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Biliary atresia

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https://www.readbyqxmd.com/read/28332100/expression-of-programmed-death-1-and-its-ligands-in-the-liver-of-biliary-atresia
#1
Pan-Liang Wang, Jun Wang, Ying Zhou, Xiao-Song Chen, Ke-Jun Zhou, Jie Wen, Jian-Jun Zhang, Wei Cai
BACKGROUND: An aberrant immune response is the predominant pathogenetic factor in biliary atresia (BA). Programmed death-1 (PD-1) and its two ligands, programmed death ligand-1 and programmed death ligand-2 (PD-L1 and PD-L2, respectively) play an important inhibitory role in immune reactions. We aimed to illustrate the expression of these molecules in BA. METHODS: Liver specimens were obtained from infants with BA during the Kasai procedure (early BA) and liver transplantation (late BA)...
March 22, 2017: World Journal of Pediatrics: WJP
https://www.readbyqxmd.com/read/28325677/il-6-tnf-%C3%AE-il-10-and-nutritional-status-in-pediatric-patients-with-biliary-atresia
#2
Maria Ines de Albuquerque Wilasco, Carolina Uribe-Cruz, Daniele Santetti, Gabriel Rodrigo Fries, Cristina Toscani Leal Dornelles, Themis Reverbel da Silveira
OBJECTIVES: The objective of the present study is to evaluate whether IL-6, TNF-α, IL-10 are associated with nutritional status in patients with cirrhosis secondary to biliary atresia and compare to healthy controls. METHODS: The parameters used for nutritional assessment were the standard deviation scores of height-for-age and of triceps skinfold thickness-for-age. The severity of cirrhosis was evaluated using the Child-Pugh score and PELD/MELD. Serum cytokines were measured using Cytometric Bead Array flow cytometry...
March 19, 2017: Jornal de Pediatria
https://www.readbyqxmd.com/read/28321310/neonatal-cholestasis-mimicking-biliary-atresia-could-it-be-urinary-tract-infection
#3
Noella Maria Delia Pereira, Ira Shah
Cholestasis can occur in newborns due to infections. However, the manifestations of the underlying infections usually dominate the presentation. We present a 2-month-old infant who presented with jaundice and no fever or signs of systemic illness. Liver biopsy was suggestive of cholangitis. He was subsequently detected to have urinary tract infection with Klebsiella pneumoniae. The child was treated with appropriate antibiotics for 2 weeks following which the cholestasis resolved. Thus, neonatal cholestasis due to infections can also occur in the post-neonatal period without clinical manifestations of an underlying infection...
2017: SAGE open medical case reports
https://www.readbyqxmd.com/read/28319568/predictive-value-of-intraoperative-troponin-i-elevation-in-pediatric-living-donor-liver-transplant-recipients-with-biliary-atresia
#4
Mingwei Sheng, Yuanbang Lin, Yiqi Weng, Rubin Xu, Ying Sun, Wenli Yu, Hongyin Du
BACKGROUND: Pediatric living donor liver transplantation is associated with slight alteration in cardiac enzymes without ongoing acute cardiac injury, but available information about the significance of these changes is limited. The aims of this study were to analyze the link between the anomalies of intraoperative serum cardiac troponin I (cTnI) and acute lung injury during the first week after liver transplantation. METHODS: In this retrospective study, 123 children suffered from biliary atresia were enrolled...
March 18, 2017: Transplantation
https://www.readbyqxmd.com/read/28304404/interactions-between-th1-cells-and-tregs-affect-regulation-of-hepatic-fibrosis-in-biliary-atresia-through-the-ifn-%C3%AE-stat1-pathway
#5
Jie Wen, Ying Zhou, Jun Wang, Jie Chen, Wenbo Yan, Jin Wu, Junkai Yan, Kejun Zhou, Yongtao Xiao, Yang Wang, Qiang Xia, Wei Cai
Regulatory T cells (Tregs) and CD4(+) T helper (Th) cells have important roles in bile duct injury of biliary atresia (BA). However, their impacts on liver fibrosis are undefined. Between 2013 and 2016, 146 patients with various stages of BA were enrolled in this study. Peripheral blood, liver biopsy and lymph node samples were collected. Flow cytometry, magnetic cell sorting and immunostaining were used to characterize lymphocytes from BA patients. Deficiency of Tregs was observed along with increased Th1, Th2 and Th17 frequencies in the peripheral blood and livers of BA patients...
