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Biliary atresia

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https://www.readbyqxmd.com/read/28927974/infiltration-of-polarized-macrophages-associated-with-liver-fibrosis-in-infants-with-biliary-atresia
#1
Yifan Yang, Rui Dong, Chao Zheng, Shan Zheng, Gong Chen
BACKGROUND/PURPOSE: Macrophages exert critical functions in liver homeostasis and have been proposed as potential targets in combatting fibrosis. We aimed to evaluate polarized functional status of liver infiltrated macrophages in infants with biliary atresia (BA). METHODS: Immunohistochemical staining for CD68, CD163, and IRF5 was performed in 40 BA infants. Liver biopsies were scored for fibrosis and tested for association with clinical biochemical characteristics...
September 2, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28923636/effect-of-preoperative-growth-status-on-clinical-outcomes-after-living-donor-liver-transplantation-in-infants
#2
Y Lu, Q Xia, Y Yang, P Wan, J Hou, Y Wang, B Qiu, M Feng, K He, M Huang, X Ren, L Gao, J Zhang
OBJECTIVE: The aim of this study was to evaluate the impact of preoperative growth status on clinical outcomes of infantile living-donor liver transplantation (LDLT). METHODS: From January 2009 to December 2014, 131 LDLT recipients ≤1 year of age met the eligibility criteria of this study, of whom 31 patients with weight z-scores less than -2 constituted the abnormal growth group. Patients in the abnormal growth group were randomly matched (1:1) to patients with normal growth status by means of a multivariate case-matched method, and thus 31 patients in the normal growth group and 31 patients in the abnormal growth group were finally enrolled into the study...
October 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28902846/downregulation-of-microrna-145-may-contribute-to-liver-fibrosis-in-biliary-atresia-by-targeting-add3
#3
Yongqin Ye, Zhihan Li, Qi Feng, Zimin Chen, Zhouguang Wu, Jianyao Wang, Xiaoshuo Ye, Dahao Zhang, Lei Liu, Wei Gao, Lihui Zhang, Bin Wang
BACKGROUND AND OBJECTIVES: Biliary atresia (BA) is a pediatric liver disease characterized by fibro-obliteration and obstruction of the extrahepatic biliary system, that invariably leads to cirrhosis and even death, if left untreated for extended time. However, its pathology and etiology still remained unknown. In this study, we tested the expression of adducin 3 (ADD3), the gene identified as a susceptibility gene in BA by GWAS, and uncovered its upstream regulatory microRNA in the pathogenesis of BA...
2017: PloS One
https://www.readbyqxmd.com/read/28901937/advances-in-paediatric-gastroenterology
#4
REVIEW
Paul K H Tam, Patrick H Y Chung, Shawn D St Peter, Christopher P Gayer, Henri R Ford, Greta C H Tam, Kenneth K Y Wong, Mikko P Pakarinen, Mark Davenport
Recent developments in paediatric gastrointestinal surgery have focused on minimally invasive surgery, the accumulation of high-quality clinical evidence, and scientific research. The benefits of minimally invasive surgery for common disorders like appendicitis and hypertrophic pyloric stenosis are all supported by good clinical evidence. Although minimally invasive surgery has been extended to neonatal surgery, it is difficult to establish its role for neonatal disorders such as oesophageal atresia and biliary atresia through clinical trials because of the rarity of these disorders...
September 9, 2017: Lancet
https://www.readbyqxmd.com/read/28901029/successful-venoarterial-extracorporeal-membrane-oxygenation-for-postoperative-septic-shock-in-a-child-with-liver-transplantation-a-case-report
#5
Michiko Abe, Kentaro Ide, Nao Nishimura, Satoshi Nakagawa, Akinari Fukuda, Seisuke Sakamoto, Mureo Kasahara
Refractory septic shock after LT is a life-threatening complication. VA ECMO is used to treat refractory cardiorespiratory failure. We present herein the case of a 5-year-old girl with post-Kasai biliary atresia, who underwent a living donor LT and suffered refractory septic shock. VA ECMO was indicated due to progressive cardiac deterioration. After full recovery of her EF, she has been steadily improving and has shown good liver function and no neurological sequelae. This is the first report of successful VA ECMO in a post- LT patient with refractory septic shock...
