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Biliary atresia

Kristin Bjørnland, Maren Hinna, Gunnar Aksnes, Kjetil Juul Stensrud, Kjetil Ertresvåg, Anniken Bjørnstad-Østensen, Truls Sanengen, Pål-Dag Line, Ingegerd Aagenæs, Lars Aabakken, Ragnhild Emblem, Runar Almaas
OBJECTIVES: The importance of case load for treatment of extrahepatic biliary atresia (BA) is debated. The aim of this study was to register results of BA treatment in a small volume centre. METHODS: Retrospective chart review study of patients with BA treated from 2000 to 2017. The institutional review board approved the study. RESULTS: Forty-five babies were identified of which 42 (93%) are alive. Forty-one patients had a Kasai portoenterostomy (PE), two had a hepaticojejunostomy and two had a primary liver transplantation...
March 16, 2018: Scandinavian Journal of Gastroenterology
M Garrido, C Escobar, C Zamora, C Rejas, J Varas, C Córdova, C Papuzinski, M Párraga, S San Martín, S Montedonico
Purpose: Biliary atresia precedes liver cirrhosis and liver transplantation. Amniotic membrane (AM) promotes tissue regeneration, inhibits fibrosis, and reduces inflammation. Here, we test amniotic membrane potential as a therapeutic tool against cholestatic liver fibrosis. Methods: Three groups of rats were used: sham surgery (SS), bile duct ligature (BDL), and bile duct ligature plus human amniotic membrane (BDL + AM). After surgery, animals were sacrificed at different weeks...
2018: Stem Cells International
Toshihiro Yasui, Tatsuya Suzuki, Fujio Hara, Shunsuke Watanabe, Naoko Uga, Atsuki Naoe, Yasuhiro Kondo
OBJECTIVES: In pediatric patients, renal dysfunction after living-donor liver transplant is a major issue that is difficult to evaluate. Recently, predictive equations for Japanese children have been introduced. MATERIALS AND METHODS: We conducted a retrospective study by prospectively collecting data on 26 patients under 16 years old who underwent living-donor liver transplant between June 2004 and March 2015. Serum creatinine and cystatin C levels were measured...
March 9, 2018: Experimental and Clinical Transplantation
Nehal El-Koofy, Hanan M Fouad, Mona E Fahmy, Heba Helmy, Olfat Shaker, Hanaa M El-Karaksy, Nabil Mohsen
BACKGROUND AND STUDY AIMS: Hepatobiliary cholestatic disorders produce excess copper (Cu) retention in the liver, which is toxic and may cause hepatitis, fulminant hepatic failure, cirrhosis and death. In this study, we measured hepatic Cu and tested its correlation with serum Cu (S. Cu) and serum ceruloplasmin (S. ceruloplasmin) in cholestatic infants. PATIENTS AND METHODS: 41 cholestatic infants were enrolled as cases and 11 healthy infants as control subjects...
March 6, 2018: Arab Journal of Gastroenterology: the Official Publication of the Pan-Arab Association of Gastroenterology
Vicky L Ng, Lisa G Sorensen, Estella M Alonso, Emily M Fredericks, Wen Ye, Jeff Moore, Saul J Karpen, Benjamin L Shneider, Jean P Molleston, Jorge A Bezerra, Karen F Murray, Kathleen M Loomes, Philip Rosenthal, Robert H Squires, Kasper Wang, Ronen Arnon, Kathleen B Schwarz, Yumirle P Turmelle, Barbara H Haber, Averell H Sherker, John C Magee, Ronald J Sokol
OBJECTIVES: To assess neurodevelopmental outcomes among participants with biliary atresia with their native liver at ages 12 months (group 1) and 24 months (group 2), and to evaluate variables predictive of neurodevelopmental impairment. STUDY DESIGN: Participants enrolled in a prospective, longitudinal, multicenter study underwent neurodevelopmental testing with either the Bayley Scales of Infant Development, 2nd edition, or Bayley Scales of Infant and Toddler Development, 3rd edition...
