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Biliary atresia

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https://www.readbyqxmd.com/read/27914924/the-management-of-childhood-liver-diseases-in-adulthood
#1
REVIEW
D Joshi, N Gupta, M Samyn, M Deheragoda, F Dobbels, M A Heneghan
An increasing number of patients with childhood liver disease survive into adulthood. These young adults are now entering adult services and require ongoing management. Aetiologies can be divided into liver diseases that develop in young adults which present to adult hepatologists i.e. biliary atresia and Alagille syndrome or liver diseases that occur in children/adolescents and adults i.e. autoimmune hepatitis or Wilson's disease .To successfully manage these young adults, a dynamic and responsive transition service is essential...
November 30, 2016: Journal of Hepatology
https://www.readbyqxmd.com/read/27890981/neonatal-cholestasis-single-centre-experience-in-central-india
#2
Mayank Jain, Sagar Adkar, Chandrashekhar Waghmare, Jenisha Jain, Shikhar Jain, Kamna Jain, Gouri Rao Passi, Rashmi Shad Vinay, M K Soni
BACKGROUND: Neonatal cholestasis syndrome (NCS) is a major cause of morbidity and mortality in infants. The disorder has rarely been studied in centers from Central India. OBJECTIVES: To study the prevalence, clinical presentation and etiology of NCS at a tertiary referral center in Central India. MATERIALS AND METHODS: The study was carried out at a tertiary referral center in Central India. The study is a descriptive study. The records of all patients with suspected NCS treated in the Department of Pediatrics from 2007-2012 were analyzed...
October 2016: Indian Journal of Community Medicine
https://www.readbyqxmd.com/read/27890655/silver-nanoparticle-treatment-ameliorates-biliary-atresia-syndrome-in-rhesus-rotavirus-inoculated-mice
#3
Ruizhong Zhang, Zefeng Lin, Vincent Ch Lui, Kenneth Ky Wong, Paul Kh Tam, Puiyan Lee, Chun Nam Lok, Jonathan R Lamb, Yan Chen, Huimin Xia
Biliary atresia (BA) is a neonatal biliary system disease closely associated with viral infection and bile duct inflammation. Silver nanoparticles (AgNps) have previously revealed antiviral and anti-inflammatory properties. In this study, we have investigated the effects of AgNps in the treatment of the Rhesus rotavirus inoculation induced BA in mice. The morphology, liver histopathology, clinical biochemistry examination, and inflammatory cells were analyzed in BA mice. Results indicated that AgNps could significantly increase the survival rate of BA mice, and reduce jaundice and weight lost and the liver enzymes and bilirubin metabolism clinical parameters were close to the normal levels...
November 25, 2016: Nanomedicine: Nanotechnology, Biology, and Medicine
https://www.readbyqxmd.com/read/27882406/bowel-perforation-after-liver-transplantation-for-biliary-atresia-a-retrospective-study-of-care-in-the-transition-from-children-to-adulthood
#4
Yusuke Yanagi, Toshiharu Matsuura, Makoto Hayashida, Yoshiaki Takahashi, Koichiro Yoshimaru, Genshirou Esumi, Tomoaki Taguchi
PURPOSE: We evaluated the outcomes of liver transplantation (LT) in pediatric and adult patients with biliary atresia (BA). We focused on bowel perforation after LT (BPLT) as the most common surgical complication and analyzed its risk factors. METHODS: This was a retrospective analysis of 70 BA patients who underwent LT. The patients were divided into three groups according to the timing of LT: within the first year of age (Group A), between 1 and 12 years of age (Group B), and after 12 years of age (Group C)...
November 23, 2016: Pediatric Surgery International
https://www.readbyqxmd.com/read/27862929/effects-of-recipient-size-and-allograft-type-on-pediatric-liver-transplantation-for-biliary-atresia
#5
Sophoclis P Alexopoulos, Victor Nekrasov, Shu Cao, Susan Groshen, Navpreet Kaur, Yuri S Genyk, Lea Matsuoka
RATIONALE: The majority of pediatric patients with end stage liver disease receive a transplant with a whole liver allograft. However, smaller recipients with biliary atresia may have improved outcomes with deceased donor partial liver or living donor allografts. This study compares the national outcomes for liver transplantation in biliary atresia, with attention to the interaction between liver allograft type and recipient size. RESULTS: From 1/2/2002 to 12/30/2014, 2123 pediatric patients underwent a primary liver transplant for biliary atresia...