March 17, 2017: Cell Death and Differentiation
https://www.readbyqxmd.com/read/28293742/the-accumulation-of-regulatory-t-cells-in-the-hepatic-hilar-lymph-nodes-in-biliary-atresia
#6
Naoya Sakamoto, Toshihiro Muraji, Haruo Ohtani, Kouji Masumoto
PURPOSE: A proposed etiopathogenesis of biliary atresia (BA) involves T-cell-mediated inflammatory bile duct damage and progressive hepatic fibrosis. Pediatric surgeons often observe swelling of the hepatic hilar lymph nodes during the Kasai procedure. Given the importance of regulatory mechanisms in immune responses, the present study was designed to analyze the quantitative changes of regulatory T cells (T reg cells) in the hepatic hilar lymph nodes (hepatic hilar LNs) and peripheral blood (PB) in BA...
March 14, 2017: Surgery Today
https://www.readbyqxmd.com/read/28289704/preferential-tnf%C3%AE-signaling-via-tnfr2-regulates-epithelial-injury-and-duct-obstruction-in-experimental-biliary-atresia
#7
Pranavkumar Shivakumar, Tatsuki Mizuochi, Reena Mourya, Sridevi Gutta, Li Yang, Zhenhua Luo, Jorge A Bezerra
Biliary atresia is an obstructive cholangiopathy of infancy that progresses to end-stage cirrhosis. Although the pathogenesis of the disease is not completely understood, previous reports link TNFα to apoptosis of the bile duct epithelium in the presence of IFNγ. Here, we investigate if TNFα signaling regulates pathogenic mechanisms of biliary atresia. First, we quantified the expression of TNFA and its receptors TNFR1 and TNFR2 in human livers and found an increased expression of the receptors at the time of diagnosis...
March 9, 2017: JCI Insight
https://www.readbyqxmd.com/read/28283556/utilization-of-reflex-testing-for-direct-bilirubin-in-the-early-recognition-of-biliary-atresia
#8
Leo Lam, Samarina Musaad, Campbell Kyle, Stephen Mouat
BACKGROUND: Delayed diagnosis of biliary atresia is an important cause of pediatric end-stage liver failure and liver transplantation. We sought to determine whether direct bilirubin is underutilized by retrospectively reviewing patients with biliary atresia. Further, we aimed to determine the role of reflex testing for direct bilirubin in patients suspected for jaundice. METHODS: The time intervals between total bilirubin and direct bilirubin measurements were retrospectively reviewed in patients with biliary atresia...
March 10, 2017: Clinical Chemistry
https://www.readbyqxmd.com/read/28263223/hypereosinophilic-syndrome-after-liver-transplantation-a-case-report-and-a-review-of-the-literature
#9
Wiebke Aulbert, Robin Kobbe, Christian Breuer, Andrea Briem-Richter, Hansjörg Schäfer, Florian Brinkert, Anne Dettmar, Markus J Kemper, Enke Grabhorn
Persistently elevated eosinophil granulocytes in the peripheral blood in children is challenging due to a complex diagnosis especially after solid organ transplantation and can lead to difficulties in finding an underlying causative factor.We report a 12-year-old boy who developed severe hypereosinophilia eleven years after liver transplantation (LT) due to biliary atresia. Accompanying symptoms were recurrent fever, fatigue, elevated liver enzymes, abdominal pain and significant weight loss. After exclusion of secondary causes of eosinophilia, an idiopathic hypereosinophilic syndrome (I-HES) was diagnosed...
March 4, 2017: Transplantation
https://www.readbyqxmd.com/read/28260293/-clinical-value-of-acoustic-radiation-force-impulse-technique-to-predict-esophageal-and-gastric-varices-in-patients-with-biliary-atresia
#10
G Y Zhang, Y Tang, N N Niu, H T Wu
Objective: To investigate the clinical value of acoustic radiation force impulse (ARFI)technique in predicting esophageal and gastric varices in patients with biliary atresia after Kasai portoenterostomy. Methods: A total of 42 patients with biliary atresia after Kasai portoenterostomy were collected from September 2015 to May 2016 in Tianjin First Central Hospital.ARFI technique was used to measure the stiffness of liver and spleen, and 28 healthy children as control.According to the result of CT examination , patients with biliary atresia were divided into two groups , twenty-three patients with esophageal and gastric varices(A group) and nineteen patients without esophageal and gastric varices (B group), Comparing the difference of liver and spleen stiffness between the two groups...
February 21, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/28259742/gamma-glutamyl-transferase-levels-at-age-2-stratifies-progression-of-portal-hypertension-in-biliary-atresia
#11
A Jay Freeman, Vicky Lee Ng, Sanjiv Harpavat, Alexander Hrycko, Zoe Apted, Pinar Bulut, Traci Leong, Saul J Karpen
No abstract text is available yet for this article.
