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Biliary atresia

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...
October 21, 2016: American Journal of Surgical Pathology
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
Matthew P Lungren, Will S Lindquester, F Glen Seidel, Nishita Kothry, Eric J Monroe, Giri Shivaram, Anne E Gill, Matthew C Hawkins
PURPOSE: To describe and assess the technical success and safety of ultrasound guided liver biopsy with gelatin sponge pledget tract embolization technique in infants less than 10 kg across three tertiary pediatric hospitals. MATERIALS AND METHODS: There were 67 pediatric patients weighing less than 10 kg (36 males; 31 females; average age 202 days; average weight 6 kg, range 1.5 kg to 9.9 kg) referred for liver biopsy performed with ultrasound guidance and gelatin sponge pledget tract embolization during a two year period...
September 30, 2016: Journal of Pediatric Gastroenterology and Nutrition
Max Bolun Liu, Thuy Bui Huong, Xuyen Hoang, Lan Doan, Shauna Trinh, Hoa Pham Anh Nguyen, Hai Thanh Le, Ai-Xuan Holterman
BACKGROUND: Biliary atresia is an idiopathic, neonatal liver disease of the bile ducts. The natural evolution of biliary atresia is known in developed countries. This study describes the clinical course of biliary atresia in Vietnam, a developing country. METHODS: Chart reviews were undertaken of patients treated with or without the Kasai procedure between January 2010 and July 2013 at a children's hospital in Vietnam. RESULTS: Of 287 children with biliary atresia, 149 (52%) were treated without the Kasai procedure and 138 (48%) were treated with the Kasai procedure...
October 12, 2016: Surgery
Yoshiaki Takahashi, Toshiharu Matsuura, Yusuke Yanagi, Koichiro Yoshimaru, Tomoaki Taguchi
BACKGROUND/PURPOSE: There is currently no unified view regarding whether liver transplantation or splenectomy should be performed for hypersplenism before liver transplantation in biliary atresia (BA) patients. We herein describe the efficacy of splenectomy before liver transplantation. METHODS: Splenectomy was performed in ten patients with hypersplenism associated with BA. We retrospectively reviewed their perioperative and postoperative courses, the number of leukocytes and thrombocytes, and the MELD score...
September 26, 2016: Journal of Pediatric Surgery
Nicholas L Webb, Ashish Jiwane, Chee Y Ooi, Scott Nightinghale, Susan E Adams, Usha Krishnan
AIM: Biliary atresia (BA) literature has focussed on the relationship between age at Kasai procedure (KP) and post-KP outcomes. This study primarily examines post-KP outcomes including, 6-month normalisation of bilirubin, 5-year native liver survival (NLS), development of portal hypertension (PHT) and incidence of ascending cholangitis at a single tertiary paediatric centre in Australia. The study also evaluated prognostic factors which may influence these aforementioned outcomes. METHODS: Retrospective chart review of all BA cases between 1999 and 2014...
October 7, 2016: Journal of Paediatrics and Child Health
J Y Wang, Z H Li, M Ye, Q Feng, Z M Chen, X S Ye, Z G Wu, B Wang, L Liu, J Yao
Biliary atresia (BA) is a destructive bile duct disease occurring in newborn children within a few weeks after birth. In this study, the effect of miR-29c and miR-129-5p on epithelial-mesenchymal transition (EMT) in experimental BA was explored by constructing BA mouse models via Rhesus rotavirus vaccine infection. miR-29c and miR-129-5p expression was analyzed by real-time quantitative polymerase chain reaction. EMT was established by induction with transforming growth factor (TGF)-β1. miR-29c and miR-129-5p were overexpressed and inhibited, respectively, by Lipofectamine transfection...
September 2, 2016: Genetics and Molecular Research: GMR
Zhen Shen, Shan Zheng, Rui Dong, Gong Chen
PURPOSE: We aimed to study whether saturation in HSV color model could be a parameter for acholic stool and utilized for designing a mobile application for screening biliary atresia (BA). METHODS: Saturations of the colors in the three validated stool color cards (Taiwan, Japan, Britain) were read using PHOTOSHOP. Stools from 40 BA patients and 40 in-hospital neonates with pneumonia were photographed and analyzed with color-analyzing mobile applications. RESULTS: Saturations of normal colors in the published stool cards were all >50% (67%~99%, median 85%) and were all <50% (7~47%, median 25%) for abnormal colors...
