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Biliary atresia

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https://www.readbyqxmd.com/read/29160012/near-infrared-spectroscopy-might-be-a-useful-tool-for-predicting-the-risk-of-vascular-complications-after-pediatric-liver-transplants-two-case-reports
#1
Juntaro Shiba, Masaaki Satoh, Koki Taira, Yasunori Niwa, Soichiro Inoue, Koichi Mizuta, Mamoru Takeuchi
In patients that have undergone liver transplants, a postoperative reduction in the blood flow of the liver graft represents a critical complication. We recently encountered an interesting phenomenon; that is, we found that the rSO2 level of the liver graft, as measured by NIRS, drops in patients that subsequently require an emergency liver biopsy. An 8-month-old female and an 8-month-old male underwent living donor liver transplants for biliary atresia. In both cases, a reduction in rSO2 was detected before an emergency liver biopsy was required...
November 21, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/29152924/population-based-birth-defects-data-in-the-united-states-2010-2014-a-focus-on-gastrointestinal-defects
#2
Philip J Lupo, Jennifer L Isenburg, Jason L Salemi, Cara T Mai, Rebecca F Liberman, Mark A Canfield, Glenn Copeland, Sarah Haight, Sanjiv Harpavat, Adrienne T Hoyt, Cynthia A Moore, Wendy N Nembhard, Hoang N Nguyen, Rachel E Rutkowski, Amy Steele, C J Alverson, Erin B Stallings, Russell S Kirby
BACKGROUND: Gastrointestinal defects are a phenotypically and etiologically diverse group of malformations. Despite their combined prevalence and clinical impact, little is known about the epidemiology of these birth defects. Therefore, the objective of the 2017 National Birth Defects Prevention Network (NBDPN) data brief was to better describe the occurrence of gastrointestinal defects. METHODS: As part of the 2017 NBDPN annual report, 28 state programs provided additional data on gastrointestinal defects for the period 2010-2014...
November 1, 2017: Birth defects research
https://www.readbyqxmd.com/read/29146975/disturbance-in-plasma-metabolic-profile-in-different-types-of-human-cytomegalovirus-induced-liver-injury-in-infants
#3
Wei-Wei Li, Jin-Jun Shan, Li-Li Lin, Tong Xie, Li-Li He, Yan Yang, Shou-Chuan Wang
Human cytomegalovirus (HCMV) infection in infants is a global problem and the liver is a target organ of HCMV invasion. However, the mechanism by which HCMV causes different types of liver injury is unclear, and there are many difficulties in the differential diagnosis of HCMV infantile cholestatic hepatopathy (ICH) and extrahepatic biliary atresia (EHBA). We established a non-targeted gas chromatography-mass spectrometry metabolomics method in conjunction with orthogonal partial least squares-discriminate analysis based on 127 plasma samples from healthy controls, and patients with HCMV infantile hepatitis, HCMV ICH, and HCMV EHBA to explore the metabolite profile of different types of HCMV-induced liver injury...
November 16, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29127962/minimally-invasive-hepatobiliary-surgery
#4
REVIEW
Omid Madadi-Sanjani, Claus Petersen, Benno Ure
Comparative studies and large-scale case series that confirm the advantages of laparoscopy in children with hepatobiliary diseases are scarce, and the use of laparoscopy remains a matter of debate. This article reviews the current literature on the role of laparoscopic and robotic surgery in pediatric patients with choledochal cyst, biliary atresia, gallbladder diseases, and hepatobiliary malignancies. Studies were identified through a search of the MEDLINE database. Laparoscopy may be beneficial for resection of choledochal cyst and cholecystectomy...
December 2017: Clinics in Perinatology
https://www.readbyqxmd.com/read/29118687/renoportal-anastomosis-in-left-lateral-lobe-living-donor-liver-transplantation-a-pediatric-case
#5
Hiroyuki Ogasawara, Chikashi Nakanishi, Shigehito Miyagi, Kazuaki Tokodai, Yasuyuki Hara, Wataru Nakanishi, Koji Miyazawa, Kenji Shimizu, Hiroyuki Kumata, Hitoshi Goto, Masafumi Goto, Michiaki Unno, Takashi Kamei
In adult liver transplantation, renoportal anastomosis (RPA) has been introduced as a useful technique for patients with grade 4 portal vein thrombosis and a splenorenal shunt. Here, we report a pediatric case in which RPA allowed a left lateral lobe living donor liver transplantation (LDLT) despite portal vein thrombosis and a large splenorenal shunt. At 36 days old, the patient underwent a Kasai operation for biliary atresia. At 17 months old, she underwent LDLT because of repetitive cholangitis. Pretransplant examinations revealed a large splenorenal shunt and portal vein thrombosis...