March 1, 2017: Clinical Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/28256002/a-case-of-prenatally-detected-left-isomerism-and-hemiazygos-continuation-of-inferior-vena-cava
#12
Behnaz Moradi, Najme-Sadat Moosavi, Mohamad Ali Kazemi, Ahmad-Reza Tahmasebpour, Farzaneh Fattahi Masrour
Heterotaxy syndromes are defined as the disorders that involve abnormal arrangement of viscera. We present a case of prenatally diagnosed left isomerism in a 30-year-old primigravida woman referred to our hospital for complex cardiac abnormality. Sonographic findings included heart block, unbalanced atrioventricular septal defect, interruption of the inferior vena cava with hemiazygos continuation, double superior vena cava, a right-sided stomach, and biliary atresia. The hemiazygos vein drained into the right atrium by the persistent left superior vena cava...
March 3, 2017: Journal of Clinical Ultrasound: JCU
https://www.readbyqxmd.com/read/28244293/epidemiology-of-biliary-atresia-in-korea
#13
Kyung Jae Lee, Ju Whi Kim, Jin Soo Moon, Jae Sung Ko
Biliary atresia (BA) is the major cause of cholestasis and the leading indication for liver transplantation (LT). However, the incidence of BA in Korea has not been reported. The aim of this study was to investigate the incidence and clinical outcomes of BA in Korea. We used the Korean universal health insurance database and extracted data regarding BA patients younger than 18 years of age admitted between 2011 and 2015. The incidence of BA was calculated by dividing the number of BA patients by the number of live births...
April 2017: Journal of Korean Medical Science
https://www.readbyqxmd.com/read/28241003/-duodenal-diaphragm-diagnosis-in-a-school-aged-child-and-minimally-invasive-treatment-case-report
#14
Andrea Barrueto Barrera, Sofia Santelices Baeza, Francisco Miranda Labra, David Schnettler Rodríguez
Duodenal atresia is the third cause of intrinsic intestinal obstruction in the neonatal period. Typical presentation includes early-onset vomiting of gastric or bilious content, abdominal distension and poor weight gain. If the obstruction is incomplete, as in a perforated duodenal diaphragm, presenting symptoms tend to appear later and be nonspecific, so diagnosis is usually delayed. We present the case of a 9-year-old girl with a history of biliary postprandial vomiting from the infancy period, without any impact on the nutritional status, managed symptomatically...
January 31, 2017: Medwave
https://www.readbyqxmd.com/read/28239322/intestinal-barrier-integrity-and-function-in-infants-with-cholestasis
#15
Nagla H Abu Faddan, Tahra M K Sherif, Omnia A Mohammed, Khalid A Nasif, Ebtesam M El Gezawy
BACKGROUND/AIMS: The safety of the human body is maintained by effective monitoring of the mucosal surface integrity and protection against potentially harmful compounds. This function of the gut called intestinal barrier function can be affected by cholestasis and the absence of bile in the intestinal lumen. We aimed to determine whether the gut barrier integrity is impaired in infants with cholestasis by evaluation of the intestinal fatty acid binding proteins (I-FABP) and ileal bile acid binding protein (I-BABP) as markers of intestinal epithelial cell damage and plasma D-lactate level as a marker of gut wall permeability...
January 2017: Intestinal Research
https://www.readbyqxmd.com/read/28238015/syndrome-of-inappropriate-antidiuretic-hormone-secretion-following-liver-transplantation
#16
Kosei Takagi, Takahito Yagi, Susumu Shinoura, Yuzo Umeda, Ryuichi Yoshida, Daisuke Nobuoka, Nobuyuki Watanabe, Takashi Kuise, Tomokazu Fuji, Hiroyuki Araki, Toshiyoshi Fujiwara
Syndrome of inappropriate antidiuretic hormone secretion (SIADH) is an extremely rare cause of hyponatremia post-liver transplantation. A 15-year-old Japanese girl with recurrent cholangitis after Kasai surgery for biliary atresia underwent successful living donor liver transplantation. Peritonitis due to gastrointestinal perforation occurred. Hyponatremia gradually developed but improved after hypertonic sodium treatment. One month later, severe hyponatremia rapidly recurred. We considered the hyponatremia's cause as SIADH...
February 2017: Acta Medica Okayama
https://www.readbyqxmd.com/read/28203327/a-rapid-screening-test-on-dried-blood-for-the-neonatal-diagnosis-of-tyrosinemia-type-i
#17
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
#18
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
#19
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
#20
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
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