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Biliary atresia

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https://www.readbyqxmd.com/read/28526690/emerging-concepts-in-biliary-repair-and-fibrosis
#1
Luca Fabris, Carlo Spirli, Massimiliano Cadamuro, Romina Fiorotto, Mario Strazzabosco
Chronic diseases of the biliary tree (cholangiopathies) represent one of the major unmet needs in clinical hepatology and a significant knowledge gap in liver pathophysiology. The common theme in cholangiopathies is that the target of the disease is the biliary tree. After damage to the biliary epithelium, inflammatory changes stimulate a reparative response with proliferation of cholangiocytes and restoration of the biliary architecture, owing to the re-activation of a variety of morphogenetic signals. Chronic damage and inflammation, will ultimately result in pathologic repair, with generation of biliary fibrosis and clinical progression of the disease...
May 19, 2017: American Journal of Physiology. Gastrointestinal and Liver Physiology
https://www.readbyqxmd.com/read/28515290/a-point-mutation-in-the-rhesus-rotavirus-vp4-protein-generated-through-rotavirus-reverse-genetics-system-attenuates-the-murine-model-of-biliary-atresia
#2
Sujit K Mohanty, Bryan Donnelly, Phylicia Dupree, Inna Lobeck, Sarah Mowery, Jaroslaw Meller, Monica McNeal, Greg Tiao
Rotavirus infection is one of the most common causes of diarrheal illness in humans. In neonatal mice, rhesus rotavirus (RRV) can induce biliary atresia (BA), a disease resulting in inflammatory obstruction of the extra-hepatic biliary tract and intrahepatic bile ducts. We have previously shown that the amino acid, arginine (R) within the sequence "SRL" (amino acids 445-447) on the RRV VP4 protein is required for viral binding and entry into biliary epithelial cells. To determine if the single amino acid (R) influences the pathogenicity of the virus, we generated a recombinant virus with a single amino acid mutation at this site through a reverse genetics system...
May 17, 2017: Journal of Virology
https://www.readbyqxmd.com/read/28506343/-current-status-of-research-on-infantile-cholestatic-liver-disease-in-china-a-visualization-analysis
#3
Qiong Liao, Chao-Min Wan, Yu Zhu, Xiao-Yan Yang, Min Shu
OBJECTIVE: To investigate the current status of research on infantile cholestatic liver disease in China and future research trends. METHODS: A co-word analysis was performed in October 2016. Document retrieval and screening were performed in the Chinese databases CNKI and Wanfang Data using "cholestasis" and "infant" as key words. Excel 2010 was used to establish a co-occurrence matrix of high-frequency key words, and Ucinet 6.0 and Netdraw were used to develop a visualized network of these high-frequency key words...
May 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/28501004/adipose-derived-mesenchymal-stem-cells-slow-disease-progression-of-acute-on-chronic-liver-failure
#4
Carlos Gilsanz, Maria-Angeles Aller, Sherezade Fuentes-Julian, Isabel Prieto, Alejandro Blázquez-Martinez, Salvador Argudo, Jorge Fernández-Delgado, Jose Beleña, Jaime Arias, María P De Miguel
A serious complication of chronic hepatic insufficiency is acute-on-chronic liver failure, a recognized syndrome characterized by acute decompensation of cirrhosis and organ/system failure. We investigated the use of adipose-derived mesenchymal stem cells (AD-MSCs) in an experimental model of acute-on-chronic liver failure, developed by microsurgical extrahepatic cholestasis in rats. Rats undergoing microsurgical extrahepatic cholestasis were treated by intraparenchymal liver injection of human or rat AD-MSCs, undifferentiated or previously differentiated in vitro toward the hepatocyte lineage...
May 10, 2017: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/28497759/long-term-challenges-and-perspectives-of-pre-adolescent-liver-disease
#5
REVIEW
Nedim Hadžić, Ulrich Baumann, Pat McKiernan, Valerie McLin, Valerio Nobili
Chronic liver disease is a growing problem that has substantial effects on public health. Many paediatric liver conditions are precursors of adult chronic liver disease, cirrhosis, and hepatocellular carcinoma. Clinical management of Wilson's disease, autoimmune liver disease, and chronic biliary disorders, such as biliary atresia, which remains the most common paediatric chronic liver disease and indication for liver transplantation, is similar in children and adults. In the past 10 or so years, paediatric hepatology has expanded into neighbouring clinical areas, such as metabolic liver diseases and systemic conditions with liver involvement...
