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Biliary atresia

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https://www.readbyqxmd.com/read/29343773/mitochondrial-mutations-in-cholestatic-liver-disease-with-biliary-atresia
#1
Hong Koh, Gun-Seok Park, Sun-Mi Shin, Chang Eon Park, Seung Kim, Seok Joo Han, Huy Quang Pham, Jae-Ho Shin, Dong-Woo Lee
Biliary atresia (BA) results in severe bile blockage and is caused by the absence of extrahepatic ducts. Even after successful hepatic portoenterostomy, a considerable number of patients are likely to show progressive deterioration in liver function. Recent studies show that mutations in protein-coding mitochondrial DNA (mtDNA) genes and/or mitochondrial genes in nuclear DNA (nDNA) are associated with hepatocellular dysfunction. This observation led us to investigate whether hepatic dysfunctions in BA is genetically associated with mtDNA mutations...
January 17, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29330965/intra-hepatic-bile-duct-primary-cilia-in-biliary-atresia
#2
Roberta Frassetto, Filippo Parolini, Salvatore Marceddu, Giulia Satta, Valeria Papacciuoli, Maria Antonia Pinna, Alessandra Mela, Giannina Secchi, Grazia Galleri, Roberto Manetti, Luisa Bercich, Vincenzo Villanacci, Antonio Dessanti, Roberto Antonucci, Francesco Tanda, Daniele Alberti, Kathleen B Schwarz, Maria Grazia Clemente
AIM: The etiopathogenesis of non-syndromic biliary atresia (BA) is obscure. The primary aim was to investigate intra-hepatic bile duct cilia (IHBC) in BA at the diagnosis and correlation with clinical outcome. The secondary aim was to analyze IHBC in routine paraffin-embedded liver biopsies using conventional scanning electron microscopy (SEM). METHODS: Surgical liver biopsies taken at the diagnosis from 22 BA infants (age range: 39 - 116 days) and from 8 children with non-BA chronic cholestasis (age range: 162 days -16,8 years) were evaluated for IHBC, both by immunofluorescence (IF) and by SEM...
January 13, 2018: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/29318378/mac-2-binding-protein-glycan-isomer-m2bpgi-is-a-new-serum-biomarker-for-assessing-liver-fibrosis-more-than-a-biomarker-of-liver-fibrosis
#3
REVIEW
Ken Shirabe, Yuki Bekki, Dolgormaa Gantumur, Kenichiro Araki, Norihiro Ishii, Atsushi Kuno, Hisashi Narimatsu, Masashi Mizokami
Assessing liver fibrosis is important for predicting the efficacy of antiviral therapy and patient prognosis. Liver biopsy is the gold standard for diagnosing liver fibrosis, despite its invasiveness and problematic diagnostic accuracy. Although noninvasive techniques to assess liver fibrosis are becoming important, reliable serum surrogate markers are not available. A glycoproteomics study aimed at identifying such markers discovered Mac 2-Binding Protein Gylcan Isomer (M2BPGi), which is a reliable marker for assessing liver fibrosis in patients with viral hepatitis and other fibrotic liver diseases such as primary biliary cholangitis, biliary atresia, autoimmune hepatitis, and nonalcoholic fatty liver disease...
January 9, 2018: Journal of Gastroenterology
https://www.readbyqxmd.com/read/29316063/bilirubin-reversibly-affects-cell-death-and-odontogenic-capacity-in-shed
#4
Haruyoshi Yamaza, Erika Tomoda, Soichiro Sonoda, Kazuaki Nonaka, Toshio Kukita, Takayoshi Yamaza
OBJECTIVE: Hyperbilirubinemia in patients with biliary atresia causes deciduous tooth injuries such as green pigmentation and dentin hypoplasia. In patients with biliary atresia who received liver transplantation, tooth structure appears to be recovered radiographically. Nevertheless, little is known about cellular mechanisms underlying bilirubin-induced damage and suppression of deciduous tooth formation. In this study, we examined the effects of bilirubin in stem cells from human exfoliated deciduous teeth (SHED) in vitro...
