term benefit from high-dose steroids in biliary atresia