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Biliary atresia

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https://www.readbyqxmd.com/read/29773306/factors-associated-with-timing-and-adverse-outcomes-in-patients-with-biliary-atresia-undergoing-kasai-hepatoportoenterostomy
#1
Michael Ross Townsend, Adeeb Jaber, Hanina Abi Nader, Shaker M Eid, Kathleen Schwarz
OBJECTIVE: To assess factors associated with timing of hepatoportoenterostomy (HPE) and adverse perioperative outcomes in patients with biliary atresia in the US. STUDY DESIGN: We examined hospitalizations in infants aged <1 year using the National Inpatient Sample database for 2000-2011. We identified cases using the International Classification of Diseases, Ninth Revision, Clinical Modification codes for biliary atresia and HPE. Multivariable logistic regression models were used to examine association between select factors and age at HPE, as well as adverse perioperative outcomes...
May 14, 2018: Journal of Pediatrics
https://www.readbyqxmd.com/read/29762915/women-s-periconceptional-diet-and-risk-of-biliary-atresia-in-offspring
#2
Suzan L Carmichael, Chen Ma, Alissa R Van Zutphen, Cynthia A Moore, Gary M Shaw
INTRODUCTION: We examined the association of biliary atresia with maternal dietary intake, using National Birth Defects Prevention Study (NBDPS) data from 152 cases and 11,112 nonmalformed controls born 1997-2011. METHODS: NBDPS is a multisite, population-based case-control study. Exposure data were from maternal telephone interviews, which included a food frequency questionnaire. Odds ratios (OR) and 95% confidence intervals (CI) were generated from logistic regression models that included nutritional factors as continuous variables and were adjusted for energy intake only or energy intake plus covariates (maternal race-ethnicity, education, age, prepregnancy body mass index, vitamin/mineral supplement intake, conception during summer)...
May 15, 2018: Birth Defects Research
https://www.readbyqxmd.com/read/29761937/-mirror-mirror-on-the-wall-%C3%A2-pediatric-liver-transplantation-in-the-case-of-situs-inversus-totalis-with-a-disrupted-inferior-vena-cava
#3
Colston A Edgerton, Megan Gross, Nagraj Kasi, Winston Hewitt, Sara Edmondson, Vinayak S Rohan, Satish N Nadig
We present the unique case of a 15-month-old male born with biliary atresia and situs inversus totalis and disrupted inferior vena cava who underwent a successful liver transplantation. The patient had previously undergone a failed Kasai procedure and presented with persistent hyperbilirubinemia. The patient was transplanted with a left lateral segment donor having standard arterial anatomy. Technical considerations included identifying completely replaced arterial anatomy in the recipient from the superior mesenteric artery and creating a branch patch between the gastroduodenal artery and HA, anastomosing the donor left hepatic vein to confluences of the donor left, middle, and right hepatic veins, using a "lazy-S" configuration of portal vein anastomosis, and suspending the allograft to the abdominal wall...
May 15, 2018: Pediatric Transplantation
https://www.readbyqxmd.com/read/29761473/glucocorticosteroids-for-infants-with-biliary-atresia-following-kasai-portoenterostomy
#4
REVIEW
Athanasios Tyraskis, Christopher Parsons, Mark Davenport
BACKGROUND: Biliary atresia is a life-threatening disease characterised by progressive destruction of both intra- and extra-hepatic biliary ducts. The mainstay of treatment is Kasai portoenterostomy, as soon as the disease has been confirmed. Glucocorticosteroids are steroid hormones which act on the glucocorticoid receptor and have a range of metabolic and immunomodulatory effects. Glucocorticosteroids are used to improve the postoperative outcomes in infants who have undergone Kasai portoenterostomy...
May 14, 2018: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/29761167/phenotypic-spectrum-and-diagnostic-pitfalls-of-abcb4-deficiency-depending-on-age-of-onset
#5
Stephanie Barbara Schatz, Christoph Jüngst, Verena Keitel-Anselmo, Ralf Kubitz, Christina Becker, Patrick Gerner, Eva-Doreen Pfister, Imeke Goldschmidt, Norman Junge, Daniel Wenning, Stephan Gehring, Stefan Arens, Dirk Bretschneider, Dirk Grothues, Guido Engelmann, Frank Lammert, Ulrich Baumann
Genetic variants in the adenosine triphosphate-binding cassette subfamily B member 4 ( ABCB4 ) gene, which encodes hepatocanalicular phosphatidylcholine floppase, can lead to different phenotypes, such as progressive familial intrahepatic cholestasis (PFIC) type 3, low phospholipid-associated cholelithiasis, and intrahepatic cholestasis of pregnancy. The aim of this multicenter project was to collect information on onset and progression of this entity in different age groups and to assess the relevance of this disease for the differential diagnosis of chronic liver disease...