September 16, 2016: Journal of Pediatric Surgery
Hiroki Nakamura, Takafumi Kawano, Katsumi Yoshizawa, Hideaki Nakajima, Kazuto Suda, Hiroyuki Koga, Kazuhiko Nakame, Satoshi Ieiri, Shigeru Takamizawa, Naoto Urushihara, Toshihiro Yanai, Atsuyuki Yamataka
PURPOSE: We present a first report of the long-term follow-up of biliary atresia (BA) patients who became anicteric with the native liver (ANL; total bilirubin <1.5mg/dL) after redo-Kasai. METHODS: Forty-six redo-Kasai cases (1984-2015) were the subjects for this study. ANL ratios were determined using the Kaplan-Meier estimate. RESULTS: BA type was I (n=3), II (n=1), and III (n=42). Mean ages (initial/redo) at Kasai were 60.3/231.9days, respectively...
September 16, 2016: Journal of Pediatric Surgery
Masaki Nio, Motoshi Wada, Hideyuki Sasaki, Takuro Kazama, Hiromu Tanaka, Hironori Kudo
BACKGROUND: The purpose of this study was to assess the clinical outcome of patients treated with the current technique of Kasai procedure compared with that of those treated with previous procedures. METHODS: We retrospectively assessed the outcomes of 256 patients with biliary atresia who underwent Kasai portoenterostomy at our hospital between 1972 and 2014. Patients were divided into four groups: group 1 (1972-1981, double Roux-en Y, n=91), group 2 (1982-1991, Suruga II, n=80), group 3 (1992-2000, double-valve Roux-en Y, n=46), and group 4 (2001-2014, total removal of the extrahepatic biliary remnants at hepatic capsule and Roux-en Y reconstruction with a spur valve, n=39)...
September 16, 2016: Journal of Pediatric Surgery
Yukihiro Sanada, Yasunaru Sakuma, Hideki Sasanuma, Atsushi Miki, Takumi Katano, Yuta Hirata, Noriki Okada, Naoya Yamada, Yoshiyuki Ihara, Taizen Urahashi, Naohiro Sata, Yoshikazu Yasuda, Koichi Mizuta
Utilizing the opened round ligament as venous grafts during liver transplantation is useful but controversial, and there are no pathological analyses of this procedure. Herein, we describe the first reported case of a pathological analysis of an opened round ligament used as a venous patch graft in a living donor liver transplantation (LDLT). A 13-year-old female patient with biliary atresia underwent LDLT using a posterior segment graft from her mother. The graft had two hepatic veins (HVs), which included the right HV (RHV; 15 mm) and the inferior RHV (IRHV; 20 mm)...
September 14, 2016: World Journal of Gastroenterology: WJG
Michael R Bowden, Looi C Ee, Usha Krishnan, Edward V O'Loughlin, Winita Hardikar, Diana Carmody, Cassandra Hainsworth, Vicki Jermyn, Mee-Mee Lee, Janine Sawyer, Michael Stormon, Kathe Holmes, Daniel A Lemberg, Andrew S Day, Campbell Paul, Philip Hazell
OBJECTIVES: Research is lacking into the emotional effects on families of serious chronic illness in infants. We examined the impact of the diagnosis of serious liver disease in infants upon parent psychological symptoms and family functioning. We hypothesized that parent psychological symptoms, family functioning and father engagement will predict infant emotional outcomes. METHODS: Parents of infants recently diagnosed with serious liver disease completed validated questionnaires about parent stress, family function, impact of the illness on the family, and father engagement...
September 24, 2016: Journal of Pediatric Gastroenterology and Nutrition
Inna Lobeck, Bryan Donnelly, Phylicia Dupree, Maxime M Mahe, Monica McNeal, Sujit K Mohanty, Greg Tiao
The Rhesus rotavirus (RRV) induced murine model of biliary atresia (BA) is a useful tool in studying the pathogenesis of this neonatal biliary obstructive disease. In this model, the mitogen associated protein kinase pathway is involved in RRV infection of biliary epithelial cells (cholangiocytes). We hypothesized that extracellular signal-related kinase (ERK) phosphorylation is integral to calcium influx, allowing for viral replication within the cholangiocyte. Utilizing ERK and calcium inhibitors in immortalized cholangiocytes and BALB/c pups, we determined that ERK inhibition resulted in reduced viral yield and subsequent decreased symptomatology in mice...
September 23, 2016: Virology
Kang Li, Xi Zhang, Li Yang, Xin-Xing Wang, De-Hua Yang, Guo-Qing Cao, Shuai Li, Yong-Zhong Mao, Shao-Tao Tang
Biliary atresia (BA) is a pediatric inflammatory disease of the biliary system which leads to cirrhosis and the need for liver transplantation. Various cells types have been reported to participate in the pro-inflammatory response, including Th1, Th2, Th17, CD8(+) T cells, or NK cells. The suppressive Treg cells, on the contrary, were not functioning properly. The underlying mechanism is largely unknown. Focusing on why the suppressive function of Treg was impaired, we found out methylation status of CpG islands within the Foxp3 promotor of Tregs in BA patients and murine models were both increased...
