|4.||End Stage Liver Disease
|5.||Liver Diseases (Liver Disease)
|1.||Poovorawan, Yong: 24 articles (07/2013 - 01/2002)|
|2.||Bezerra, Jorge A: 23 articles (12/2014 - 11/2002)|
|3.||Chen, Chao-Long: 21 articles (11/2015 - 02/2004)|
|4.||Chongsrisawat, Voranush: 20 articles (02/2013 - 01/2002)|
|5.||Sokol, Ronald J: 19 articles (12/2014 - 11/2002)|
|6.||Vejchapipat, Paisarn: 18 articles (07/2013 - 07/2004)|
|7.||Mizuta, Koichi: 16 articles (04/2015 - 01/2004)|
|8.||Chuang, Jiin-Haur: 15 articles (11/2015 - 02/2004)|
|9.||Mack, Cara L: 13 articles (08/2015 - 07/2004)|
|10.||Magee, John C: 13 articles (05/2015 - 03/2003)|
|1.||Bile Acids and Salts (Bile Acids)IBA
07/10/2012 - "We discovered that the sea lamprey adapts to biliary atresia through a unique mechanism of de novo synthesis and secretion of bile salts in intestine after developmental biliary atresia, in addition to known mechanisms, such as the reduction of bile salt synthesis in liver. "
07/10/2012 - "Intestinal synthesis and secretion of bile salts as an adaptation to developmental biliary atresia in the sea lamprey."
01/01/2012 - "Elevated bile acids in newborns with Biliary Atresia (BA)."
08/21/1999 - "Most children who present with extrahepatic biliary atresia and other forms of cholestatic hepatobiliary disease have significantly raised concentrations of conjugated bile acids as measured by tandem mass spectrometry at the time when samples are taken for the Guthrie test. "
08/21/1999 - "To assess the feasibility of screening for cholestatic hepatobiliary disease and extrahepatic biliary atresia by using tandem mass spectrometry to measure conjugated bile acids in dried blood spots obtained from newborn infants at 7-10 days of age for the Guthrie test. "
|2.||Octreotide (Sandostatin)FDA LinkGeneric
04/01/2008 - "These results (1) indicate that primary prevention of GI bleeding by sclerotherapy of esophageal varices is technically feasible and fairly effective in infants with biliary atresia and large varices, even in those with end-stage liver disease, (2) suggest that decreasing the risk of bleeding may allow liver transplantation under better conditions, and (3) further suggest that octreotide associated with sclerotherapy lowers the rate of complications."
12/01/2004 - "Herein, the authors report a case of refractory chylous ascites after Kasai operation for biliary atresia, which was treated successfully with subcutaneous octreotide, a synthetic somatostatin analogue. "
|3.||Rifampin (Rifampicin)FDA LinkGeneric
07/01/1993 - "To determine the efficacy of rifampicin as a treatment for refractory pruritus, the medical records of 33 children (median age 25 months, range 4-135; 19 boys) with chronic cholestasis liver disease (21 with Alagille's syndrome, eight with progressive intrahepatic cholestasis, one with extrahepatic biliary atresia, one with an inborn error of bile acid metabolism, and one with cryptogenic cirrhosis) were reviewed retrospectively. "
05/01/2008 - "We present a case of a 17-month-old child with congenital biliary atresia with generalized pruritus refractory to treatment with oral antihistamines, topical steroids, cholestyramine, and rifampin but improved remarkably with the addition of naltrexone. "
01/01/2013 - "The aims of this study were to evaluate the specificity of a non-draining hepatobiliary scintigraphy (HBS) for biliary atresia (BA) in preterm and full-term babies, to verify the relationship between non-draining scan and higher levels of direct bilirubin and to find an objective criterion to guide the time in performing HBS. "
05/01/1985 - "Studies on the changes of serum bilirubin level after surgery in biliary atresia."
12/01/2015 - "Published analyses indicate that newborn screening for biliary atresia by using serum bilirubin concentrations or stool color cards is potentially life-saving and cost-effective. "
08/01/2015 - "The total amount of conjugated bilirubin above 20 μmol/l is sufficient to require further evaluation for biliary atresia. "
08/01/2015 - "Biliary atresia has to be excluded if conjugated bilirubin level is above than 20 μmol/l, and/or more than 20% of total bilirubin. "
|5.||Ursodeoxycholic Acid (Urso)FDA LinkGeneric
07/01/2008 - "In this cohort of infants with extrahepatic biliary atresia, ursodeoxycholic acid was not shown to be effective, and its use was associated with a plethora of hepatic and extrahepatic complications."