September 12, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28891487/outcome-of-biliary-atresia-after-kasai-s-portoenterostomy-few-concerns-author-s-reply
#6
Rajeev Gurunath Redkar
No abstract text is available yet for this article.
August 15, 2017: Indian Pediatrics
https://www.readbyqxmd.com/read/28891486/outcome-of-biliary-atresia-after-kasai-s-portoenterostomy-few-concerns
#7
Vikrant Sood, Seema Alam
No abstract text is available yet for this article.
August 15, 2017: Indian Pediatrics
https://www.readbyqxmd.com/read/28889961/analysis-of-the-reversibility-of-biliary-cirrhosis-in-young-rats-submitted-to-biliary-obstruction
#8
Maria Julia de Aro Braz, Leonardo Ervolino Corbi, Ana Cristina Aoun Tannuri, Maria Cecília Mendonça Coelho, Josiane Oliveira Gonçalves, Suellen Serafini, Uenis Tannuri
BACKGROUND/PURPOSE: Biliary atresia and other liver biliary obstructions are relevant conditions in pediatric surgery due to their progression to biliary cirrhosis and indication for liver transplantation. It is known that the period during which biliary obstruction persists determines the development of cirrhosis and its reversibility after a biliary drainage procedure. However, no time or histological markers of biliary cirrhosis reversibility have been established. MATERIALS AND METHODS: One hundred and twenty-nine young Wistar rats underwent surgery for ligation of the common bile duct and were maintained until 8weeks...
August 24, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28889651/living-donor-liver-transplantation-for-biliary-atresia-an-analysis-of-2-085-cases-in-the-registry-of-the-japanese-liver-transplantation-society
#9
Mureo Kasahara, Koji Umeshita, Seisuke Sakamoto, Akinari Fukuda, Hiroyuki Furukawa, Shotaro Sakisaka, Eiji Kobayashi, Eiji Tanaka, Yukihiro Inomata, Seiji Kawasaki, Mitsuo Shimada, Norihiro Kokudo, Hiroto Egawa, Hideki Ohdan, Shinji Uemoto
Biliary atresia (BA) is the most common indication for liver transplantation (LT) in pediatric population. This study analyzed the comprehensive factors that might influence the outcomes of patients with BA who undergo living donor LT by evaluating the largest cohort with the longest follow-up in the world. Between November 1989 and December 2015, 2,085 BA patients underwent LDLT in Japan. There were 763 male and 1,322 female recipients with a mean age of 5.9 years and body weight of 18.6 kg. The 1-, 5-, 10-, 15-and 20-year graft survival rates for the BA patients undergoing LDLT were 90...
September 10, 2017: American Journal of Transplantation
https://www.readbyqxmd.com/read/28878446/review-of-redo-kasai-portoenterostomy-for-biliary-atresia-in-the-transition-to-the-liver-transplantation-era
#10
REVIEW
Wataru Sumida, Hiroo Uchida, Yujiro Tanaka, Takahisa Tainaka, Chiyoe Shirota, Naruhiko Murase, Kazuo Oshima, Ryo Shirotsuki, Kousuke Chiba
Portoenterostomy (PE) is the standard therapy for biliary atresia (BA). PE offers the chance of survival to children with BA. PE was the ultimate therapeutic modality for BA before liver transplantation (LT) was available. Failure of biliary drainage with PE was almost invariably fatal in children with BA. In such cases, redo-PE was performed to salvage patients following PE failure. PE remains the standard first treatment for BA despite the availability of LT. Further, redo-PE is also performed in a limited number of cases despite the development of LT as an alternative means of PE...
August 2017: Nagoya Journal of Medical Science
https://www.readbyqxmd.com/read/28871567/a-case-of-biliary-atresia-with-pancreaticobiliary-maljunction
#11
Kosuke Endo, Akiko Yokoi, Yasuhiko Mishima, Akihiko Tamaki, Keiichi Morita, Yuichi Okata, Chieko Hisamatsu, Hiroaki Fukuzawa, Makiko Yoshida, Yoshinobu Akasaka, Kosaku Maeda
BACKGROUND: The pathogenesis of biliary atresia (BA) is still unknown. There are several reports on the etiology of BA, including pancreaticobiliary maljunction (PBM). We experienced a case of Kasai type IIIa BA with PBM, in which we found elevation of pancreatic enzymes in the gallbladder. We evaluated whether PBM is related to the pathogenesis of BA based on our findings. CASE PRESENTATION: The patient was born at 40 weeks of gestation. His body weight at birth was 2850 g...
