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Biliary atresia

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https://www.readbyqxmd.com/read/28652893/low-bone-mineral-density-and-the-severity-of-cholestasis-in-biliary-atresia
#1
Krittapak Homchan, Tawatchai Chaiwatanarat, Wanvisa Udomsinprasert, Voranush Chongsrisawat, Yong Poovorawan, Sittisak Honsawek
AIM: To investigate the prevalence of osteopenia and osteoporosis in postoperative biliary atresia (BA) children and the association of bone mineral density (BMD) and biochemical parameters in postKasai BA subjects. METHODS: A total of 70 patients with postKasai BA were enrolled in this prospective study. The patients were classified into two groups according to their jaundice status. BMD of the lumbar spine was analyzed using dual energy X-ray absorptiometry. RESULTS: The prevalence of low bone mass (osteopenia and osteoporosis) in BA patients were 51...
June 8, 2017: World Journal of Hepatology
https://www.readbyqxmd.com/read/28647807/preoperative-nutritional-status-and-its-impact-on-cholangitis-after-kasai-portoenterostomy-in-biliary-atresia-patients
#2
Dandan Li, Xiaoai Chen, Kang Fu, Jixin Yang, Jiexiong Feng
OBJECTIVE: To explore the preoperative nutrition status in patients with biliary atresia (BA) and its effect on the cholangitis of Kasai portoenterostomy (KPE). METHODS: 106 patients who had been diagnosed with BA type III and were undergoing KPE were divided into two groups according to the score obtained using STRONGkids. Preoperative and postoperative data were collected to compare general characteristics and postoperative outcomes between the different groups...
June 24, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28638943/elevated-serum-heat-shock-protein-70-and-liver-stiffness-reflect-hepatic-dysfunction-and-severity-in-postoperative-biliary-atresia
#3
Sittisak Honsawek, Wanvisa Udomsinprasert, Napaphat Jirathanathornnukul, Voranush Chongsrisawat, Yong Poovorawan
BACKGROUND: Biliary atresia (BA) is a severe chronic liver disease characterized by progressive obstructive cholangiopathy of biliary tract. Heat shock protein 70 (HSP70) is involved in protecting cells against a wide variety of stress and plays a protective role in tissue damage. The purpose of this study was to investigate serum HSP70 and liver stiffness in BA and determine the association of serum HSP70, liver stiffness, and outcome parameters in post-Kasai BA patients. METHODS: One hundred post-Kasai BA patients and 40 controls were enrolled...
June 21, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28635067/maternal-coping-with-the-prospect-of-liver-transplant-among-their-school-age-children
#4
Katsuhiro Hiratsuka, Nobue Nakamura, Naho Sato
The purpose of the current study was to describe the following: maternal coping with the prospect of becoming the living-donor liver transplant for their child; the daily lives of school-age children surviving biliary atresia with their native liver; and to explore the relationship between these individuals. Semistructured interviews were conducted with 6 school-age children surviving biliary atresia with their native liver and their mothers. The interviews were conducted from June to August 2014, and a qualitative content analysis was used...
June 2017: International Journal of Nursing Practice
https://www.readbyqxmd.com/read/28632515/p4-stump-approach-for-intraoperative-portal-vein-stenting-in-pediatric-living-donor-liver-transplantation-an-innovative-technique-for-a-challenging-problem
#5
Chao-Long Chen, Yu-Fan Cheng, Viola Huang, Ting-Lung Lin, Yi-Chia Chan, Hsin-You Ou, Chee-Chien Yong, Shih-Ho Wang, Chih-Che Lin
OBJECTIVE: The aim of this study was to evaluate the utility of the P4 stump stenting approach for treating portal vein (PV) complications in pediatric living donor liver transplantation (LDLT). BACKGROUND: PV complications cause significant morbidity and mortality in pediatric LDLT. Biliary atresia in the backdrop of pathological PV hypoplasia and sclerosis heightens the complexity of PV reconstruction. The authors developed a novel approach for intraoperative PV stenting via the graft segment 4 PV stump (P4 stump) to address this challenge...