November 10, 2016: Liver Transplantation
https://www.readbyqxmd.com/read/27859955/seventeen-years-of-kasai-portoenterostomy-for-biliary-atresia-in-a-single-southeast-asian-paediatric-centre
#6
Li Wei Chiang, Chuan Yaw Lee, Gita Krishnaswamy, Shireen Anne Nah, Ajmal Kader, Christina Ong, Yee Low, Kong Boo Phua
AIM: Biliary atresia (BA) has preponderance in Asian populations with Kasai portoenterostomy (KP) regarded as the first-line standard of care. Yet reports from Southeast Asia remain scant. This study reviews the demographics, short- and medium-term outcomes for our cohort, and evaluates prognostic factors for outcome. METHODS: All patients diagnosed with BA between January 1997 and December 2013 were included. Clinical data were obtained from medical records. Jaundice clearance was defined as total bilirubin < 20 µmol/L within 6 months after KP...
November 8, 2016: Journal of Paediatrics and Child Health
https://www.readbyqxmd.com/read/27859498/the-srl-peptide-of-rhesus-rotavirus-vp4-protein-governs-cholangiocyte-infection-and-the-murine-model-of-biliary-atresia
#7
Sujit K Mohanty, Bryan Donnelly, Inna Lobeck, Ashley Walther, Phylicia Dupree, Abigail Coots, Jaroslaw Meller, Monica McNeal, Karol Sestak, Greg Tiao
: Biliary atresia (BA) is a neonatal obstructive cholangiopathy which progresses to end stage liver disease, often requiring transplantation. The murine model of BA, employing rhesus rotavirus (RRV), parallels human disease and has been used to elucidate mechanistic aspects of a virus induced biliary cholangiopathy. We previously reported that RRV VP4 gene plays an integral role in activating the immune system and induction of BA. Utilizing rotavirus binding and blocking assays, this study elucidated how RRV VP4 protein governs cholangiocyte susceptibility to infection both in vitro and in vivo in the murine model of BA...
November 17, 2016: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/27830578/adjuvant-steroid-treatment-following-kasai-portoenterostomy-and-clinical-outcomes-of-biliary-atresia-patients-an-updated-meta-analysis
#8
Min-Zhong Zhang, Peng-Cheng Xun, Ka He, Wei Cai
BACKGROUND: It is controversial whether adjuvant steroid treatment should be given to biliary atresia (BA) patients following a Kasai portoenterostomy (KPE). This study aimed to quantitatively and systematically evaluate the effect of adjuvant steroid therapy post-KPE in relation to major clinical outcomes of BA patients. METHODS: We systematically reviewed the literature in PubMed, Embase, the Cochrane Library, China Knowledge Resource Integrated Database, Wanfang Database, Scholarly and Academic Information Navigator and manually searched for relevant papers published before August, 2015...
November 3, 2016: World Journal of Pediatrics: WJP
https://www.readbyqxmd.com/read/27824632/ichthyosis-congenita-with-biliary-atresia-a-rare-association
#9
Shrikiran Aroor, Sandeep Kumar, Suneel Mundkur, Katta M Girisha
No abstract text is available yet for this article.
November 7, 2016: Clinical Dysmorphology
https://www.readbyqxmd.com/read/27818959/amlodipine-induced-gingival-overgrowth-in-a-child-after-liver-transplant
#10
André Guollo, Ana Paula Molina Vivas, Rodrigo Nascimento Lopes, Gilda Porta, Fábio Abreu Alves
Drug-induced gingival overgrowth (GO) has been associated with phenytoin, cyclosporine, and calcium channel blocker therapies. This study reports the case of an 11-year-old girl who was referred for evaluation of GO, which had occurred over the last 6 months. Her medical history included a liver transplant due to biliary atresia 3 years ago, immunosuppressive therapy, and hypertension, which is why she was started on a daily intake of amlodipine. The intraoral examination showed generalized GO, and the treatment consisted of a gingivectomy...