May 2018: Hepatology Communications
https://www.readbyqxmd.com/read/29751790/obliterative-cholangiopathy-in-acquired-cystic-biliary-atresia-type-iii-after-cyst-perforation-a-case-report
#6
Tsugumichi Koshinaga, Kensuke Ohashi, Kakou Ono, Hide Kaneda, Takeshi Furuya
BACKGROUND: In biliary atresia, the disease process of obliterative cholangiopathy may begin in the perinatal period; however, no chronological evidence exists on how the cholangiopathy progresses to biliary obliteration. This is the first acquired case with the final diagnosis of type III cystic biliary atresia with an extrahepatic biliary cyst which showed the progression of obliterative cholangiopathy in chronological order after birth. CASE PRESENTATION: An 81-day-old girl presented with acute abdominal distress due to bilious peritonitis caused by biliary cyst perforation, for which she underwent emergency biliary drainage...
May 11, 2018: BMC Pediatrics
https://www.readbyqxmd.com/read/29748604/microrna-29b-142-5p-contribute-to-the-pathogenesis-of-biliary-atresia-by-regulating-the-ifn-%C3%AE-gene
#7
Yifan Yang, Zhu Jin, Rui Dong, Chao Zheng, Yanlei Huang, Yijie Zheng, Zhen Shen, Gong Chen, Xiaoying Luo, Shan Zheng
Biliary atresia is one of the most common liver disease in infancy. The cause and pathogenesis remain largely unknown. This study aimed to investigate the potential regulatory effect of miR-29b/142-5p on IFN-γ gene methylation. miRNAs microarray was performed on four pairs of liver and blood specimens from biliary atresia and choledochal cysts. We found the overexpression of miR-142-5p and mRNA level of DNA methyltransferase (DNMT) 1, and miR-29b and DNMT3a/DNMT3b were significantly negatively correlated in biliary atresia livers...
May 10, 2018: Cell Death & Disease
https://www.readbyqxmd.com/read/29745028/intellectual-and-academic-outcomes-after-pediatric-liver-transplantation-relationship-with-transplant-related-factors
#8
Soheil Afshar, Melanie Porter, Belinda Barton, Michael Stormon
As survival rates for pediatric liver transplantation continue to increase, research attention is turning towards long-term functional consequences, with particular interest in whether medical and transplant-related factors are implicated in neurocognitive outcomes. The relative importance of different factors is unclear, due to a lack of methodological uniformity, inclusion of differing primary diagnoses, variable transplant policies, and organ availability in different jurisdictions. This cross-sectional, single-site study sought to address various methodological limitations in the literature and the paucity of studies conducted outside of North America and Western Europe by examining the intellectual and academic outcomes of Australian pediatric liver transplant recipients (N = 40)...
May 9, 2018: American Journal of Transplantation
https://www.readbyqxmd.com/read/29735782/an-overview-of-liver-transplant-pathology-data-from-a-tertiary-referral-centre-in-western-india
#9
Fiona Fonseca, Bijal Kulkarni, Meenal Hastak, Vinay Kumaran, Vibha Varma, Sorabh Kapoor
INTRODUCTION AND AIM: 1. Study of liver explants - Etiologic types of end-stage chronic liver disease (ESCLD) and acute liver failure (ALF) in adults and children. 2. Assessment of donor steatosis and incidental granulomas. 3. Post-transplant liver biopsies. MATERIAL AND METHODS: Specimens of 180 explant hepatectomies, 173 donor wedge and 30 core liver biopsies, and 58 post transplant liver biopsies received in our department from April 2013 to March 2017. RESULTS: 1...
