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Biliary atresia

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https://www.readbyqxmd.com/read/28432216/embryonic-cholecystitis-and-defective-gallbladder-contraction-in-the-sox17-haploinsufficient-model-of-biliary-atresia
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
https://www.readbyqxmd.com/read/28393506/-postcholecystectomy-syndrome-in-children-fact-or-fiction
#8
N Gallego Mellado, N Albertos Mira-Marcelí, P Deltell Colomer, M E Kuan Argüello, J Mira Navarro, J Gonzálvez Piñera
INTRODUCTION: The postcholecystectomy syndrome (SPC) is broadly defined and published in adults, whereas in the pediatric population are hardly any articles about it. Up to a third of adults have dyspeptic symptoms without organic cause the first year after cholecystectomy. Our goal is to determine the incidence of SPC in our population. METHODS: An observational study was performed, collecting data from patients who had been done laparoscopic cholecystectomy in our hospital since 2005...
July 10, 2016: Cirugía Pediátrica: Organo Oficial de la Sociedad Española de Cirugía Pediátrica
https://www.readbyqxmd.com/read/28374309/early-and-peri-operative-prognostic-indicators-in-infants-undergoing-hepatic-portoenterostomy-for-biliary-atresia-a-review
#9
REVIEW
Robert N Lopez, Chee Y Ooi, Usha Krishnan
PURPOSE OF REVIEW: Biliary atresia is the most common indication for liver transplantation among children. In recent years, prospective, multi-centre collaboration has been underway with the aim of providing high-quality data on the natural history of the condition, prior to and following hepatic portoenterostomy. RECENT FINDINGS: There is increasing evidence that specific histological findings, and age, at the time of portoenterostomy have relevance as prognostic indicators...
April 2017: Current Gastroenterology Reports
https://www.readbyqxmd.com/read/28364348/analysis-of-soluble-factors-in-conditioned-media-derived-from-primary-cultures-of-cirrhotic-liver-of-biliary-atresia
#10
Taisuke Yamazaki, Mariko Wakai, Shin Enosawa, Takayoshi Tokiwa
Biliary atresia (BA) is a rare and serious liver disease in newborn infants. Previously, we reported that non-parenchymal cell (NPC) fractions from cirrhotic liver of BA may contain hepatic stem/progenitor cells in primary culture of NPC fractions. In this study, NPC fractions were subjected to primary or passage culture and found that clusters of hepatocyte-like cells appear even without adding hepatocyte growth factor (HGF) to the culture medium, but not in their passage culture used as a control. Based on these findings, conditioned media (CMs) were collected and soluble factors in the CMs were analyzed in order to elucidate the mechanism of the appearance of hepatocyte-like cells or their clusters...
March 31, 2017: In Vitro Cellular & Developmental Biology. Animal
https://www.readbyqxmd.com/read/28358366/forkhead-box-a3-attenuated-the-progression-of-fibrosis-in-a-rat-model-of-biliary-atresia
#11
Rui Dong, Yifan Yang, Zhen Shen, Chao Zheng, Zhu Jin, Yanlei Huang, Zhien Zhang, Shan Zheng, Gong Chen
Biliary atresia is a rare, devastating disease of infants where a fibroinflammatory process destroys the bile ducts, leading to fibrosis and biliary cirrhosis, and death if untreated. The cause and pathogenesis remain largely unknown. We tried to investigate factors involved in biliary atresia, especially forkhead box A3 (Foxa3), which might exert a role in the treatment of liver disease. We used RNA sequencing to sequence the whole transcriptomes of livers from six biliary atresia and six choledochal cysts patients...
March 30, 2017: Cell Death & Disease
https://www.readbyqxmd.com/read/28355206/hepatic-mdr3-expression-impacts-lipid-homeostasis-and-susceptibility-to-inflammatory-bile-duct-obstruction-in-neonates
#12
Alexandra N Carey, Wujuan Zhang, Kenneth D R Setchell, Julia E Simmons, Tiffany Shi, Celine S Lages, Mary Mullen, Kaitlin Carroll, Rebekah Karns, Kazuhiko Bessho, Rachel Sheridan, Xueheng Zhao, Susanne N Weber, Alexander G Miethke
BACKGROUND: Heterozygous mutations in the gene ABCB4, encoding the phospholipid floppase MDR3 (Mdr2 in mice), are associated with various chronic liver diseases. Here, we hypothesize that reduced ABCB4 expression predisposes to extrahepatic biliary atresia (EHBA). METHODS: Livers from neonatal wildtype (wt) and heterozygous Mdr2-deficient mice were subjected to mass-spectrometry based lipidomics and RNAseq studies. Following postnatal infection with rhesus rotavirus (RRV), liver immune responses and EHBA phenotype were assessed...
March 29, 2017: Pediatric Research
https://www.readbyqxmd.com/read/28355202/microrna-155-modulates-bile-duct-inflammation-via-targeting-suppressor-of-cytokine-signaling-1-in-biliary-atresia
#13
Rui Zhao, Rui Dong, Yifan Yang, Yuqing Wang, Jin Ma, Jiang Wang, Hao Li, Shan Zheng
BACKGROUND: Biliary atresia (BA) is an etiologically perplexing disease, manifested by neonatal cholestasis, repeated cholangitis, and progressive biliary fibrosis. MiR-155 has been implicated to modulate the immune response, which contributes to biliary injury. However, its potential role in the pathogenesis of BA has not been addressed so far. METHODS: The microRNA changes from BA patients and controls were identified via microarray. The immunomodulatory function of miR-155 was investigated via cell transfection and reporter assay system...