June 19, 2017: Annals of Surgery
https://www.readbyqxmd.com/read/28626977/magnetic-compression-anastomosis-for-postoperative-biliary-atresia
#6
Rei Matsuura, Takehisa Ueno, Yuko Tazuke, Natsumi Tanaka, Hiroaki Yamanaka, Yuichi Takama, Kengo Nakahata, Taku Yamamichi, Noboru Maeda, Keigo Osuga, Eigoro Yamanouchi, Hiroomi Okuyama
We report a case of successful magnetic compression anastomosis (MCA) for obstructed cyst-jejunostomy in a young woman who had undergone surgery for type 1 biliary atresia (BA) on day 78 of life. A 16-year-old girl was admitted with obstructive jaundice. Jaundice resolved with percutaneous trans-hepatic cholangiodrainage (PTCD) but contrast medium injected from the PTCD tube did not flow through the anastomosis. Magnets were placed on each side of the anastomosis, in the cyst and the jejunum, to compress the partition...
June 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/28625694/the-incidence-of-chylous-ascites-after-liver-transplantation-and-the-proposal-of-a-diagnostic-and-management-protocol
#7
Toshiharu Matsuura, Yusuke Yanagi, Makoto Hayashida, Yoshiaki Takahashi, Koichiro Yoshimaru, Tomoaki Taguchi
BACKGROUND: No protocol has been established for the diagnosis and management of chylous ascites after liver transplantation (LT). In this study, we retrospectively reviewed our cases of posttransplant chylous ascites (PTCA) and aimed to propose a diagnostic and management protocol. PATIENTS AND METHODS: We retrospectively reviewed the clinical records of 96 LT recipients who underwent LT at our department. The incidence of PTCA and the associated risk factors were analyzed and our protocol for chylous ascites was evaluated...
June 11, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28615387/screening-for-biliary-atresia-it-s-in-the-cards
#8
Richard A Schreiber, Alison Butler
No abstract text is available yet for this article.
June 2017: Canadian Family Physician Médecin de Famille Canadien
https://www.readbyqxmd.com/read/28594581/learning-curve-analysis-of-laparoscopic-kasai-portoenterostomy
#9
Zhihan Li, Yongqin Ye, Zhouguang Wu, Bin Wang
OBJECTIVE: To compare the surgical outcomes of Kasai Portoenterostomy (PE) and investigate the learning curve of laparoscopic Kasai Portoenterostomy (Lap-PE). MATERIALS AND METHODS: Retrospective chart review of 80 cases of biliary atresia (type III) undergoing Lap-PE at Shenzhen Children's Hospital from January 2011 to June 2015, all of which were performed by the same surgical team. According to the operative sequences, the cases were equally divided into four phases (Phase I, II, III, and IV), which contain 20 cases in each...
June 8, 2017: Journal of Laparoendoscopic & Advanced Surgical Techniques. Part A
https://www.readbyqxmd.com/read/28590589/living-donor-liver-transplantation-for-post-kasai-biliary-atresia-analysis-of-pretransplant-predictors-of-outcomes-in-infants
#10
Toshihiro Kitajima, Seisuke Sakamoto, Kengo Sasaki, Hajime Uchida, Soichi Narumoto, Akinari Fukuda, Satoshi Teramukai, Shinji Uemoto, Mureo Kasahara
After decades of dramatic surgical innovations in pediatric living donor liver transplantation (LDLT), LDLT for biliary atresia (BA) still poses various challenges. This study reviewed our experience with LDLT for children with post-Kasai BA and evaluated outcomes and prognostic factors. From 2005 to 2016, 168 post-Kasai BA LDLT patients were enrolled and divided into three groups by age. Patient characteristics and perioperative data were compared. Predictors of morbidity and mortality following LDLT were analyzed in 93 infants...
June 7, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/28588613/preoperative-serum-il-12p40-is-a-potential-predictor-of-kasai-portoenterostomy-outcome-in-infants-with-biliary-atresia
#11
Shaimaa Samy Goda, Mohamed Ahmed Khedr, Soha Zaki Elshenawy, Tarek Mohamed Ibrahim, Hanaa Ahmed El-Araby, Mostafa Mohamed Sira
The standard-of-care treatment for biliary atresia (BA) is surgical restoration of bile flow by Kasai portoenterostomy. We aimed to study serum interleukin- (IL-) 12p40, a natural antagonist for the proinflammatory IL-12p70, and its relation to surgical outcomes of BA. The study included 75 infants with neonatal cholestasis: BA group (n = 25), non-BA cholestasis group (n = 30), and neglected BA group (n = 20), in addition to thirty healthy neonates serving as controls. IL-12p40 was measured by ELISA in all individuals and a second assessment was performed 3 months postoperatively in the BA group...