July 2016: Autopsy & case reports
https://www.readbyqxmd.com/read/27817193/effect-of-microrna-155-on-the-interferon-gamma-signaling-pathway-in-biliary-atresia
#11
Yu-An Hsu, Chih-Hung Lin, Hui-Ju Lin, Chi-Chun Huang, Hsiu-Chu Lin, Ying-Chi Chen, Ching-Yao Chang, Su-Hua Huang, Jane-Ming Lin, Kuan-Rong Lee, Lei Wan
MicroRNAs (miRNAs) are ~22-nucleotide long RNAs that negatively regulate gene expression and inflammatory responses in eukaryotes. The aim of this work was to evaluate the roles of miRNA (miR)-155 on the interferon-γ (IFN-γ)-induced response in biliary atresia (BA), which is the most common form of pediatric chronic liver disease and a leading indication for pediatric liver transplantation. The expression of miR-155 and the suppressor of cytokine signaling 1 (SOCS1) gene in human and mice liver tissues of BA and healthy controls was evaluated...
December 31, 2016: Chinese Journal of Physiology
https://www.readbyqxmd.com/read/27812908/liver-transplantation-in-infants-with-biliary-atresia-comparison-of-primary-versus-temporary-abdominal-closure
#12
Nagoud Schukfeh, Anna-Charlotte Holland, Dieter P Hoyer, Anja Gallinat, Andreas Paul, Maren Schulze
INTRODUCTION: Biliary atresia (BA) is the most frequent disease leading to liver transplantation (LT) in infants. Since the patients often require transplantation before reaching a body weight of 10 kg, it is necessary to perform living-related or split liver transplantation by using a left lateral segment. However, this graft often exceeds a graft body weight ratio (GBWR) of 4. Because of the size mismatch and poor portal venous blood flow in most recipients, temporary abdominal closure is often favoured...
November 3, 2016: Langenbeck's Archives of Surgery
https://www.readbyqxmd.com/read/27807737/modified-stool-color-card-with-digital-images-was-efficient-and-feasible-for-early-detection-of-biliary-atresia-a-pilot-study-in-beijing-china
#13
Yuan-Yuan Kong, Jin-Qi Zhao, Jie Wang, Lei Qiu, Hai-He Yang, Mei Diao, Long Li, Yan-Hong Gu, Akira Matsui
BACKGROUND: The aim of this pilot study in Beijing, China, was to validate a screening system for early detection of biliary atresia (BA) by using a modified version of the stool color card (SCC). METHODS: From 2013 to 2014, a total of 29 799 live born infants were screened. SCC was distributed in maternal facilities. Guardians were asked to check their infants' stool colors daily using SCC up until four months after birth. The screening results among 92.5% of participants were reported...
November 2016: World Journal of Pediatrics: WJP
https://www.readbyqxmd.com/read/27806795/-infantile-cholestasis-caused-by-cftr-mutation-case-report-and-literature-review
#14
L Li, N L Wang, J Y Gong, J S Wang
Objective: To study the clinical presentation, biochemical features and genetic analysis of an infant with cholestasis related to the CFTR mutations. Method: The clinical presentation, laboratory investigations and management of a case with infantile cholestasis caused by CFTR mutations were summarized and the relevant literature was reviewed. Result: (1) The patient was a 5 months old boy with cholestasis which developed in neonatal period with delayed meconium exclusion.The laparoscopic exploration was performed to exclude biliary atresia because of acholic stool when he was two months old...
November 2, 2016: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/27801781/function-and-regulation-of-micrornas-and-their-potential-as-biomarkers-in-paediatric-liver-disease
#15
REVIEW
Diego A Calvopina, Miranda A Coleman, Peter J Lewindon, Grant A Ramm
MicroRNAs (miRNAs) are short non-coding RNAs involved in biological and pathological processes of every cell type, including liver cells. Transcribed from specific genes, miRNA precursors are processed in the cytoplasm into mature miRNAs and as part of the RNA-induced silencing complex (RISC) complex binds to messenger RNA (mRNA) by imperfect complementarity. This leads to the regulation of gene expression at a post-transcriptional level. The function of a number of different miRNAs in fibrogenesis associated with the progression of chronic liver disease has recently been elucidated...