12/01/2008 - "Our study demonstrates the beneficial effect of ursodeoxycholic acid on liver function in children after successful surgery for biliary atresia."
12/01/2008 - "The aim of this study was to assess the effect of ursodeoxycholic acid treatment on liver function in children who underwent successful surgery for biliary atresia. "
07/01/2008 - "The objective of this study is to review ursodeoxycholic acid in infants and children with extrahepatic biliary atresia. "
12/01/2008 - "Effect of ursodeoxycholic acid on liver function in children after successful surgery for biliary atresia."
|6.||Phenobarbital (Luminal)FDA Link
10/01/2013 - "Per our routine protocol the scans were obtained after pretreatment with phenobarbital (5 mg/kg/day for 5 days) to achieve a serum level of ≥15 mcg/ml. Normal hepatic uptake with no biliary excretion by 24 h was considered consistent with biliary atresia. "
10/01/2013 - "Phenobarbital-enhanced hepatobiliary scintigraphy in the diagnosis of biliary atresia: two decades of experience at a tertiary center."
03/01/1996 - "Biliary atresia (BA) is characterized by luminal obstruction of the extrahepatic bile duct with fibrous remnants. "
12/01/1989 - "Our results point to hepatobiliary scintigraphy after phenobarbital as the best noninvasive procedure for both diagnosis and postoperative follow-up of biliary atresia. "
08/29/1970 - "Phenobarbitone in intrahepatic biliary atresia."
05/07/2014 - "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. "
09/01/2015 - "However, more RCTs with longer follow-up are necessary to demonstrate the effect of steroids on the long-term outcomes of biliary atresia."
11/01/2014 - "Postoperative steroids after Kasai portoenterostomy for biliary atresia: a meta-analysis."
11/01/2013 - "The effect of adjuvant steroids in infants with biliary atresia (BA) is not clear and evidence of benefit is lacking. "
11/01/2013 - "Steroids in biliary atresia: single surgeon, single centre, prospective study."
|8.||Adrenal Cortex Hormones (Corticosteroids)IBA
07/01/1997 - "Controversy exists regarding the efficacy of corticosteroids on bile flow after Kasai portoenterostomy in biliary atresia (BA). "
12/01/2007 - "The objective of this study was to evaluate adjuvant corticosteroids after Kasai portoenterostomy for biliary atresia. "
12/01/2007 - "Randomized, double-blind, placebo-controlled trial of corticosteroids after Kasai portoenterostomy for biliary atresia."
05/07/2014 - "Use of corticosteroids after hepatoportoenterostomy for bile drainage in infants with biliary atresia: the START randomized clinical trial."
11/01/2013 - "We aimed to evaluate early response to two different corticosteroids doses after Kasai portoenterostomy for biliary atresia (BA). "
|9.||Trimethoprim-Sulfamethoxazole Combination (Co-Trimoxazole)FDA LinkGeneric
01/01/1996 - "Pharmacokinetics for combination trimethoprim/ sulfamethoxazole (TMP/SMX) was studied in only four patients with biliary atresia (BA): three girls, 6.2,8.0 and 8.2 years of age and one boy 8.4 years of age, as this is an uncommon obstructive anomaly of the extrahepatic biliary system and has been described as having a poor prognosis. "
01/01/1996 - "Kinetic effects of trimethoprim-sulfamethoxazole in children with biliary atresia: a new dosing regimen."
03/01/1994 - "Trimethoprim/sulfamethoxazole kinetics in children with biliary atresia."
10/01/1981 - "Trimethoprim-sulfamethoxazole for cholangitis following hepatic portoenterostomy for biliary atresia."
04/01/2003 - "The aim of this study was to evaluate the efficacy of trimethoprim-sulfamethoxazole (TMP/SMZ) and neomycin as the prophylactic agents against the recurrence of cholangitis in children with biliary atresia (BA) after a Kasai portoenterostomy. "
|10.||Dinoprostone (PGE2)FDA Link
10/01/1989 - "In conclusion the PGE2 has choleretic action in biliary atresia and could be a coadjuvant treatment to improve the bile flow."