March 29, 2017: Pediatric Research
https://www.readbyqxmd.com/read/28350200/does-the-level-of-transection-of-the-biliary-remnant-affect-outcome-after-laparoscopic-kasai-portoenterostomy-for-biliary-atresia
#14
Hiroki Nakamura, Hiroyuki Koga, Go Miyano, Manabu Okawada, Takashi Doi, Atsuyuki Yamataka
BACKGROUND: We assessed postoperative outcome in relation to the level of transection of the biliary remnant at the time of laparoscopic Kasai portoenterostomy (LKP) in biliary atresia (BA) patients. METHODS: The subjects for this study were 12 consecutive nonsyndromic type III BA patients who had LKP at our institute between 2009 and 2014. All LKPs were video recorded. Four board-certified pediatric surgeons assessed the level of transection of the biliary remnant and suturing during the anastomosis in each video blindly...
March 28, 2017: Journal of Laparoendoscopic & Advanced Surgical Techniques. Part A
https://www.readbyqxmd.com/read/28343205/-biliary-atresia-signs-and-symptoms-diagnosis-clinical-management
#15
Ewa Orłowska, Piotr Czubkowski, Piotr Socha
Biliary atresia is a chronic cholangiopathy leading to progressive fibrosis of both intra- and extrahepatic bile ducts. The cause of the condition is unknown. Fundamental management of biliary atresia is surgical intervention and the outcomes of the treatment depend on the child's age with best results when performed within the first 2 months of life. Thus, the main role of pediatric healthcare is an urgent differential diagnosis and prompt qualification for the surgery, optimal postoperative management and early qualification for the liver transplantation in patients with persistent cholestasis...
2017: Wiadomości Lekarskie: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/28335859/making-it-new-again-insight-into-liver-development-regeneration-and-disease-from-zebrafish-research
#16
Shuang Wang, Sophie R Miller, Elke A Ober, Kirsten C Sadler
The adult liver of most vertebrates is predominantly comprised of hepatocytes. However, these cells must work in concert with biliary, stellate, vascular, and immune cells to accomplish the vast array of hepatic functions required for physiological homeostasis. Our understanding of liver development was accelerated as zebrafish emerged as an ideal vertebrate system to study embryogenesis. Through work in zebrafish and other models, it is now clear that the cells in the liver develop in a coordinated fashion during embryogenesis through a complex yet incompletely understood set of molecular guidelines...
2017: Current Topics in Developmental Biology
https://www.readbyqxmd.com/read/28332100/expression-of-programmed-death-1-and-its-ligands-in-the-liver-of-biliary-atresia
#17
Pan-Liang Wang, Jun Wang, Ying Zhou, Xiao-Song Chen, Ke-Jun Zhou, Jie Wen, Jian-Jun Zhang, Wei Cai
BACKGROUND: An aberrant immune response is the predominant pathogenetic factor in biliary atresia (BA). Programmed death-1 (PD-1) and its two ligands, programmed death ligand-1 and programmed death ligand-2 (PD-L1 and PD-L2, respectively) play an important inhibitory role in immune reactions. We aimed to illustrate the expression of these molecules in BA. METHODS: Liver specimens were obtained from infants with BA during the Kasai procedure (early BA) and liver transplantation (late BA)...
March 22, 2017: World Journal of Pediatrics: WJP
https://www.readbyqxmd.com/read/28325677/il-6-tnf-%C3%AE-il-10-and-nutritional-status-in-pediatric-patients-with-biliary-atresia
#18
Maria Ines de Albuquerque Wilasco, Carolina Uribe-Cruz, Daniele Santetti, Gabriel Rodrigo Fries, Cristina Toscani Leal Dornelles, Themis Reverbel da Silveira
OBJECTIVES: The objective of the present study is to evaluate whether IL-6, TNF-α, IL-10 are associated with nutritional status in patients with cirrhosis secondary to biliary atresia and compare to healthy controls. METHODS: The parameters used for nutritional assessment were the standard deviation scores of height-for-age and of triceps skinfold thickness-for-age. The severity of cirrhosis was evaluated using the Child-Pugh score and PELD/MELD. Serum cytokines were measured using Cytometric Bead Array flow cytometry...
March 19, 2017: Jornal de Pediatria
https://www.readbyqxmd.com/read/28321310/neonatal-cholestasis-mimicking-biliary-atresia-could-it-be-urinary-tract-infection
#19
Noella Maria Delia Pereira, Ira Shah
Cholestasis can occur in newborns due to infections. However, the manifestations of the underlying infections usually dominate the presentation. We present a 2-month-old infant who presented with jaundice and no fever or signs of systemic illness. Liver biopsy was suggestive of cholangitis. He was subsequently detected to have urinary tract infection with Klebsiella pneumoniae. The child was treated with appropriate antibiotics for 2 weeks following which the cholestasis resolved. Thus, neonatal cholestasis due to infections can also occur in the post-neonatal period without clinical manifestations of an underlying infection...
2017: SAGE open medical case reports
https://www.readbyqxmd.com/read/28319568/predictive-value-of-intraoperative-troponin-i-elevation-in-pediatric-living-donor-liver-transplant-recipients-with-biliary-atresia
#20
Mingwei Sheng, Yuanbang Lin, Yiqi Weng, Rubin Xu, Ying Sun, Wenli Yu, Hongyin Du
BACKGROUND: Pediatric living donor liver transplantation is associated with slight alteration in cardiac enzymes without ongoing acute cardiac injury, but available information about the significance of these changes is limited. The aims of this study were to analyze the link between the anomalies of intraoperative serum cardiac troponin I (cTnI) and acute lung injury during the first week after liver transplantation. METHODS: In this retrospective study, 123 children suffered from biliary atresia were enrolled...
March 18, 2017: Transplantation
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