January 9, 2018: Oral Diseases
https://www.readbyqxmd.com/read/29315162/factors-influencing-time-to-diagnosis-of-biliary-atresia
#5
Sanjiv Harpavat, Philip J Lupo, Loriel Liwanag, John Hollier, Mary L Brandt, Milton J Finegold, Benjamin L Shneider
OBJECTIVES: Diagnosing biliary atresia (BA) quickly is critical, because earlier treatment correlates with delayed or reduced need for liver transplantation. However, diagnosing BA quickly is also difficult, with infants usually treated after 60 days of life. In this study, we aim to accelerate BA diagnosis and treatment, by better understanding factors influencing the diagnostic timeline. METHODS: Infants born between 2007-2014 and diagnosed with BA at our institution were included (n = 65)...
January 6, 2018: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29308768/factors-influencing-outcome-after-hepatic-portoenterostomy-among-extrahepatic-bile-duct-atresia-patients-in-hospital-sultanah-bahiyah-alor-setar
#6
D Namasemayam, M Nallusamy
INTRODUCTION: Biliary atresia implies high risk of mortality if not diagnosed and treated early. We undertook this study to assess the prognostic factors affecting the outcome of Kasai surgery. METHODS: A retrospective clinical study was conducted among 58 patients from Hospital Sultanah Bahiyah Alor Setar. Data were analysed with logistic regression analysis using SPSS. Factors studied included age during surgery, bile duct diameter at the porta hepatic, race of patient, preoperative total bilirubin level and cholangitis...
December 2017: Medical Journal of Malaysia
https://www.readbyqxmd.com/read/29303884/the-first-episode-of-spontaneous-bacterial-peritonitis-is-a-threat-event-in-children-with-end-stage-liver-disease
#7
Sandra M G Vieira, Fernando P Schwengber, Melina Melere, Marília R Ceza, Melina Souza, Carlos O Kieling
OBJECTIVE: Studies on native liver survival (NLS) after the first episode of spontaneous bacterial peritonitis (SBP) are rare. Our objective was to evaluate NLS in children up to 1 year after SBP. METHODS: A historical cohort study of 18 children followed after the first episode of SBP was conducted. NLS, in-hospital mortality, causes of death, and rate of multidrug-resistant organisms were reported. RESULTS: Biliary atresia was the most prevalent diagnosis (72...
January 4, 2018: European Journal of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/29287007/bone-mineral-density-of-very-long-term-survivors-after-childhood-liver-transplantation
#8
Looi C Ee, Charlton Noble, Jonathan Fawcett, Geoffrey J Cleghorn
OBJECTIVES: Liver transplant patients are at risk of osteopenia and fractures but limited information is available in long-term survivors after childhood transplantation. This study aimed to assess bone mineral density (BMD) of very long-term, >5 years, survivors after liver transplantation in childhood. METHODS: Patients aged <18 years at transplant, having survived >5 years after transplant were potentially eligible but only those with ongoing review in our state were included...
December 28, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29251369/association-of-polymorphism-in-the-vegfa-gene-3-utr-936t-c-with-susceptibility-to-biliary-atresia-in-a-southern-chinese-han-population
#9
Fei Liu, Jixiao Zeng, Deli Zhu, Ruizhong Zhang, Xiaogang Xu, Mengmeng Wang, Yan Zhang, Huimin Xia, Zhichun Feng
BACKGROUND: Biliary atresia (BA) is a neonatal disease characterized by chronic inflammation of the bile ducts and progressive aggravation of jaundice, but with a poor prognosis and high mortality. The etiology of BA is still uncertain which may be related to gene defect, virus infection, immune disorder, gene polymorphism. As a proinflammatory cytokine, VEGFA gene polymorphism (rs3025039) has been shown to be related to the pathogenesis of BA in Taiwanese population. METHODS: We investigated the association between VEGFA gene polymorphism (rs3025039) and BA susceptibility using the largest case-control cohort, totaling with 506 BA patients and 1473 healthy controls in a Southern Chinese Han population...