10/01/1989 - "In the last four years PGE2 was used in ten cases with extrahepatic biliary atresia. "
10/01/1989 - "We studied the choleretic effect of Prostaglandin E2 (PGE2) in patients with biliary atresia. "
10/01/1989 - "[Effect of prostaglandin (PGE2) in biliary atresia]."
08/01/1993 - "There is no clinical study proving the efficacy of either bile acids or prostaglandin E2 in biliary atresia. "
12/01/2011 - "Surgical treatment with Kasai portoenterostomy has improved the prognosis for patients with biliary atresia, although most patients ultimately require liver transplantation. "
11/01/2000 - "Although the prognosis of biliary atresia has been dramatically improved in the era of liver transplantation, the Kasai operation is still the first line of surgical treatment. "
10/21/1995 - "The need for paediatric liver transplantation, which in most paediatric series is the remedy for biliary atresia after Kasai's operation has failed, is not sufficiently covered by organ retrieval at the present time. "
03/01/2008 - "Although living-related liver transplantation (LRLT) is effective for patients with biliary atresia (BA) after a failed Kasai operation, the pretransplant factors affecting post-LRLT mortality and the optimal timing of the procedure remain unclear. "
12/01/2010 - "Living-donor liver transplantation is an effective treatment of biliary atresia, with good long-term outcome. "
05/01/1994 - "Hepatic portoenterostomy was successful in 28 (32%) of 87 patients with biliary atresia (all younger than 3 months), and 23 showed improvement following hepatic portoenterostomy; hepatic portoenterostomy failed in 36 patients. "
06/01/2013 - "Treatment for patients with biliary atresia is a Kasai hepatic portoenterostomy; however, the efficacy of repeat Kasai hepatic portoenterostomy is unclear. "
03/01/2001 - "The outcome of the hepatic portoenterostomy (Kasai) procedure for biliary atresia is improved when it is performed before 90 days of age. "
06/01/1982 - "Improved results with hepatic portoenterostomy: a reassessment of its value in the treatment of biliary atresia."
06/01/1980 - "Surgical intervention utilizing the Kasai hepatic portoenterostomy has improved the outcome of patients with biliary atresia and provided a population of patients with unique health problems. "
06/01/2009 - "The aim of this study was to investigate from the perspectives of the donor parents of children with biliary atresia, the essence of family experiences during the period when waiting for a living donor liver transplant. "
12/01/2015 - "Liver transplant for biliary atresia (BA) has been reported to be associated with worse outcome, but this remains controversial. "
12/01/2015 - "Liver transplant for biliary atresia is associated with a worse outcome - Myth or fact?"
11/01/2015 - "Immunohistochemical characterization of the regenerative compartment in biliary atresia: a comparison between Kasai procedure and transplant cases."
10/01/2015 - "A cryopreserved iliac artery graft was used for the reconstruction of the portal vein in a 29-month-old infant with biliary atresia. "
07/01/2008 - "The gold standard for diagnosis of biliary atresia was the appearance of the extrahepatic biliary tree via laparotomy. "
01/01/2005 - "Exploratory laparotomy with cholecystocholangiography is often performed for the definitive diagnosis of biliary atresia (BA) when radiological and biochemical studies are inconclusive. "
07/01/2010 - "Furthermore, for patients without biliary atresia, unnecessary laparotomy can be avoided."
12/01/2009 - "Endoscopic retrograde cholangiopancreaticography prior to explorative laparotomy avoids unnecessary surgery in patients suspected for biliary atresia."
01/01/2009 - "A laparotomy could be prevented in 12% of children with suspicion of biliary atresia. "
11/01/1999 - "To determine the safety and effectiveness of endoscopic injection sclerotherapy (EIS) for children with biliary atresia. "
11/01/2012 - "Endoscopic surveillance and primary prophylaxis sclerotherapy of esophageal varices in biliary atresia."
04/01/2008 - "We report the results of prophylactic sclerotherapy in 13 infants with biliary atresia and large varices. "
04/01/2008 - "Prophylactic endoscopic sclerotherapy of large esophagogastric varices in infants with biliary atresia."
11/01/1999 - "Endoscopic injection sclerotherapy for esophagogastric variceal bleeding in children with biliary atresia."