March 5, 2018: Journal of Pediatrics
Jim C H Wilde, Ana M Calinescu, Barbara E Wildhaber
Biliary atresia (BA) and congenital choledochal malformations (CCM) are rare. Both pathologies must (BA) or may (CCM) be operated during the neonatal period or early infancy. We briefly describe the classical operative techniques for both pathologies, followed by the most common and severe perioperative complications encountered during and up to 30 days after surgery in children operated for BA and CCM early in life. For patients with BA, intestinal complications represent the most common and hazardous perioperative surgical complications...
March 6, 2018: European Journal of Pediatric Surgery
Wison Laochareonsuk, Piyawan Chiengkriwate, Surasak Sangkhathat
BACKGROUND: A genome-wide association study in East Asians suggested a genetic association between biliary atresia (BA) and a cluster of variants within the Adducin 3 (ADD3) and ADD3 antisense RNA1 (ADD3-AS1) genes. Another study in Thai neonates reported an association between BA and rs17095355. To validate those findings, this study aimed to analyze the BA association with single nucleotide polymorphisms (SNPs) and the additive influence of ADD3 and ADD3-AS1 in Thai neonates. METHODS: DNAs from 56 BA cases and 166 controls were genotyped for rs2501577, rs11194981, rs12268910 (ADD3) and rs17095355 (ADD3-AS1), using TaqMan PCR...
March 5, 2018: Pediatric Surgery International
Keigo Murakami, Shuko Hata, Yasuhiro Miki, Hironobu Sasano
Background A potential correlation between sex hormones, such as androgens and estrogens, and the development and progression of hepatocellular carcinoma (HCC) has been proposed. However, its details, in particular, aromatase status in diseased human liver has remained largely unknown. Materials and methods We immunolocalized aromatase, 17β-hydroxysteroid dehydrogenase (17β-HSD) type 1 and 17β-HSD type 2 in a total of 155 cases, consisting of normal liver (n = 10), nonalcoholic steatohepatitis (NASH) (n = 18), primary sclerosing cholangitis (PSC) (n = 6), primary biliary cholangitis (PBC) (n = 13), biliary atresia (n = 18), alcoholic hepatitis (n = 11), hepatitis C virus (HCV) (n = 31), HCV sustained virologic response (HCV-SVR) (n = 10), hepatitis B virus (HBV) (n = 20), HBV sustained virologic response (HBV-SVR) (n = 8) and infants (n = 10)...
February 28, 2018: Hormone Molecular Biology and Clinical Investigation
Jia-Feng Wu, Chee-Seng Lee, Wen-Hsi Lin, Yung-Ming Jeng, Huey-Ling Chen, Yen-Hsuan Ni, Hong-Yuan Hsu, Mei-Hwei Chang
We investigated the utility of transient elastography for diagnosing biliary atresia (BA) in cholestatic infants and predicting the outcome of BA. Forty-eight cholestatic infants (9-87 days of age) with direct bilirubin level > 1 mg/dL were enrolled. Liver stiffness measurement (LSM) by transient elastography was performed during the cholestasis workup, and 15 subjects were diagnosed as BA. We assessed liver histology using liver biopsies from 36 subjects and graded fibrosis status using the METAVIR score...
February 27, 2018: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
Yong Hee Kim, Myung-Joon Kim, Hyun Joo Shin, Haesung Yoon, Seok Joo Han, Hong Koh, Yun Ho Roh, Mi-Jung Lee
OBJECTIVES: To evaluate MRI findings and to generate a decision tree model for diagnosis of biliary atresia (BA) in infants with jaundice. METHODS: We retrospectively reviewed features of MRI and ultrasonography (US) performed in infants with jaundice between January 2009 and June 2016 under approval of the institutional review board, including the maximum diameter of periportal signal change on MRI (MR triangular cord thickness, MR-TCT) or US (US-TCT), visibility of common bile duct (CBD) and abnormality of gallbladder (GB)...