December 18, 2017: Journal of Clinical Laboratory Analysis
https://www.readbyqxmd.com/read/29240507/neonatal-jaundice-aetiology-diagnosis-and-treatment
#10
Subhabrata Mitra, Janet Rennie
A significant proportion of term and preterm infants develop neonatal jaundice. Jaundice in an otherwise healthy term infant is the most common reason for readmission to hospital. Jaundice is caused by an increase in serum bilirubin levels, largely as a result of breakdown of red blood cells. Bilirubin is conveyed in the blood as 'unconjugated' bilirubin, largely bound to albumin. The liver converts bilirubin into a conjugated form which is excreted in the bile. Very high levels of unconjugated bilirubin are neurotoxic...
December 2, 2017: British Journal of Hospital Medicine
https://www.readbyqxmd.com/read/29240010/well-preserved-renal-function-in-children-with-untreated-chronic-liver-disease
#11
Ulla B Berg, Antal Németh
OBJECTIVES: Based on studies with hepatorenal syndrome, it is widely regarded that renal function is impacted in chronic liver disease. Therefore we investigated renal function in our children with chronic liver disease. METHODS: In a retrospective study of 277 children with chronic liver disease, renal function was investigated as glomerular filtration rate (GFR) and effective renal plasma flow (ERPF), measured as clearance of inulin and para-amino hippuric acid or clearance of iohexol...
December 12, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29235550/biliary-tract-mmp7-a-diagnostic-biomarker-for-biliary-atresia
#12
Hugh Thomas
No abstract text is available yet for this article.
December 13, 2017: Nature Reviews. Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/29232532/reconsideration-of-laparoscopic-kasai-operation-for-biliary-atresia
#13
Sheng-Yang Huang, Chou-Ming Yeh, Hou-Chuan Chen, Chia-Man Chou
INTRODUCTION: Minimal invasive surgery for all kinds of surgical diseases had been practiced for years. The laparoscopic Kasai operation is one of the most challenging procedures and remains controversial for treating biliary atresia (BA). PURPOSE: This work presented the initial experience of our Institute and compared the outcomes of open and laparoscopic Kasai operations for BA. MATERIALS AND METHODS: Patients 18 years old and younger, and were operated in our Institute for BA from January 2011 to August 2017, were included in this study...
December 12, 2017: Journal of Laparoendoscopic & Advanced Surgical Techniques. Part A
https://www.readbyqxmd.com/read/29229480/liver-transplantation-for-deterioration-in-native-liver-function-after-portoenterostomy-for-biliary-atresia-in-japan-short-versus-long-term-survivors
#14
Takanori Ochi, Hiroki Nakamura, Momoko Wada, Tsuyoshi Tamura, Hiroyuki Koga, Tadaharu Okazaki, Masahiko Urao, Yoichi Ishizaki, Seiji Kawasaki, Mureo Kasahara, Koichi Mizuta, Geoffrey J Lane, Atsuyuki Yamataka
PURPOSE: We reviewed our post-Kasai portoenterostomy biliary atresia (BA) patients who required liver transplantation (LTx) for deterioration in native liver (NL) function to investigate mortality in relation to age at LTx. METHODS: BA patients indicated for LTx when less than 18years old (U18; n=17) and when 18 or older (18+; n=13) were compared. All achieved jaundice clearance postoperatively (TBil ≤1.2mg/dL (≈20μmol/L)). RESULTS: In U18, living-donor (LD) LTxs were performed at a median of 6...
November 13, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29221637/effect-of-previous-abdominal-surgery-and-gallbladder-appearance-on-biliary-atresia-outcomes
#15
Ayman Goneidy, Evelyn Geok Peng Ong
BACKGROUND: Biliary atresia (BA) is typically treated by Kasai portoenterostomy (KPE), and there is a relationship between age at surgery and outcome. We hypothesize that previous abdominal surgery (PAS) for associated congenital intestinal conditions could be used to identify BA earlier, perhaps improving prognosis. METHODS: A retrospective case note review was performed of all BA patients at a single centre from 1999 to 2016. Demographics and clinical outcome data were collected...
