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Biliary atresia

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https://www.readbyqxmd.com/read/28100381/-trend-of-mortality-of-congenital-malformation-in-children-aged-5-years-in-beijing-2006-2015
#1
J Wang, D Y Li, W X Zhang, Y C Li, J Wang
Objective: To investigate the change in mortality of congenital malformation in children aged <5 years in Beijing from 2006 to 2015. Methods: Using the death surveillance data in children aged <5 years in Beijing from 2006 to 2015, which was collected from the real-time surveillance network, we calculated the area and age distributions of the mortality of congenital malformation in children aged <5 years in Beijing. Meanwhile, the variations of age, time and space in the causes of deaths were discussed...
January 10, 2017: Zhonghua Liu Xing Bing Xue za Zhi, Zhonghua Liuxingbingxue Zazhi
https://www.readbyqxmd.com/read/28090475/use-of-omega-3-polyunsaturated-fatty-acids-to-treat-inspissated-bile-syndrome-a-case-report
#2
Woo Young Jun, Min Jeng Cho, Hye Seung Han, Sun Hwan Bae
Inspissated bile syndrome (IBS) is a rare condition in which thick intraluminal bile, including bile plugs, sludge, or stones, blocks the extrahepatic bile ducts in an infant. A 5-week-old female infant was admitted for evaluation of jaundice and acholic stool. Diagnostic tests, including ultrasound sonography, magnetic resonance cholangiopancreatography, and a hepatobiliary scan, were not conclusive. Although the diagnosis was unclear, the clinical and laboratory findings improved gradually on administration of urodeoxycholic acid and lipid emulsion containing omega-3 polyunsaturated fatty acids (PUFAs) for 3 weeks...
December 2016: Pediatric Gastroenterology, Hepatology & Nutrition
https://www.readbyqxmd.com/read/28079575/cystic-biliary-atresia-and-choledochal-cysts-are-distinct-histopathologic-entities
#3
Inna N Lobeck, Rachel Sheridan, Mark Lovell, Phylicia Dupree, Greg M Tiao, Kevin E Bove
Cystic biliary atresia (CBA), a rare cystic expansion of atretic extrahepatic bile ducts in young infants, overlaps in age at presentation and imaging features with early choledochal cysts (CC). Treatment and prognosis differ; histologic differences are unsettled. We compared 10 patients with CBA, 1975 to 2015, to an age-similar cohort of 13 infants, and to older patients who had surgery for CC. Operative details, imaging, and clinical courses were correlated to pathologic specimens. Immunostains for smooth muscle actin and myosin heavy chain were used to evaluate cyst walls and atretic segments...
January 10, 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28060195/quality-of-life-and-parental-worrying-in-a-national-cohort-of-biliary-atresia-children-living-with-their-native-livers
#4
Hanna Lampela, Mikko P Pakarinen, Timo Jahnukainen, Hannu Jalanko, Silja Kosola
OBJECTIVES: To evaluate health related quality of life (HRQoL) and parental distress in a national cohort of children with biliary atresia (BA) with their native livers in relation to BA complications and HRQoL of normal population controls. METHODS: We invited all Finnish children with BA surviving with their native livers at age 2-18 years to participate in 2009 and in 2014. Parents filled the PedsQL proxy questionnaire, a survey of their child's health and evaluated parental distress on a visual analogue scale from 0 to 7...
January 4, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28052132/association-between-promoter-hypomethylation-and-overexpression-of-autotaxin-with-outcome-parameters-in-biliary-atresia
#5
Wanvisa Udomsinprasert, Nakarin Kitkumthorn, Apiwat Mutirangura, Voranush Chongsrisawat, Yong Poovorawan, Sittisak Honsawek
OBJECTIVE: Biliary atresia (BA) is a progressive fibroinflammatory liver disease. Autotaxin (ATX) has a profibrotic effect resulting from lysophosphatidic acid activity. The purpose of this study was to examine ATX expression and ATX promoter methylation in peripheral blood leukocytes and liver tissues from BA patients and controls and investigate their associations with outcome parameters in BA patients. METHODS: A total of 130 subjects (65 BA patients and 65 age-matched controls) were enrolled...
2017: PloS One
https://www.readbyqxmd.com/read/28051047/evaluation-of-the-use-of-laparoscopic-guided-cholecystocholangiography-and-liver-biopsy-in-definitive-diagnosis-of-neonatal-cholestatic-jaundice
#6
Khalid Shreef, Abdullah Alhelal
BACKGROUND: Once it is established that a jaundiced infant has direct hyperbilirubinemia, the principal diagnostic concern is to differentiate hepatocellular from obstructive cholestasis. Traditional tests such as ultrasonography, percutaneous liver biopsy and technetium 99 m hepatobiliary iminodiacetic acid (HIDA) scan are often not sufficiently discriminating. Definitive exclusion of biliary atresia (BA) in the infant with cholestatic jaundice usually requires mini-laparotomy and intra-operative cholangiography...