April 9, 2018: Annals of Hepatology
https://www.readbyqxmd.com/read/29733490/the-efficacy-of-serum-brain-natriuretic-peptide-for-the-early-detection-of-portopulmonary-hypertension-in-biliary-atresia-patients-before-liver-transplantation
#10
Koichiro Yoshimaru, Toshiharu Matsuura, Yoshiaki Takahashi, Yusuke Yanagi, Hazumu Nagata, Shouichi Ohga, Tomoaki Taguchi
Severe portopulmonary hypertension (POPH) is a contraindication for liver transplantation (LT) because of the high risk of postoperative heart failure. The early detection of POPH is important for patients with biliary atresia (BA). Brain natriuretic peptide (BNP) is known to be correlated with liver fibrosis in patients with liver cirrhosis. The aim of this study was to elucidate the efficacy of BNP measurement for the follow-up of patients with BA. Thirty-two patients with BA were identified from September 2011 to December 2016...
May 7, 2018: Pediatric Transplantation
https://www.readbyqxmd.com/read/29730676/interconversion-of-elasticity-measurements-between-two-dimensional-shear-wave-elastography-and-transient-elastography
#11
Yeun-Yoon Kim, Myung-Joon Kim, Hyun Joo Shin, Haesung Yoon, Ha Yan Kim, Mi-Jung Lee
AIMS: To enable comparison and interconversion of elasticity measurements between two-dimensional (2D) shear wave elastography (SWE) and transient elastography (TE). MATERIALS AND METHODS: Elasticities of three phantoms were measured by 2D SWE (supersonic shear imaging) using four probes and TE using two probes. We performed regression analyses to evaluate correlation between the measurements and phantom elasticities, and make converting equations. In pediatric biliary atresia patients who had stiffness measurements by both 2D SWE and TE within 1-year interval, TE measurements were retrospectively converted into correlating 2D SWE values...
May 2, 2018: Medical Ultrasonography
https://www.readbyqxmd.com/read/29729773/surgical-outcomes-in-alagille-syndrome-and-pfic-a-single-institution-s-20-year-experience
#12
Celia D Flores, Yangyang R Yu, Tamir A Miloh, John Goss, Mary L Brandt
BACKGROUND: Alagille Syndrome (AGS) and Progressive Familial Intrahepatic Cholestasis (PFIC) are rare pediatric biliary disorders that lead to progressive liver disease. This study reviews our experience with the surgical management of these disorders over the last 20years. METHODS: We retrospectively reviewed the records of children diagnosed with AGS or PFIC from January 1996 to December 2016. Data collected included demographics, surgical intervention (liver transplant or biliary diversion), and complications...
May 2018: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29728912/effect-of-cryopreservation-on-the-appearance-and-liver-function-of-hepatocyte-like-cells-in-cultures-of-cirrhotic-liver-of-biliary-atresia
#13
Taisuke Yamazaki, Shin Enosawa, Takayoshi Tokiwa
Previously, we reported that non-parenchymal cell (NPC) fractions from cirrhotic liver of biliary atresia (BA) may contain stem/progenitor cells, and clusters of hepatocyte-like cells appear via hepatocyte growth factor/c-Met signaling in primary cultures of NPCs. BA is a rare and serious liver disease, and procurement of BA cells is difficult. Therefore, cryopreservation of BA liver cells is an unavoidable challenge. In this study, we examined the appearance and liver function of hepatocyte-like cells in cultures of BA liver-derived NPC fractions after cryopreservation for 1 or 6 mo using a chemically defined cryopreservation solution, STEM-CELLBANKER...
May 4, 2018: In Vitro Cellular & Developmental Biology. Animal
https://www.readbyqxmd.com/read/29726831/stool-color-card-as-a-screening-tool-for-biliary-atresia
#14
Siba Prosad Paul, Jagrati Chopra
No abstract text is available yet for this article.
April 15, 2018: Indian Pediatrics
https://www.readbyqxmd.com/read/29718863/endoscopic-retrograde-cholangiopancreatography-in-adult-patients-with-biliary-atresia-process-compliant-case-series
#15
Jong Jin Hyun, Shayan S Irani, Richard A Kozarek
INTRODUCTION: Biliary atresia is a progressive inflammatory disease of the bile duct that eventually results in biliary cirrhosis. It is a rare neonatal disease that mandates treatment within the first 2 years of life in order for the infant to survive. Patients usually undergo palliative Kasai portoenterostomy. Even when Kasai portoenterostomy has been performed in a timely manner, progression is still inevitable. In fact, the majority of patients require curative liver transplantation at a later stage before reaching adulthood...