December 5, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29216021/long-term-results-and-quality-of-life-assessment-in-biliary-atresia-patients-a-35-year-experience-in-a-tertiary-hospital
#16
Carol Wing Yan Wong, Patrick Ho Yu Chung, Paul Kwong Hang Tam, Kenneth Kak Yuen Wong
OBJECTIVES: To review long-term transplant-free survival and quality of life of patients with biliary atresia. METHODS: A retrospective study reviewing all patients with Kasai operation between 1 January 1980 and 31 December 2015 was performed to evaluate the transplant-free survival. Subgroup analysis of patients over 20 years old was carried out to assess the quality of life using the Short Form-36 Health Survey and incidences of disease-related complications...
December 5, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29209118/chronic-liver-disease-is-universal-in-children-with-biliary-atresia-living-with-native-liver
#17
Way Seah Lee, Sik Yong Ong, Hee Wei Foo, Shin Yee Wong, Chen Xi Kong, Ru Bin Seah, Ruey Terng Ng
AIM: To examine the medical status of children with biliary atresia (BA) surviving with native livers. METHODS: In this cross-sectional review, data collected included complications of chronic liver disease (CLD) (cholangitis in the preceding 12 mo, portal hypertension, variceal bleeding, fractures, hepatopulmonary syndrome, portopulmonary hypertension) and laboratory indices (white cell and platelet counts, total bilirubin, albumin, international normalized ratio, alanine aminotransferase, aspartate aminotransferase, γ-glutamyl transpeptidase)...
November 21, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/29205755/short-term-outcomes-of-paediatric-liver-transplant-recipients-after-transition-to-adult-healthcare-service
#18
Alberto Ferrarese, Giacomo Germani, Silvia Lazzaro, Mara Cananzi, Francesco Paolo Russo, Marco Senzolo, Martina Gambato, Alberto Zanetto, Umberto Cillo, Enrico Gringeri, Giorgio Perilongo, Patrizia Burra
BACKGROUND & AIMS: Survival rates after liver transplantation (LT) in paediatric recipients have significantly improved over time. However, data regarding outcomes after transition from Paediatric to Adult Healthcare Service (AHS) are still lacking. Therefore, we aimed to prospectively evaluate the outcome of LT recipients after transition, to access patients' non-adherence and identify potential risk factors for non-adherence. METHODS: All consecutive adolescent LT recipients moving to the AHS at Padua University Hospital were evaluated between 2010 and 2015...
December 5, 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/29196972/proximal-total-splenic-artery-embolization-for-refractory-hepatic-encephalopathy
#19
Harufumi Maki, Junichi Kaneko, Junichi Arita, Nobuhisa Akamatsu, Yoshihiro Sakamoto, Kiyoshi Hasegawa, Sumihito Tamura, Hidemasa Takao, Eisuke Shibata, Norihiro Kokudo
A Japanese woman with a history of Kasai operation for biliary atresia had living-donor liver transplantation at the age of 22. The first episode of refractory HE and late cellular rejection was treated by a high dose of methylprednisolone. The second episode of refractory HE was treated by balloon-occluded retrograde transvenous obliteration for a spleno-renal shunt. However, the third episode of refractory HE occurred 11 years after liver transplantation. The liver cirrhosis and hypersplenism were present with a Child-Pugh score of C-10...
December 1, 2017: Clinical Journal of Gastroenterology
https://www.readbyqxmd.com/read/29183423/outcomes-of-kasai-hepatoportoenterostomy-in-children-with-biliary-atresia-in-johannesburg-south-africa
#20
T De Maayer, S G Lala, J Loveland, G Okudo, R Mohanlal, C Hajinicolaou
BACKGROUND: Without timely surgical intervention, most children with biliary atresia (BA) are not expected to live beyond 2 years of age. The initial intervention, the Kasai hepatoportoenterostomy (KPE), aims to achieve biliary drainage. Liver transplantation (LT) is performed if jaundice fails to clear or when biliary cirrhosis occurs. In under-resourced South African (SA) academic state hospitals, KPE procedures are the standard of care for the majority of children with BA, but LT is becoming more routinely available...
November 6, 2017: South African Medical Journal, Suid-Afrikaanse Tydskrif Vir Geneeskunde
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