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Biliary atresia

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https://www.readbyqxmd.com/read/28203327/a-rapid-screening-test-on-dried-blood-for-the-neonatal-diagnosis-of-tyrosinemia-type-i
#1
Farahnaz Bodaghkhan, Bita Geramizadeh, Abbas Abdollah Rajeh, Mahmoud Haghighat, Mohsen Dehghani, Naser Honar, Mojgan Zahmatkeshan, Mohammad-Hadi Imanieh
BACKGROUND: Tyrosinemia is an inherited metabolic disorder characterized by elevated levels of tyrosine and its metabolites in plasma. Without treatment, the disease will progress to hepatic and renal failure, so that without liver transplantation will cause death in less than 10 years of age. So, early diagnosis and treatment can be life saving and crucial. It means that with early treatment starting in the neonatal period, the patient can have normal life with very few restrictions in diets containing tyrosine and phenylalanine...
October 2016: Iranian Journal of Pediatrics
https://www.readbyqxmd.com/read/28180936/fn14-hepatic-progenitor-cells-are-associated-with-liver-fibrosis-in-biliary-atresia
#2
Lulu Zheng, Zhibao Lv, Zhenhua Gong, Qingfeng Sheng, Zhimei Gao, Yuting Zhang, Shenghua Yu, Junmei Zhou, Zhengjun Xi, Xueli Wang
PURPOSE: The liver in biliary atresia (BA) is characterized by progressing fibrosis which is promoted by unclear reasons. We aimed to understand the factors influencing liver fibrosis. This study hypothesized that HPCs (hepatic progenitor cells) are activated and associated with liver fibrosis in biliary atresia. METHODS: Liver samples from biliary atresia patients are as BA group, and the normal liver derived from hepatoblastoma infants during operation are control group...
February 8, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28159941/outcome-of-biliary-atresia-after-kasai-s-portoenterostomy-a-15-year-experience
#3
Rajeev Redkar, Parag J Karkera, Vinod Raj, Anant Bangar, Varun Hathiramani, Janani Krishnan
OBJECTIVE: To study the outcome of Biliary atresia after Kasai's portoenterostomy and clinical and biochemical factors affecting the outcome. METHODS: This medical record review of patients of biliary atresia operated from January 2000 to December 2014. The following data were collected and analyzed - sex, age at surgery, liver function tests, associated congenital anomalies, and clearance of jaundice (at 3 months). Final outcome was classified as alive, dead, or jaundice-free at last follow-up (minimum 1 year)...
February 2, 2017: Indian Pediatrics
https://www.readbyqxmd.com/read/28149148/pediatric-liver-transplantation-our-experiences
#4
Ahmet Basturk, Aygen Yılmaz, Ersin Sayar, Ayhan Dinçhan, İbrahim Aliosmanoğlu, Halil Erbiş, Bülent Aydınlı, Reha Artan
OBJECTIVE: The aim of our study was to evaluate our liver transplant pediatric patients and to report our experience in the complications and the long-term follow-up results. MATERIALS AND METHODS: Patients between the ages of 0 and 18 years, who had liver transplantation in the organ transplantation center of our university hospital between 1997 and 2016, were included in the study. The age, sex, indications for the liver transplantation, complications after the transplantation, and long-term follow-up findings were retrospectively evaluated...
October 2016: Eurasian Journal of Medicine
https://www.readbyqxmd.com/read/28125097/utility-of-measuring-direct-bilirubin-at-first-72%C3%A2-h-of-age-in-neonates-admitted-to-the-neonatal-intensive-care-unit
#5
A J Sloane, U S Nawab, D Carola, Z H Aghai
OBJECTIVE: To assess the utility of measuring direct bilirubin (DB) during the first 72 h of life in infants admitted to the neonatal intensive care unit (NICU). STUDY DESIGN: Infants born between May 2006 and June 2013, and admitted to the NICU were included. Abnormal DB was defined as: DB level⩾1 mg dl(-1) with a corresponding TB of ⩽5 mg dl(-1) or DB level >20% of the corresponding TB>5 mg dl(-1). RESULTS: The DB levels were measured in 3715 infants during the first 72 h of life...
January 26, 2017: Journal of Perinatology: Official Journal of the California Perinatal Association
https://www.readbyqxmd.com/read/28107282/early-posthepatoportoenterostomy-predictors-of-native-liver-survival-in-biliary-atresia
#6
Scott Nightingale, Michael O Stormon, Edward V O'Loughlin, Albert Shun, Gordon Thomas, Eric I Benchimol, Andrew S Day, Susan Adams, Edward Shi, Chee Y Ooi, Binita M Kamath, Annie Fecteau, Jacob C Langer, Eve A Roberts, Simon C Ling, Vicky L Ng
OBJECTIVES: Most infants with biliary atresia (BA) require liver transplantation (LT) after hepatoportoenterostomy (HPE), including those who initially clear jaundice. The aim of the present study was to identify clinical and routine laboratory factors in infants with BA post-HPE that predict native liver survival at 2 years. METHODS: A retrospective cohort study was conducted in 217 patients with BA undergoing HPE in Sydney, Australia and Toronto, Canada between January 1986 and July 2009...
February 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28104292/hepatic-prominin-1-expression-is-associated-with-biliary-fibrosis
#7
Marie V Nguyen, Jessica A Zagory, William H Dietz, Alex Park, Michael Fenlon, Menghan Zhao, Jiabo Xu, Ingrid Lua, Nirmala Mavila, Kinji Asahina, Kasper S Wang
BACKGROUND: Intrahepatic biliary fibrosis, as seen with cholestatic liver injuries such as biliary atresia, is mechanistically distinct from fibrosis caused by hepatocyte toxicity. We previously demonstrated the expansion of cells expressing the stem/progenitor cell marker Prominin-1, within regions of developing fibrosis in biliary atresia. Thus, we hypothesized that Prominin-1 expression is biliary fibrosis-specific. METHODS: Gene expression of Prominin-1 was analyzed in adult mice undergoing either cholestatic bile duct ligation or hepatotoxic carbon tetrachloride administration by quantitative polymerase chair reaction...
January 16, 2017: Surgery
https://www.readbyqxmd.com/read/28100381/-trend-of-mortality-of-congenital-malformation-in-children-aged-5-years-in-beijing-2006-2015
#8
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
#9
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
#10
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...
March 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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
REVIEW
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...
February 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27992499/diffusion-tensor-imaging-for-evaluating-biliary-atresia-in-infants-and-neonates
#17
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
#18
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
#19
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
#20
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
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