September 2017: Case Reports in Gastroenterology
https://www.readbyqxmd.com/read/29113911/access-to-care-in-rare-liver-diseases-new-challenges-and-new-opportunities
#6
REVIEW
David Jones, Ekkehard Sturm, Ansgar W Lohse
Patients with rare diseases are often disadvantaged, and this applies to rare liver disease in particular. Reasons for disadvantage include delayed or overlooked diagnosis, lack of local expertise and high quality care, poor scientific understanding of the disease process and limited therapeutic options. In adult liver disease in particular this can be compounded by prejudices towards patients with liver disease in general because of a perception (incorrect for all rare liver disease), that liver disease is lifestyle related and thus "self-inflicted"...
November 4, 2017: Journal of Hepatology
https://www.readbyqxmd.com/read/29110825/long-term-outlook-in-biliary-atresia
#7
Arun Kelay, Mark Davenport
The oldest survivors from the Kasai portoenterostomy originate in Sendai, Japan and are approaching their 60th birthday. These represent the tip of an expanding cohort of adults born with this previously fatal condition. Increasingly transition to adult-biased hepatologists and physicians will be the expectation of many with this condition. However unlike their usual patients with alcohol, drugs, virally mediated liver disease these are different with different expectations of health and quality of life. Cure is not on the cards for most of these and they survive still with impaired bile flow and increased liver fibrosis and cirrhosis with the threat of cholangitis and portal hypertension still apparent...
October 2017: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/29105184/differentiation-between-cystic-biliary-atresia-and-choledochal-cyst-a-retrospective-analysis
#8
Jue Tang, Dan Zhang, Wei Liu, Ji-Xiao Zeng, Jia-Kang Yu, Yi Gao
AIM: Cystic biliary atresia (CBA) can be easily misdiagnosed as choledochal cyst (CC). Some patients have already progressed to severe liver fibrosis and missed the optimal surgical time, when the differential diagnosis is made. We aim to determine the differentiation between CBA and CC, and to validate the value of aspartate aminotransferase-to-platelet ratio index (APRI) in the assessment of liver fibrosis and prediction of post-operative outcome for infants with biliary cystic malformations (BCMs)...
November 3, 2017: Journal of Paediatrics and Child Health
https://www.readbyqxmd.com/read/29097260/advances-in-the-generation-of-bioengineered-bile-ducts
#9
REVIEW
Alexander W Justin, Kourosh Saeb-Parsy, Athina E Markaki, Ludovic Vallier, Fotios Sampaziotis
The generation of bioengineered biliary tissue could contribute to the management of some of the most impactful cholangiopathies associated with liver transplantation, such as biliary atresia or ischemic cholangiopathy. Recent advances in tissue engineering and in vitro cholangiocyte culture have made the achievement of this goal possible. Here we provide an overview of these developments and review the progress towards the generation and transplantation of bioengineered bile ducts.
October 30, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/29093620/laparoscopic-finding-of-a-hepatic-subcapsular-spider-like-telangiectasis-sign-in-biliary-atresia
#10
Ying Zhou, Meng Jiang, Shao-Tao Tang, Li Yang, Xi Zhang, De-Hua Yang, Meng Xiong, Shuai Li, Guo-Qing Cao, Yong Wang
AIM: To assess the diagnostic value of a laparoscopic finding of a hepatic subcapsular spider-like telangiectasis (HSST) sign in biliary atresia. METHODS: A retrospective study was conducted first and then a validation set was used to investigate the value of an HSST sign in predicting biliary atresia (BA). In the retrospective study, laparoscopic images of the liver surface were reviewed in 126 patients with infantile cholestasis (72 BA patients and 54 non-BA cholestasis patients) and a control group of 38 patients with non-hepatic conditions...