February 23, 2018: European Radiology
Yanan Li, Bo Xiang, Yang Wu, Chuan Wang, Qi Wang, Yiyang Zhao, Siyuan Chen, Yi Ji, Zhicheng Xu
OBJECTIVE: The aim of this study was to evaluate the mid-term results of laparoscopic Kasai portoenterostomy (LKPE) for biliary atresia (BA) at our hospital. METHODS: From May 2009 to May 2012, the charts of infants with BA who underwent LKPE were reviewed retrospectively. The clearance of jaundice (CJ) as well as 3-year, and 5-year survival with native liver (SNL) rates were analyzed and compared with those after open Kasai portoenterostomy (OKPE). RESULTS: Forty-nine patients with BA who underwent LKPE were assigned to group A, and 40 patients with BA who underwent OKPE were assigned to group B...
February 21, 2018: Journal of Pediatric Gastroenterology and Nutrition
Naman S Shetty, Ira Shah
Kawasaki's disease (KD) is a systemic vasculitis often seen with viral and bacterial infections. Cholangitis is a known complication in biliary atresia patients post Kasai Portoenterostomy (KP). However KD, in a biliary atresia patient post KP has not been previously reported. A 1 years old girl who had previously undergone a KP for BA, presented with cholangitis which was presumed to be caused by a previous enterobacter infection that she had 2 months ago. However, on treating the cholangitis, the patient developed fever again after ten days which persisted even after changing the antibiotics...
March 1, 2018: Annals of Hepatology
Dandan Li, Pei Wang, Ying He, Chunlei Jiao, Didi Zhuansun, Nannan Wei, Jixin Yang, Jiexiong Feng
OBJECTIVE: To assess the efficacy of intravenous immunoglobulin (IVIG) as add-on treatment for intractable cholangitis (IC) after Kasai portoenterostomy (KPE) in biliary atresia (BA) patients. METHODS: 113 BA patients who had one or more episodes of cholangitis after KPE were recruited in this study. According to whether response to routine conservative treatment, all patients were divided into IC group and simple cholangitis (SC) group. Meanwhile, patients with IC subdivided into IVIG group and control group according to whether application of IVIG...
February 21, 2018: Pediatric Surgery International
Yohei Yamada, Ken Hoshino, Takayuki Oyanagi, Ryohei Gatayama, Jun Maeda, Nobuyuki Katori, Yasushi Fuchimoto, Taizo Hibi, Masahiro Shinoda, Kentaro Matsubara, Hideaki Obara, Ryo Aeba, Yuko Kitagawa, Hiroyuki Yamagishi, Tatsuo Kuroda
Children with single ventricle physiology have complete mixing of the pulmonary and systemic circulations, requiring staged procedures to achieve a separation of these circulations, or Fontan circulation. The single ventricle physiology significantly increases the risk of mortality in children undergoing non-cardiac surgery. As liver transplantation for patients with single ventricle physiology is particularly challenging, only a few reports have been published. We herein report a case of successful LDLTx for an 8-month-old pediatric patient with biliary atresia, heterotaxy, and complex heart disease of single ventricle physiology...
February 19, 2018: Pediatric Transplantation
Juan S Rubio, Carolina Rumbo, Pablo A Farinelli, Nicolás Aguirre, Diego A Ramisch, Hugo Paladini, Pablo D Angelo, Pablo Barros Schelotto, Gabriel E Gondolesi
Collateral circulation secondary to liver cirrhosis may cause the development of large PSSs that may steal flow from the main portal circulation. It is important to identify these shunts prior to, or during the transplant surgery because they might cause an insufficient portal flow to the implanted graft. There are few reports of "steal flow syndrome" cases in pediatrics, even in biliary atresia patients that may have portal hypoplasia as an associated malformation. We present a 12-month-old female who received an uneventful LDLT from her mother, and the GRWR was 4...