October 2016: African Journal of Paediatric Surgery: AJPS
https://www.readbyqxmd.com/read/28045767/autophagy-in-hepatocytes-in-infants-with-alpha-1-atd-and-different-liver-disease-outcomes-a-retrospective-analysis
#7
Elżbieta Czarnowska, Agnieszka Bakuła, Joanna B Bierła, Justyna Niderla-Bielińska, Agnieszka Sowińska, Joanna Cielecka-Kuszyk, Piotr Socha
OBJECTIVES: It is unclear whether a distinct activity of pathways removing the AT protein in Alpha-1-Antitrypsin Deficiency (α1ATD) are associated with an unfavorable predisposition toliver disease in the future. The aim of this study was to determine whether liverspecific activity of AT protein disposal occurs at infancy in α1ATD with PiZZ phenotype (ATZ). METHODS: Liver samples of 17 infants with unfavorable ATZ outcome (Group I, n = 8, median age  = 0...
December 30, 2016: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28039901/living-donor-liver-transplantation-in-a-pediatric-patient-with-preexisting-yolk-sac-tumor
#8
Taiichi Wakiya, Yoshikazu Toyoki, Keinosuke Ishido, Daisuke Kudo, Norihisa Kimura, Shinji Tsutsumi, Tadashi Odagiri, Akiko Suto, Chiaki Uchida, Kenichi Hakamada
There is ongoing discussion regarding the indications and timing of LT for patients with a preexisting extrahepatic malignancy. We herein report a pediatric case that underwent LDLT after therapy for YST. The patient, a 13-year-old female with biliary atresia, had undergone portoenterostomy at 2 months of age. She developed a left ovarian tumor with a high serum alpha-fetoprotein concentration at 10 years of age. She underwent left oophorectomy and was diagnosed with ovarian YST (Stage I). After surgery, hepatopulmonary syndrome progressed gradually...
December 30, 2016: Pediatric Transplantation
https://www.readbyqxmd.com/read/28007417/outcomes-of-laparoscopic-kasai-portoenterostomy-for-biliary-atresia-a-systematic-review
#9
Mohammed Hassan Hussain, Naved Alizai, Bijendra Patel
AIMS: Biliary atresia (BA) is a rare disease for which mainstay of treatment consists of open Kasai portoenterostomy. The aim of this review was to assess the outcomes of laparoscopic Kasai portoenterostomy, which offers potential benefits of minimally invasive surgery. Outcomes identified were postoperative cholangitis rates, incidence of adhesions at subsequent liver transplantation, native liver survival rates and actuarial survival rates. METHODS: A comprehensive systematic literature search was conducted in the PubMed and Cochrane databases using the keywords hepatic portoenterostomy, biliary atresia and laparoscopy...
November 14, 2016: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27992499/diffusion-tensor-imaging-for-evaluating-biliary-atresia-in-infants-and-neonates
#10
Bo Liu, Jinhua Cai, Jin Zhu, Helin Zheng, Yun Zhang, Longlun Wang
BACKGROUND: Preliminary studies have shown that diffusion tensor imaging (DTI) is helpful in evaluating liver disorders. However, there is no published literature on the use of DTI in the diagnosis of biliary atresia (BA). This study aimed to investigate the diagnostic value of the liver average apparent diffusion coefficient (ADC) and fractional anisotropy (FA) measured using DTI for BA in neonates and infants. METHODS: Fifty-nine patients with infant jaundice were included in this study...
2016: PloS One
https://www.readbyqxmd.com/read/27989363/complications-in-pediatric-hepatobiliary-surgery
#11
Gabriella Grisotti, Robert A Cowles
This review highlights the complications and their risk factors encountered in pediatric hepatobiliary surgery, specifically in the context of pediatric hepatic resection, excision of choledochal cyst, and the Kasai hepatoportoenterostomy procedure for biliary atresia as well as other procedures potentially affecting the biliary tree. With the understanding that these are relatively rare procedures, case reports and small case series are included in addition to larger series when available. The review focuses on publications in English over the past 15 years...
December 2016: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/27975111/an-overview-of-cirrhosis-in-children
#12
Jonathan Cordova, Hilary Jericho, Ruba K Azzam
Cirrhosis is the end result of nearly all forms of progressive liver disease. The diffuse hepatic process can be characterized as a state of inflammation progressing to fibrosis and resulting in nodular regeneration, ultimately leading to disorganized liver architecture and function. The underlying etiology of cirrhosis in children may often differ from adults owing to specific disease processes that manifest in childhood, including biliary atresia, galactosemia, and neonatal hepatitis. Although basic management strategies in children are similar to those in adults, the care given to children with cirrhosis must keep the child's growth and development of paramount importance...