September 5, 2017: Surgical Case Reports
https://www.readbyqxmd.com/read/28871319/onset-ages-of-hepatopulmonary-syndrome-and-pulmonary-hypertension-in-patients-with-biliary-atresia
#12
Takehisa Ueno, Ryuta Saka, Yuichi Takama, Hiroaki Yamanaka, Yuko Tazuke, Kazuhiko Bessho, Hiroomi Okuyama
PURPOSE: Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PoPH) are extrahepatic complications of biliary atresia (BA). Their detection is sometimes delayed, which may result in missed opportunities for liver transplantation. The aim of this study was to determine the onset ages of HPS and PoPH in BA patients. METHODS: BA patients followed at our institution were identified. Patients visited our clinic for routine blood work, as well as regular electrocardiography, chest X-rays, and arterial blood gas tests...
September 4, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28865156/gene-disease-associations-identify-a-connectome-with-shared-molecular-pathways-in-human-cholangiopathies
#13
Zhenhua Luo, Anil G Jegga, Jorge A Bezerra
Cholangiopathies are a diverse group of progressive diseases whose primary cell targets are cholangiocytes. To identify shared pathogenesis and molecular connectivity among the three main human cholangiopathies (biliary atresia [BA], primary biliary cholangitis [PBC] and primary sclerosing cholangitis [PSC]), we built a comprehensive platform of published data on gene variants, gene expression and functional studies, and applied network-based analytics in search for shared molecular circuits. Mining the data platform with largest connected component and interactome analyses, we validated previously reported associations and identified essential- and hub-genes...
September 2, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28860900/portal-vein-stenting-for-delayed-jejunal-varix-bleeding-associated-with-portal-venous-occlusion-after-hepatobiliary-and-pancreatic-surgery
#14
Dongho Hyun, Kwang Bo Park, Sung Ki Cho, Hong Suk Park, Sung Wook Shin, Sung Wook Choo, Young Soo Do, In Wook Choo, Dong Wook Choi
OBJECTIVE: The study aimed to describe portal stenting for postoperative portal occlusion with delayed (≥ 3 months) variceal bleeding in the afferent jejunal loop. MATERIALS AND METHODS: Eleven consecutive patients (age range, 2-79 years; eight men and three women) who underwent portal stenting between April 2009 and December 2015 were included in the study. Preoperative medical history and the postoperative clinical course were reviewed. Characteristics of portal occlusion and details of procedures were also investigated...
September 2017: Korean Journal of Radiology: Official Journal of the Korean Radiological Society
https://www.readbyqxmd.com/read/28852838/prognostic-factors-indicating-survival-with-native-liver-after-kasai-procedure-for-biliary-atresia
#15
Juma Obayashi, Kohei Kawaguchi, Shutaro Manabe, Hideki Nagae, Munechika Wakisaka, Junki Koike, Masayuki Takagi, Hiroaki Kitagawa
BACKGROUND: The number of the bile ducts in the portal canal/measured surface area of the portal canal (BDP ratio) indicates prognosis in biliary atresia (BA), as does an elevated cytokeratin 7 positivity percentage (PCK7). We compared these two markers. METHODS: We reviewed 32 BA cases undergoing Kasai operation from 1976 to 2016 with >5 portal canals in biopsy samples. Group I required liver transplantation or died within a year of operation (n = 8). Group II survived with their native liver (n = 24)...
August 29, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28844953/how-the-biliary-tree-maintains-immune-tolerance
#16
REVIEW
Haiyan Zhang, Patrick S C Leung, M Eric Gershwin, Xiong Ma
The liver is a vital organ with distinctive anatomy, histology and heterogeneous cell populations. These characteristics are of particular importance in maintaining immune homeostasis within the liver microenvironments, notably the biliary tree. Cholangiocytes are the first line of defense of the biliary tree against foreign substances, and are equipped to participate through various immunological pathways. Indeed, cholangiocytes protect against pathogens by TLRs-related signaling; maintain tolerance by expression of IRAK-M and PPARγ; limit immune response by inducing apoptosis of leukocytes; present antigen by expressing human leukocyte antigen molecules and costimulatory molecules; recruit leukocytes to the target site by expressing cytokines and chemokines...