June 2017: Lancet. Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28497648/intrahepatic-cholangiojejunostomy-for-complex-biliary-stenosis-after-pediatric-living-donor-liver-transplantation
#6
Fernando A Alvarez, Rodrigo Sanchez Claria, Juan Glinka, Martin de Santibañes, Juan Pekolj, Eduardo de Santibañes, Miguel A Ciardullo
The treatment of biliary stenosis after pediatric LDLT is challenging. We describe an innovative technique of peripheral IHCJ for the treatment of patients with complex biliary stenosis after pediatric LDLT in whom percutaneous treatment failed. During surgery, the percutaneous biliary drainage is removed and a flexible metal stylet is introduced trough the tract. Subsequently, the most superficial aspect of the biliary tree is recognized by palpation of the stylet's round tip in the liver surface. The liver parenchyma is then transected until the bile duct is reached...
May 12, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28497003/achondroplasia-and-biliary-atresia-a-rare-association-and-review-of-literature
#7
Ranjit I Kylat
Achondroplasia (ACH) occurs in most cases as de novo mutations of the gene-encoding fibroblast growth factor receptor 3 (FGFR3). Biliary atresia (BA) is a progressive neonatal inflammatory and fibro-obliterative cholangiopathy affecting the extra- and intrahepatic biliary tree to varying degrees, and it results in obstruction to bile flow and cholestatic jaundice in neonates. BA is thought to be a multifactorial disease, genome association studies have shown abnormalities in susceptibility genes, and levels of fibroblast growth factor 21 (FGF21) and fibroblast growth factor 23 (FGF23) have been noted to be increased...
June 2017: Journal of Pediatric Genetics
https://www.readbyqxmd.com/read/28493866/initial-assessment-of-the-infant-with-neonatal-cholestasis-is-this-biliary-atresia
#8
Benjamin L Shneider, Jeff Moore, Nanda Kerkar, John C Magee, Wen Ye, Saul J Karpen, Binita M Kamath, Jean P Molleston, Jorge A Bezerra, Karen F Murray, Kathleen M Loomes, Peter F Whitington, Philip Rosenthal, Robert H Squires, Stephen L Guthery, Ronen Arnon, Kathleen B Schwarz, Yumirle P Turmelle, Averell H Sherker, Ronald J Sokol
INTRODUCTION: Optimizing outcome in biliary atresia (BA) requires timely diagnosis. Cholestasis is a presenting feature of BA, as well as other diagnoses (Non-BA). Identification of clinical features of neonatal cholestasis that would expedite decisions to pursue subsequent invasive testing to correctly diagnose or exclude BA would enhance outcomes. The analytical goal was to develop a predictive model for BA using data available at initial presentation. METHODS: Infants at presentation with neonatal cholestasis (direct/conjugated bilirubin >2 mg/dl [34...
2017: PloS One
https://www.readbyqxmd.com/read/28478841/somatic-mutation-a-cause-of-biliary-atresia-a-hypothesis
#9
Alexandre Fabre, Céline Roman, Bertrand Roquelaure
Despite many years of research, the causes of biliary atresia still remain elusive. Infection, immune disorder, toxins or maternal microchimerism have been cited as potential triggers of biliary atresia. This is a rare disease with a stable incidence over the years although with sizeable ethnic variations. This stability suggests that environmental factors have in fact only a slight influence. During the search for etiologies, twin studies have often helped disentangle the genetic from the environmental. For this condition, twin studies have mainly demonstrated a lack of concordance between twins (either monozygotic or dizygotic), ruling out Mendelian, infectious or toxic causes...