May 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29707407/variants-associated-with-infantile-cholestatic-syndromes-detected-in-extrahepatic-biliary-atresia-by-whole-exome-studies-a-20-case-series-from-thailand
#16
Surasak Sangkhathat, Wison Laochareonsuk, Wanwisa Maneechay, Kanita Kayasut, Piyawan Chiengkriwate
Biliary atresia (BA) is the most severe form of obstructive cholangiopathy occurring in infants. Definitive diagnosis of BA usually relies on operative findings together with supporting pathological patterns found in the extrahepatic bile duct. In infancy, overlapping clinical patterns of cholestasis can be found in other diseases including biliary hypoplasia and progressive familial intrahepatic cholestasis. In addition, BA has been reported as a phenotype in some rare genetic syndromes. Unlike BA, other cholangiopathic phenotypes have their own established genetic markers...
June 2018: Journal of Pediatric Genetics
https://www.readbyqxmd.com/read/29705277/endotoxin-metabolism-reflects-hepatic-functional-reserve-in-end-stage-liver-disease
#17
N Okada, Y Sanada, T Urahashi, Y Ihara, N Yamada, Y Hirata, T Katano, S Otomo, K Ushijima, K Mizuta
BACKGROUND: The hepatic clearance of endotoxin (Et) may reflect hepatic functional reserve and ischemic injury to hepatocytes. Therefore, we examined the relationships between Et activity (EA) and the metrics Pediatric End-Stage Liver Disease (PELD)/Model of End-Stage Liver Disease (MELD) score and alanine transaminase (ALT) levels in the postoperative period. METHODS: We performed 8 living-donor liver transplantations (LDLTs) for biliary atresia at our center from April 2012 to December 2012...
April 25, 2018: Transplantation Proceedings
https://www.readbyqxmd.com/read/29704300/can-we-minimize-waiting-list-mortality-in-young-children-with-biliary-atresia
#18
EDITORIAL
A Schlegel, P Muiesan
No abstract text is available yet for this article.
April 27, 2018: Liver Transplantation
https://www.readbyqxmd.com/read/29698570/interleukin-2-promotes-hepatic-regulatory-t-cell-responses-and-protects-from-biliary-fibrosis-in-murine-sclerosing-cholangitis
#19
Amy E Taylor, Alexandra N Carey, Ramesh Kudira, Celine S Lages, Tiffany Shi, Simon Lam, Rebekah Karns, Julia Simmons, Kumar Shanmukhappa, Maha Almanan, Claire A Chougnet, Alexander G Miethke
In the Mdr2 -/- mouse model low phospholipid bile instigates biliary epithelial injury, sterile inflammation, and fibrosis, thereby recapitulating disease mechanisms implicated in biliary atresia (BA) and primary sclerosing cholangitis. We hypothesize that T-lymphocytes contribute to the biliary injury and fibrosis in murine sclerosing cholangitis (SC) and that they are susceptible to suppression by regulatory T cells (Tregs). In juvenile Mdr2 -/- mice, intrahepatic CD8 lymphocytes were expanded and contraction of intrahepatic Tregs coincided with rising serum alanine transferase (ALT) and alkaline phosphatase (ALP) levels between days 14-30 of life...
April 26, 2018: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/29685956/the-intragenic-epistatic-association-of-add3-with-biliary-atresia-in-southern-han-chinese-population
#20
Zhe Wang, Xiaoli Xie, Jinglu Zhao, Ming Fu, Yonglan Li, Wei Zhong, Huimin Xia, Yan Zhang, Rui-Zhong Zhang
Biliary atresia (BA) is a multifactorial pathogenic disease with possible genetic components. As a member of membrane skeletal proteins in the liver and bile ducts, a haplotype composed by five SNPs on ADD3 has been identified as associated with BA. However, limited study was designed to further elaborate the mutual relationship among those replicated SNPs to disease. We selected three susceptibility SNPs in ADD3 and conducted a replication study using 510 BA cases and 1473 controls to evaluate the individual function of the SNPs and further stratified the potential roles with disease and its subclinical features...
April 23, 2018: Bioscience Reports
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