August 24, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28838451/hepatic-artery-reconstruction-using-3-in-1-segmental-resection-in-pediatric-living-donor-liver-transplantation-a-case-report-and-literature-review
#17
Y Luo, D Zhao, M Zhang, T Zhou, B-J Qiu, J-J Zhang, Q Xia
We report a transplant of the left lateral liver segments with 3 arteries for a pediatric recipient from a living donor. A 6-month-old female infant was diagnosed with liver cirrhosis secondary to biliary atresia and scheduled for living donor liver transplantation (LDLT; mother as donor). Left lateral hepatectomy was performed at the donor site. The dissection of the left hepatic artery (HA), which was divided immediately after its origin, showed 3 branches for segments II, III, and IV. The arteries for segment II, segment III, and segment IV were anastomosed to the recipient HA...
September 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28834223/liver-and-systemic-hemodynamics-in-cirrhotic-children-impact-on-the-surgical-management-in-pediatric-living-donor-liver-transplantation
#18
Catherine de Magnée, Francis Veyckemans, Thierry Pirotte, Renaud Menten, Dana Dumitriu, Philippe Clapuyt, Karlien Carbonez, Catherine Barrea, Thierry Sluysmans, Christine Sempoux, Isabelle Leclercq, Francis Zech, Xavier Stephenne, Raymond Reding
BACKGROUND AND AIMS: Cirrhosis in adults is associated with modifications of systemic and liver hemodynamics, whereas little is known about the pediatric population. The aim of this work was to investigate whether alterations of hepatic and systemic hemodynamics were correlated with cirrhosis severity in children. The impact of hemodynamic findings on surgical management in pediatric living donor liver transplantation was evaluated. METHODS: Liver and systemic hemodynamics were studied prospectively in 52 children (median age= 1 year; 33 biliary atresia)...
August 17, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/28834194/pediatric-liver-transplantation-for-hepatocellular-cancer-and-rare-liver-malignancies-us-multi-and-single-center-experience-1981-2015
#19
Rohan Vinayak, Ruy J Cruz, Sarangarajan Ranganathan, Ravi Mohanka, George Mazariegos, Kyle Soltys, Geoff Bond, Sameh Tadros, Abhinav Humar, J Wallis Marsh, Robert R Selby, Jorge Reyes, Kimberly Haberman, Rakesh Sindhi
Pediatric liver transplantation (LTx) is increasingly performed for rare unresectable liver malignancies other than hepatoblastoma (HBL). PURPOSE: We performed a retrospective review of outcomes after LTx for malignancy in the multi-center US Scientific Registry for Transplant Recipients (SRTR, n=677, 1987-2015). We then reviewed the Children's Hospital of Pittsburgh (CHP, n=74, 1981-2014) experience focusing on LTx for unresectable hepatocellular cancer (HCC), non-HBL embryonal tumors (EMB) and metastatic liver tumors (METS)...
August 17, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/28834141/fatal-graft-versus-host-disease-after-living-donor-liver-transplantation-from-an-hla-dr-mismatched-donor
#20
Keita Shimata, Rieko Sakamoto, Tadashi Anan, Koushi Uchida, Masaki Honda, Masahiko Kouroki, Tomonari Urabe, Shintaro Hayashida, Hidekazu Yamamoto, Yasuhiko Sugawara, Yukihiro Inomata
Acute GVHD is a rare complication after liver transplantation that has a high mortality rate. We experienced an infant case complicated with acute GVHD. An 8-month-old infant with biliary atresia underwent LDLT with a graft obtained from his mother. Their HLAs showed a donor-dominant one-way match, not at HLA-DR but at HLA-A, HLA-B, and HLA-C (recipient; A 31/33, B 51/54, C 1/14, DR 9/11, donor; A 31/-, B 51/-, C 14/-, DR 8/11). The patient exhibited a high fever, skin rash, and diarrhea, and was diagnosed with acute GVHD based on the blood chimerism test...
August 19, 2017: Pediatric Transplantation
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