May 2017: Medical Hypotheses
https://www.readbyqxmd.com/read/28474973/hepatic-hilar-lymph-node-reactivity-at-kasai-portoenterostomy-for-biliary-atresia
#10
K E Bove, R Sheridan, L Fei, R Anders, C T Chung, O W Cummings, M J Finegold, L Finn, S Ranganathan, G Kim, M Lovell, M S Magid, H Melin-Aldana, P Russo, B Shehata, L Wang, F White, Z Chen, C Spino, J C Magee
We hypothesized that if infection is the proximate cause of congenital biliary atresia, an appropriate response to antigen would occur in lymph nodes contiguous with the biliary remnant. We compared the number of follicular germinal centers (GC) in 79 surgically excised hilar lymph nodes (LN) and 27 incidentally discovered cystic duct LNs in 84 subjects at the time of hepatic portoenterostomy (HPE) for biliary atresia (BA) to autopsy controls from the pancreaticobiliary region of non-septic infants >3 months old at death...
January 1, 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28474587/biliary-atresia-current-trends-in-outcome-and-management
#11
Krishna Kumar Govindarajan
No abstract text is available yet for this article.
April 15, 2017: Indian Pediatrics
https://www.readbyqxmd.com/read/28457406/comparison-of-the-results-of-living-donor-liver-transplantation-due-to-acute-liver-failure-and-biliary-atresia-in-a-quaternary-center
#12
L Éboli, A C Tannuri, N Gibelli, T Silva, P Braga, U Tannuri
OBJECTIVE: The objective of this study was to compare the complications, outcomes, and survival prevalence in patients undergoing living donor liver transplantation due to biliary atresia (BA) or acute liver failure (ALF). RESULTS: In the period of June 1998-July 2016, 199 children underwent living transplantation due to BA or ALF. Of these 199, 184 were included in the analysis. The average age, weight, and body mass index of BA patients were lower than those of ALF (P < ...
May 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28450276/pregnancy-and-bile-acid-disorders
#13
Vanessa Pataia, Peter H Dixon, Catherine Williamson
During pregnancy, extensive adaptations in maternal metabolic and immunological physiology occur. Consequently, pre-existing disease may be exacerbated or attenuated and new disease susceptibility may be unmasked. Cholestatic diseases, characterized by a supraphysiological raise in bile acid levels, require careful monitoring during pregnancy. This review describes the latest advances in the knowledge of intrahepatic cholestasis of pregnancy (ICP), the commonest bile acid disorder specific to pregnancy, with a focus on the disease etiology and potential mechanisms of ICP-associated adverse pregnancy outcomes, including fetal demise...
April 27, 2017: American Journal of Physiology. Gastrointestinal and Liver Physiology
https://www.readbyqxmd.com/read/28432216/embryonic-cholecystitis-and-defective-gallbladder-contraction-in-the-sox17-haploinsufficient-model-of-biliary-atresia
#14
Hiroki Higashiyama, Aisa Ozawa, Hiroyuki Sumitomo, Mami Uemura, Ko Fujino, Hitomi Igarashi, Kenya Imaimatsu, Naoki Tsunekawa, Yoshikazu Hirate, Masamichi Kurohmaru, Yukio Saijoh, Masami Kanai-Azuma, Yoshiakira Kanai
The gallbladder excretes cytotoxic bile acids to the duodenum through the cystic duct and common bile duct system. Sox17 haploinsufficiency causes the biliary atresia-like phenotypes and hepatitis in late organogenesis mouse embryos. However, the molecular and cellular mechanisms in the Sox17-haploinsufficient gallbladder and liver in the etiology of biliary atresia remain unclear. In this study, transcriptomic analyses revealed the early onset of cholecystitis in the Sox17(+/-) embryos, together with the appearance of ectopic cystic duct-like epithelia in their gallbladders...
April 21, 2017: Development
https://www.readbyqxmd.com/read/28416017/patient-complexity-and-genotype-phenotype-correlations-in-biliary-atresia-a-cross-sectional-analysis
#15
Guo Cheng, Patrick Ho-Yu Chung, Edwin Kin-Wai Chan, Man-Ting So, Pak-Chung Sham, Stacey S Cherny, Paul Kwong-Hang Tam, Maria-Mercè Garcia-Barceló
BACKGROUND: Biliary Atresia (BA) is rare and genetically complex, and the pathogenesis is elusive. The disease course is variable and can represent heterogeneity, which hinders effective disease management. Deciphering the BA phenotypic variance is a priority in clinics and can be achieved by the integrative analysis of genotype and phenotype. We aim to explore the BA phenotypic features and to delineate the source of its variance. METHODS: The study is a cross-sectional observational study collating with case/control association analysis...