September 22, 2016: American Journal of Physiology. Gastrointestinal and Liver Physiology
Abdelmoneim Em Kheir, Wisal Ma Ahmed, Israa Gaber, Sara Ma Gafer, Badreldin M Yousif
Cholestasis in early infancy represents a diagnostic dilemma and most of these infants suffer either from extrahepatic biliary atresia or idiopathic neonatal hepatitis. Differentiation between the two conditions may be extremely difficult both clinically and biochemically, and a diagnostic liver biopsy is usually required. We report on a Sudanese infant who presented at the age of 4 weeks with prolonged cholestatic jaundice, abdominal ultrasound was inconclusive, HIDA scan was suggestive of extrahepatic biliary atresia and the diagnosis of idiopathic neonatal hepatitis was only reached by liver biopsy...
2016: Sudan J Paediatr
Shikha S Sundaram, Cara L Mack, Amy G Feldman, Ronald J Sokol
Biliary Atresia is a progressive, fibro-obliterative disorder of the intra and extrahepatic bile ducts in infancy. The majority of affected children will eventually develop end-stage liver disease and require liver transplantation. Indications for liver transplant in biliary atresia include failed Kasai portoenterostomy, significant and recalcitrant malnutrition, recurrent cholangitis, and the progressive manifestations of portal hypertension. Extra-hepatic complications of this disease, such as hepatopulmonary syndrome and portopulmonary hypertension, are also indications for liver transplantation...
September 21, 2016: Liver Transplantation
Celine S Lages, Julia Simmons, Avery Maddox, Keaton Jones, Rebekah Karns, Rachel Sheridan, Shiva Kumar Shanmukhappa, Sujit Mohanty, Matthew Kofron, Pierre Russo, Yui-Hsi Wang, Claire Chougnet, Alexander G Miethke
: Biliary atresia is a fibroinflammatory obstruction of the extrahepatic biliary tree in neonates. While intrahepatic bile duct proliferation is universal at diagnosis, bile duct paucity develops later. We hypothesized that polarized T helper lymphocyte responses orchestrate progression of intrahepatic biliary injury in this disease. IL-17A-GFP, CD11c/DTR and IL17RA-/- mice were used to examine T-lymphocyte polarization, inflammatory leukocyte recruitment and biliary injury in rhesus-rotavirus induced biliary atresia...
September 19, 2016: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
Ken Liu, Vikram Joshi, Payal Saxena, Arthur J Kaffes
BACKGROUND AND AIM: Endoscopic retrograde cholangiopancreatography (ERCP) in patients with roux-en-Y anastomosis (REYA) is challenging. Use of double balloon enteroscope-assisted ERCP (DBE-ERCP) has been successful. We aim to determine predictors of successful biliary cannulation with DBE-ERCP in patients with REYA. METHODS: We retrospectively studied patients with REYA who had DBE-ERCP between 2009 and 2015. RESULTS: 86 DBE-ERCP were done on 52 patients...
September 17, 2016: Digestive Endoscopy: Official Journal of the Japan Gastroenterological Endoscopy Society
Takeshi Saito, Akemi Sakamoto, Masahiko Hatano, Jun Iwai, Yasuyuki Higashimoto, Hideo Yoshida
Purpose Systemic and local immune environments in human biliary atresia (BA) were analyzed. Methods Plasma concentrations of 19 inflammatory components in 16 preoperative BA patients (median age, 51 days), 13 normal controls (NCs) (44 days), and 15 cholestatic controls (CC) (23 days) were measured using flow cytometry, and compared according to post-Kasai outcomes in BA patients. Hepatic mRNA levels of representative helper T (Th) cell cytokines and forkhead box protein 3 (FoxP3) quantified by real-time reverse transcription polymerase chain reaction were compared between BA and non-BA...
September 16, 2016: European Journal of Pediatric Surgery
Yung Fong Tsai, Hsiu Pin Chen, Fu Chao Liu, Shih Hao Liu, Chun Yu Chen, Chih Wen Cheng, Jr Rung Lin
Post-transplant malignancy is a major cause of late mortality for liver transplant recipients (LTRs). This nationwide population-based cohort study investigated the cancer type, incidence, and risk factors associated with post-transplant malignancies in 2938 Taiwanese LTRs who underwent transplantation between 1998 and 2012. Data from the National Health Insurance Research Database were extracted on the basis of the International Classification of Disease, Ninth Revision, Clinical Modification codes. Among these patients, 284 post-transplant malignancies were diagnosed...
September 10, 2016: Oncotarget
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