October 21, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/29070776/ccl5-is-induced-by-tlr-3-signaling-in-hucct1-human-biliary-epithelial-cells-possible-involvement-in-the-pathogenesis-of-biliary-atresia
#11
Taku Shimada, Tadaatsu Imaizumi, Kyogo Shirai, Tetsuya Tatsuta, Toshiro Kimura, Ryo Hayakari, Hidemi Yoshida, Tomoh Matsumiya, Hiroshi Kijima, Hiroki Mizukami, Kenichi Hakamada
Biliary atresia (BA) is a disease of the newborn that is characterized by progressive, inflammatory and sclerosing cholangiopathy. Innate immune responses to viral components are thought to be involved in the pathogenesis of BA. It is also reported that some chemokines, such as CCL5, are possibly involved in the pathogenesis of experimental animal model of BA. We treated human biliary epithelial HuCCT1 cells with polyinosinic-polycytidylic acid (poly IC), an authentic double-stranded RNA (dsRNA) which mimics viral RNA, and analyzed the CCL5 expression by quantitative reverse transcription-PCR (qRT-PCR) and enzyme-linked immunosorbent assay (ELISA)...
2017: Biomedical Research
https://www.readbyqxmd.com/read/29067750/quality-of-life-of-biliary-atresia-after-living-donor-liver-transplant-in-japan
#12
Ryota Kikuchi, Koichi Mizuta, Taizen Urahashi, Yukihiro Sanada, Naoya Yamada, Erika Onuma, Iori Sato, Kiyoko Kamibeppu
BACKGROUND: Health-related quality of life (HRQOL) is an important outcome in solid organ transplantation. This study evaluated and explored the factors of generic and transplant-specific HRQOL in Japanese pediatric and adolescent patients with biliary atresia (BA) after living donor liver transplant (LDLT). METHODS: A cross-sectional survey using anonymous questionnaires was completed between April and July 2015. Patients' medical records were accessed. The Japanese version of Pediatric Quality of Life Inventory(™) Generic Core Scales and Transplant Modules (child self-report and parent proxy-report) was administered...
October 25, 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/29063959/update-on-investigations-pertaining-to-the-pathogenesis-of-biliary-atresia
#13
REVIEW
Alexandra Kilgore, Cara L Mack
Biliary atresia is a devastating biliary disease of neonates that results in liver transplantation for the vast majority. The etiology of biliary atresia is unknown and is likely multifactorial, with components of genetic predisposition, environmental trigger and autoimmunity contributing to disease pathogenesis. This review highlights recent work related to investigations of disease pathogenesis in biliary atresia.
December 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/29063249/early-us-findings-of-biliary-atresia-in-infants-younger-than-30-days
#14
Sook Min Hwang, Tae Yeon Jeon, So-Young Yoo, Yon Ho Choe, Suk-Koo Lee, Ji Hye Kim
PURPOSE: To investigate and compare ultrasound (US) findings for the diagnosis of biliary atresia (BA) in infants younger than 30 days with those of infants older than 30 days. MATERIALS AND METHODS: From 2000 to 2015, we reviewed hepatobiliary US images in 12 BA infants younger than 30 days (younger BA group) and 62 BA infants older than 30 days (older BA group) before Kasai procedure. Eight (67%) of younger BA group underwent follow-up US examinations before Kasai procedure...
October 23, 2017: European Radiology
https://www.readbyqxmd.com/read/29056230/correlation-between-gamma-glutamyl-transpeptidase-activity-and-outcomes-after-kasai-portoenterostomy-for-biliary-atresia
#15
Kyong Ihn, In Geol Ho, Eun Young Chang, Seok Joo Han
BACKGROUND/PURPOSE: The role of serum gamma-glutamyl transpeptidase (GGT) levels in predicting clinical outcomes after Kasai portoenterostomy (KPE) is unknown. This study analyzed whether postoperative GGT along with the aspartate aminotransferase-to-platelet ratio index (APRi) predicted prognosis of biliary atresia (BA). METHODS: Data were retrospectively reviewed for 169 BA patients categorized into jaundice-free (JF) (total bilirubin <2.0 mg/dL ≤6 months post-KPE) and persistent jaundice (PJ) groups (total bilirubin ≥2...