April 17, 2017: BMC Medical Genomics
https://www.readbyqxmd.com/read/28407283/acoustic-radiation-force-impulse-imaging-for-assessing-liver-fibrosis-preoperatively-in-infants-with-biliary-atresia-comparison-with-liver-fibrosis-biopsy-pathology
#16
Fen Gao, Ya-Qing Chen, Jing Fang, Sheng-Li Gu, Luan Li, Xiao-Ying Wang
OBJECTIVES: The purpose of this study was to investigate the diagnostic performance of acoustic radiation force impulse (ARFI) in assessing liver fibrosis preoperatively in infants with biliary atresia (BA). METHODS: A total of 50 consecutive infants with BA and 50 healthy infants who underwent ARFI examination were recruited. Siemens Acuson S2000 in Virtual Touch Quantification mode (Siemens Medical Solutions, Mountain View, CA) was used to measure shear wave speeds (SWSs)...
April 13, 2017: Journal of Ultrasound in Medicine: Official Journal of the American Institute of Ultrasound in Medicine
https://www.readbyqxmd.com/read/28403505/the-role-of-endoscopic-retrograde-cholangiopancreatography-in-the-diagnosis-of-biliary-atresia-14-years-experience
#17
Ahmed A Negm, Claus Petersen, Andrea Markowski, Birgit Luettig, Kristina I Ringe, Tim O Lankisch, Michael P Manns, Benno Ure, Andrea S Schneider
No abstract text is available yet for this article.
April 12, 2017: European Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28402981/prenatal-nonvisualization-of-the-gallbladder-a-diagnostic-and-prognostic-dilemma
#18
Aina Ruiz, Ana Robles, Francisca Salva, Ana Filgueira, Cristina Díaz, Miquel Juan, Albert Tubau
INTRODUCTION: Nonvisualization of the fetal gallbladder has been associated with benign conditions such as isolated gallbladder agenesis or severe diseases such as biliary atresia (BA). Recently, gamma-glutamyl transpeptidase (GGTP) fetal blood levels were reported as useful after 22 weeks. OBJECTIVE: To determine the contribution of fetal blood GGTP levels after 22 weeks, based on 2 cases. Case 1: 20+4-week secundipara, with subcutaneous edema and pleural effusion...
April 13, 2017: Fetal Diagnosis and Therapy
https://www.readbyqxmd.com/read/28397681/-isolated-gallbladder-agenesis-without-biliary-atresia-in-a-16-year-old-boy
#19
Bojan Kovacevic, Laura Sophie Mössinger, Thomas Boel
We present a case of a 16-year-old male patient with recurrent abdominal pain in the upper right quadrant, signed up for elective laparoscopic cholecystectomy due to a single gallstone seen on preoperative abdominal ultrasound. Because of dilatation of the common bile duct subsequent magnetic resonance cholangiopancreatography was performed, and surprisingly, the gallbladder was found missing. The operation was therefore cancelled. Even though gallbladder agenesis is a rare condition, it still represents a diagnostic challenge as most cases are found peroperatively, and the patients undergo unnecessary surgery...
February 6, 2017: Ugeskrift for Laeger
https://www.readbyqxmd.com/read/28397065/referral-patterns-and-factors-influencing-age-at-admission-of-infants-with-cholestasis-in-india
#20
Gopinathan Mathiyazhagan, Barath Jagadisan
OBJECTIVES: To define the recognition, age at admission, referral time and referral pattern of neonatal cholestasis in India. METHODS: This prospective, observational study was conducted from February 2015 through March 2016 in the Pediatric gastroenterology unit of JIPMER, Pondicherry in infants with cholestasis < 6 mo of age. RESULTS: Among 64 infants, median age of admission was 52 d (IQR 28-63 d). Fifty of sixty four infants (78.1%) came with parent-reported cholestasis-related symptoms of either jaundice alone (57...
April 11, 2017: Indian Journal of Pediatrics
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