October 27, 2016: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/27801752/survey-on-clinical-practice-of-primary-prophylaxis-in-portal-hypertension-in-children
#16
Odile Jeanniard-Malet, Mathieu Duché, Alexandre Fabre
Primary prophylaxis in portal hypertension in children is controversial, as there are few studies documenting its efficacy on the risk of bleeding.28 centres out of the 38 we contacted returned a completed questionnaire about their clinical practices. More than 75% of the centres use endoscopy to screen patients diagnosed with portal cavernoma, biliary atresia, cystic fibrosis and other fibrotic chronic liver diseases with suspected portal hypertension.In cases of grade 2 varices with red marks and grade 3 varices more than 90% of centres perform sclerotherapy or endoscopic variceal ligation (EVL)...
October 31, 2016: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/27797067/spontaneous-resolution-of-de-novo-hepatitis-b-after-living-donor-liver-transplantation-with-hepatitis-b-core-antibody-positive-graft-a-case-report
#17
Yasuyuki Hara, Kazuaki Tokodai, Chikashi Nakanishi, Shigehito Miyagi, Naoki Kawagishi
BACKGROUND: Hepatitis B core antibody (HBcAb)-positive graft is reported to cause de novo hepatitis B after liver transplantation with a probability of 38-100 % without prophylaxis. Hepatitis B surface antigen loss is reported to be achieved with a probability of only 3-8 % in the patients treated by antiviral agents. We present an extremely rare case of spontaneous resolution of de novo hepatitis B after living donor liver transplantation (LDLT) with HBcAb-positive graft. CASE PRESENTATION: An 8-year-old female patient underwent LDLT for end-stage biliary atresia using an HBcAb-positive left lobe graft...
December 2016: Surgical Case Reports
https://www.readbyqxmd.com/read/27776008/key-histopathologic-features-of-liver-biopsies-that-distinguish-biliary-atresia-from-other-causes-of-infantile-cholestasis-and-their-correlation-with-outcome-a-multicenter-study
#18
Pierre Russo, John C Magee, Robert A Anders, Kevin E Bove, Catherine Chung, Oscar W Cummings, Milton J Finegold, Laura S Finn, Grace E Kim, Mark A Lovell, Margret S Magid, Hector Melin-Aldana, Sarangarajan Ranganathan, Bahig M Shehata, Larry L Wang, Frances V White, Zhen Chen, Catherine Spino
The liver biopsy guides diagnostic investigation and therapy in infants with undiagnosed cholestasis. Histologic features in the liver may also have prognostic value in the patient with biliary atresia (BA). We assessed the relative value of histologic features in 227 liver needle biopsies in discriminating between BA and other cholestatic disorders in infants enrolled in a prospective Childhood Liver Disease Research Network (ChiLDReN) cohort study by correlating histology with clinical findings in infants with and without BA...
December 2016: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/27749613/king-s-variceal-prediction-score-a-novel-non-invasive-marker-of-portal-hypertension-in-paediatric-chronic-liver-disease
#19
Peter Witters, Dominic Hughes, Palaniswamy Karthikeyan, Somashekara Ramakrishna, Mark Davenport, Anil Dhawan, Tassos Grammatikopoulos
BACKGROUND AND AIMS: Variceal haemorrhage can be a life-threatening complication of chronic liver disease in children. There is limited evidence about the optimal prophylactic management and selection criteria of children who will benefit from upper gastrointestinal endoscopy. METHODS: Children presenting in our centre with suspected portal hypertension or gastrointestinal bleeding and undergoing their first oesophagogastroduodenoscopy between 2005-2012 were included...
October 4, 2016: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/27749391/ultrasound-guided-liver-biopsy-with-gelatin-sponge-pledget-tract-embolization-in-infants-weighing-less-than-10-kg
#20
Matthew P Lungren, Will S Lindquester, Frank Glen Seidel, Nishita Kothary, Eric J Monroe, Giri Shivaram, Anne E Gill, Matthew C Hawkins
OBJECTIVES: The aim of the study was to describe and assess the technical success and safety of ultrasound-guided liver biopsy with gelatin sponge pledget tract embolization technique in infants <10 kg across 3 tertiary pediatric hospitals. MATERIALS AND METHODS: There were 67 pediatric patients weighing <10 kg (36 boys; 31 girls; average age 202 days; average weight 6 kg, range 1.5-9.9 kg) referred for liver biopsy performed with ultrasound guidance and gelatin sponge pledget tract embolization during a 2-year period...
December 2016: Journal of Pediatric Gastroenterology and Nutrition
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