October 6, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29046057/bile-duct-ligature-in-young-rats-a-revisited-animal-model-for-biliary-atresia
#16
Matias Garrido, Camila Escobar, Constanza Zamora, Carolina Rejas, Juan Varas, Mario Párraga, Sebastian San Martin, Sandra Montedónico
Biliary atresia leads to cirrhosis in the vast majority of patients and constitutes the first cause of paediatric liver transplantation. Animal models allow us to understand the molecular basis and natural history of diseases. The aim of this study is to describe a surgically created animal model of biliary atresia with emphasis in long-term liver function. Forty-two 3-week-old Sprague-Dawley rats were randomly divided into two groups: bile duct ligature (BDL) and control. The animals were sacrificed on the 2nd, 4th, and 6th postoperative weeks...
September 13, 2017: European Journal of Histochemistry: EJH
https://www.readbyqxmd.com/read/29039049/japanese-biliary-atresia-registry
#17
Masaki Nio
The Japanese Biliary Atresia Registry (JBAR) was launched in 1989 by the Japanese Biliary Atresia Society (JBAS). JBAR employed an initial questionnaire, a questionnaire for liver transplantation, and a follow-up questionnaire. The questionnaire has been sent to the pediatric surgeons of JBAS member institutions and the hospitals in which council members of the Japanese Society of Pediatric Surgeons are working. One hundred and twenty-three hospitals have been included in JBAR between 1989 and 2015. Each patient is to be followed up for 30 years...
October 16, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/29039048/sclerosing-and-obstructive-cholangiopathy-in-biliary-atresia-mechanisms-and-association-with-biliary-innate-immunity
#18
REVIEW
Kenichi Harada
Biliary atresia (BA) is histologically characterized by a progressive, sclerosing cholangitis and the obstruction of extrahepatic bile ducts. In terms of the etiology and pathogenesis of BA, several viral infections consisting of dsRNA, including Reoviridae, have been implicated. Human biliary epithelial cells (BECs) possess an innate immune system consisting of Toll-like receptors (TLRs). BECs have negative regulatory mechanisms of TLR tolerance to avoid an excessive inflammatory response to lipopolysaccharide (LPS), a TLR4 ligand; however, they lack the tolerance to poly(I:C) (a synthetic analog of viral dsRNA), a TLR3 ligand...
December 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/29036008/ethnic-disparity-in-the-incidence-and-outcome-of-biliary-atresia-in-new-zealand
#19
Helen M Evans, M Innes Asher, Sophia Cameron-Christie, Sonja Farthing, John McCall, Stephen P Robertson, Hayley Wong, Philip N Morreau
In order to determine incidence and outcome of biliary atresia (BA) between ethnic groups in New Zealand (NZ), a retrospective review was undertaken of children with BA born 2002-2014. Prioritised ethnicity was used to determine ethnicity and was compared to population data. Uni- and multivariate analyses were undertaken to determine demographic and biochemical factors associated with outcome. Overall incidence was 1 in 9,181 (Māori 1 in 5,285; European 1 in 16,228; p < 0.0001). Overall and transplant-free survival rates at 1, 2 and 5 years were 92%, 86%, 82% and 70%, 49%, 30% respectively with Māori having improved transplant-free survival (p < 0...
October 13, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29034890/derivation-of-a-disease-specific-human-induced-pluripotent-stem-cell-line-from-a-biliary-atresia-patient
#20
Lipeng Tian, Lindsey Eldridge, Pooja Chaudhari, Linyi Zhang, Robert A Anders, Kathleen B Schwarz, Zhaohui Ye, Yoon-Young Jang
Biliary atresia (BA) is a common cause of pediatric end-stage liver disease. While its etiology is not yet clear, evidence has suggested that BA results from interactions between genetic susceptibility and environmental factors. Disease relevant human cellular models of BA will facilitate identification of both genetic and environmental factors that are important for disease prevention and treatment. Here we report the generation of a human induced pluripotent stem cell line from a BA patient using episomal vectors...
October 2017: Stem Cell Research
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