February 16, 2018: Pediatric Transplantation
Suk Kyun Hong, Kyung-Suk Suh, Hyo-Sin Kim, Kyung Chul Yoon, Sung-Woo Ahn, Hyeyoung Kim, Nam-Joon Yi, Kwang-Woong Lee
BACKGROUND: Improvements in laparoscopic imaging systems and instruments have increased the performance of pure laparoscopic living donor hepatectomy. This operation is no longer limited to left lateral sectionectomy but is used for left hepatectomy and right hepatectomy.1-5 This report describes a donor who underwent pure laparoscopic left lateral sectionectomy and in situ reduction using 3D laparoscopy and indocyanine green (ICG) near-infrared fluorescence cholangiography to obtain a monosegment...
February 12, 2018: Journal of Gastrointestinal Surgery: Official Journal of the Society for Surgery of the Alimentary Tract
Xiu-Qi Chen, Wen-Hai Tan, Fang-Fang Jiang, Xun Chen
OBJECTIVE: To investigate the clinical features of invasive pulmonary fungal infections (IPFIs) after biliary atresia (BA) surgery and related risk factors. METHODS: A retrospective analysis was performed for the clinical data of 49 children with IPFIs after BA surgery, including clinical features, lung imaging findings, and pathogenic features. The risk factors for IPFIs after BA surgery were also analyzed. RESULTS: The most common pathogens of IPFIs after BA surgery was Candida albicans (17 strains, 45%), followed by Candida tropicalis (7 strains, 18%), Aspergillus (6 strains, 16%), Candida krusei (3 strains, 8%), Candida glabrata (3 strains, 8%), and Candida parapsilosis (2 strains, 5%)...
February 2018: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
Song Sun, Shan Zheng, Xuexin Lu, Gong Chen, Yangyang Ma, Lian Chen, Kuiran Dong
PURPOSE: The objective of this study was to determine the predictive index for prognosis in patients with biliary atresia (BA). METHODS: A total of 71 patients were divided into two groups. Group A included 39 postoperative BA patients who survived for more than 5 years with normal liver function and did not present cirrhosis, and group B included 32 patients who died from liver failure within 1 year after surgery. The clinical data of the two study groups were compared, and liver pathology was evaluated using a scoring system...
February 9, 2018: Pediatric Surgery International
Ravi Kishore, Sundeep M C Kisku, Reju Joseph Thomas, Srinivasa Kishore Jeenipalli
INTRODUCTION: The study describes a refinement in the gallbladder hitch stitch and assesses the value of the laparoscopic cholangiogram in children with suspected biliary atresia. METHODS: Twenty children with neonatal jaundice and no drainage as shown on the HIDA scan underwent a diagnostic laparoscopy through an umbilical 5 mm port. A 3 mm laparoscopic needle holder inserted through a 3.5 mm port to the left of the umbilicus was used to hitch the gallbladder to the abdominal wall...
February 9, 2018: Pediatric Surgery International
Yoshiaki Takahashi, Toshiharu Matsuura, Koichiro Yoshimaru, Yusuke Yanagi, Makoto Hayashida, Tomoaki Taguchi
A graft volume/standard liver volume ratio (GV/SLV) > 35% or graft/recipient weight ratio (GRWR) > 0.8% has been considered as a standard criteria of graft selection. Even if the graft size meets these selection criteria, small-for-size syndrome can still occur depending on the portal venous flow (PVF). The aim of this study was to identify other factors contributing to portal hyperperfusion and the post-transplant course, focusing on the graft volume-to-spleen volume ratio (GV/SV). Thirty-seven BA patients who underwent living donor liver transplantation were reviewed retrospectively...
February 9, 2018: Transplant International: Official Journal of the European Society for Organ Transplantation
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