December 1, 2016: Pediatric Annals
https://www.readbyqxmd.com/read/27975109/cholestasis-in-infancy
#13
Melissa G Andrianov, Ruba K Azzam
Jaundice is a key manifestation of hepatobiliary disease in all age groups. Jaundice is a common finding in the first 2 weeks after birth, occurring in 2.4% to 15% of newborns. The neonatal liver is at increased susceptibility to cholestasis, with an incidence ranging from 1 in 2,500 to 1 in 5,000 live births. Etiologies vary, but the most common is biliary atresia. In 2004, the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition published guidelines for the evaluation of cholestasis that clearly stated any infant with jaundice persisting beyond age 2 weeks (3 weeks in breast-fed infants with an otherwise normal history and physical examination) should be evaluated with a fractionated serum bilirubin level...
December 1, 2016: Pediatric Annals
https://www.readbyqxmd.com/read/27957786/impaired-intention-to-treat-survival-after-listing-for-liver-transplantation-in-children-with-biliary-atresia-compared-to-other-chronic-liver-diseases-20%C3%A2-years-experience-from-the-nordic-countries
#14
S Malenicka, B-G Ericzon, M H Jørgensen, H Isoniemi, T H Karlsen, M Krantz, V Naeser, M Olausson, A Rasmussen, K Rönnholm, T Sanengen, T Scholz, B Fischler, A Nemeth
Biliary atresia (BA) is the most common indication for LT in children. We investigated whether this diagnosis per se, compared to other chronic liver diseases (OCLD), had an influence on patient survival. Data from 421 Scandinavian children, 194 with BA and 227 with OCLD, listed for LT between 1990 and 2010 were analyzed. The intention-to-treat survival and influencing risk factors were studied. Patients with BA had higher risk of death after listing than patients with OCLD. The youngest (<1 year) and smallest (<10 kg) children with the highest bilirubin (>510 μmol/L), highest INR (>1...
December 13, 2016: Pediatric Transplantation
https://www.readbyqxmd.com/read/27957246/elevation-of-serum-urokinase-plasminogen-activator-receptor-and-liver-stiffness-in-postoperative-biliary-atresia
#15
Wanvisa Udomsinprasert, Sittisak Honsawek, Napaphat Jirathanathornnukul, Voranush Chongsrisawat, Yong Poovorawan
AIM: To investigate serum urokinase-type plasminogen activator receptor (uPAR) and liver stiffness in biliary atresia (BA) and examine the correlation of circulating uPAR, liver stiffness, and clinical outcomes in postoperative BA children. METHODS: Eighty-five postKasai BA children and 24 control subjects were registered. Circulating uPAR was measured using enzyme-linked immunosorbent essay. Liver stiffness was analyzed using transient elastography. RESULTS: BA children had significantly greater circulating uPAR and liver stiffness scores than control subjects (P < 0...
November 28, 2016: World Journal of Hepatology
https://www.readbyqxmd.com/read/27932178/extra-anatomical-meso-portal-venous-jump-graft-repair-for-early-portal-vein-thrombosis-after-liver-transplant-in-an-infant-with-a-hypoplastic-portal-vein-a-case-report
#16
R Matsumoto, K Uchida, S Nishida, E R Island, D M Levi, J Fan, A Tekin, G Selvaggi, A G Tzakis
BACKGROUND AND PURPOSE: Small infants with biliary atresia and hypoplastic portal veins (PV) are at risk for portal vein thrombosis (PVT) after liver transplantation (LT), which can lead to graft loss and mortality. Extra-anatomical PV reconstruction techniques have been established for adult cases of PVT; however, they have not been widely accepted for infants. METHODS: Here, we report the successful use of an extra-anatomical meso-portal venous jump graft to treat early PVT after LT in a 6-month-old infant with biliary atresia and PV hypoplasia...
November 2016: Transplantation Proceedings
https://www.readbyqxmd.com/read/27932172/liver-transplantation-in-an-adult-recipient-with-situs-inversus-totalis-case-report-and-review-of-the-literature
#17
P Tabrizian, T T Joseph, P Radkani, E Cohen, M Facciuto
BACKGROUND: Over the past few decades, reports have demonstrated the feasibility of liver transplantation in adult patients with situs inversus. However, this disease entity remains rare and experience remains limited in adult recipients with situs inversus undergoing transplantation. METHODS: A 23-year-old woman with situs inversus totalis and end-stage liver disease secondary to congenital biliary atresia was referred to our center and underwent a successful orthotopic liver transplantation...
November 2016: Transplantation Proceedings
https://www.readbyqxmd.com/read/27914924/the-management-of-childhood-liver-diseases-in-adulthood
#18
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
#19
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
#20
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
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