2017: Gastroenterology Research and Practice
https://www.readbyqxmd.com/read/28583322/ocular-findings-in-patients-with-cholestatic-disorders-of-infancy-a-single-centre-experience
#12
Hanaa El-Karaksy, Dalia Hamed, Hanan Fouad, Engy Mogahed, Heba Helmy, Fotouh Hasanain
BACKGROUND AND STUDY AIMS: Neonatal cholestasis can be associated with ocular findings that might aid in its diagnosis, e.g., Alagille syndrome (AGS) and Niemann Pick disease (NPD). We aimed to investigate the frequency of ocular manifestations in infants with cholestasis. PATIENTS AND METHODS: This cross-sectional study included cholestatic infants presenting to the Paediatric Hepatology Unit, Cairo University Paediatric Hospital, Cairo, Egypt. All infants underwent examination of lid, ocular motility, anterior and posterior segments and measurement of intraocular pressure, cycloplegic refraction, ocular ultrasonography and vision...
June 2, 2017: Arab Journal of Gastroenterology: the Official Publication of the Pan-Arab Association of Gastroenterology
https://www.readbyqxmd.com/read/28580726/the-first-case-of-domino-split-liver-transplantation-in-maple-syrup-urine-disease
#13
Uta Herden, Jun Li, Lutz Fischer, Florian Brinkert, Martin Blohm, René Santer, Bjoern Nashan, Enke Grabhorn
The enzymatic defect in MSUD results in accumulation of neurotoxic metabolites of BCAAs. LTX has shown to be a feasible strategy in patients non-responsive to diet. Because of sufficient enzyme activity in extrahepatic tissues in healthy people, the MSUD liver graft is a suitable domino organ. We present the first case of a technical challenging ex situ split of a MSUD domino organ transplanted into two pediatric recipients. The domino graft donor was a 21-year-old female (58 kg) suffering from MSUD with recurrent metabolic decompensation despite strict diet...
June 5, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28572448/structural-and-functional-dissection-of-the-interplay-between-lipid-and-notch-binding-by-human-notch-ligands
#14
Richard J Suckling, Boguslawa Korona, Pat Whiteman, Chandramouli Chillakuri, Laurie Holt, Penny A Handford, Susan M Lea
Recent data have expanded our understanding of Notch signalling by identifying a C2 domain at the N-terminus of Notch ligands, which has both lipid- and receptor-binding properties. We present novel structures of human ligands Jagged2 and Delta-like4 and human Notch2, together with functional assays, which suggest that ligand-mediated coupling of membrane recognition and Notch binding is likely to be critical in establishing the optimal context for Notch signalling. Comparisons between the Jagged and Delta family show a huge diversity in the structures of the loops at the apex of the C2 domain implicated in membrane recognition and Jagged1 missense mutations, which affect these loops and are associated with extrahepatic biliary atresia, lead to a loss of membrane recognition, but do not alter Notch binding...
June 1, 2017: EMBO Journal
https://www.readbyqxmd.com/read/28556285/diagnostic-performance-of-sonographic-features-in-patients-with-biliary-atresia-a-systematic-review-and-meta-analysis
#15
Hee Mang Yoon, Chong Hyun Suh, Jeong Rye Kim, Jin Seong Lee, Ah Young Jung, Young Ah Cho
OBJECTIVES: To evaluate the diagnostic performance of sonographic features of biliary atresia in patients with infantile cholestasis. METHODS: The Ovid-MEDLINE and EMBASE databases were searched for studies of the diagnostic performance of sonographic features of biliary atresia in patients with infantile cholestasis. A meta-analysis was performed to evaluate the diagnostic performance of the triangular cord sign and other sonographic features in patients with biliary atresia...
May 27, 2017: Journal of Ultrasound in Medicine: Official Journal of the American Institute of Ultrasound in Medicine
https://www.readbyqxmd.com/read/28531808/prenatal-diagnosis-of-biliary-atresia-a-case-series
#16
O Shen, H Y Sela, H Nagar, R Rabinowitz, E Jacobovich, D Chen, E Granot
BACKGROUND: Biliary atresia is a progressive disease presenting with jaundice, and is the most common indication for liver transplantation in the pediatric population. Prenatal series have yielded conflicting results concerning a possible association between BA and prenatal nonvisualization of the gallbladder. AIMS: This retrospective case series was performed to assess the association between biliary atresia, prenatal nonvisualization of the gallbladder and other sonographic signs...
May 19, 2017: Early Human Development
https://www.readbyqxmd.com/read/28526690/emerging-concepts-in-biliary-repair-and-fibrosis
#17
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
#18
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
#19
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
#20
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...
July 2017: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
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