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Liver disease in children

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https://www.readbyqxmd.com/read/28937543/cholangiocarcinoma-among-children-and-adolescents-a-review-of-the-literature-and-seer-database-analysis
#1
Jennifer R Newsome, Rajkumar Venkatramani, Andras Heczey, Heather E Danysh, Douglas S Fishman, Tamir Miloh
OBJECTIVES: Cholangiocarcinoma (CCA) is a biliary malignancy found primarily in adults. The incidence of CCA in children is unknown. The aim of this study was to describe characteristics of CCA in children and adolescents. METHODS: Using the Surveillance, Epidemiology, and End Results Program (SEER 18) database, we identified incident cases of CCA diagnosed at <20 years of age during the period of 1973-2013. Additionally, we reviewed published case reports describing pediatric patients with CCA...
September 20, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28937538/severe-neonatal-cholestasis-in-cerebrotendinous-xanthomatosis-genetics-immunostaining-mass-spectrometry
#2
Jing-Yu Gong, Kenneth D R Setchell, Jing Zhao, Wujuan Zhang, Brian Wolfe, Yi Lu, Karolin Lackner, A S Knisely, Neng-Li Wang, Chen-Zhi Hao, Mei-Hong Zhang, Jian-She Wang
OBJECTIVES: Cerebrotendinous xanthomatosis (CTX) is caused by defects in sterol 27-hydroxylase (CYP27A1, encoded by CYP27A1), a key enzyme in the bile acid synthesis pathway. CTX usually presents as neurologic disease in adults or older children. The very rare reports of CTX manifest as neonatal cholestasis assess the cholestasis as transient, with patient survival. Our experience differs. METHODS: Homozygous or compound heterozygous CYP27A1 mutations were detected in 8 neonatal cholestasis patients by whole exome sequencing, panel sequencing, or Sanger sequencing...
September 20, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28935574/taurocholate-induces-biliary-differentiation-of-liver-progenitor-cells-causing-hepatic-stellate-cell-chemotaxis-in-the-ductular-reaction-role-in-pediatric-cystic-fibrosis-liver-disease
#3
Katarzyna N Pozniak, Michael A Pearen, Tamara N Pereira, Cynthia Sm Kramer, Priyakshi Kalita-De Croft, Sujeevi K Nawaratna, Manuel A Fernandez-Rojo, Geoffrey N Gobert, Janina Ee Tirnitz-Parker, John K Olynyk, Ross W Shepherd, Peter J Lewindon, Grant A Ramm
Cystic fibrosis liver disease (CFLD) in children causes progressive fibrosis leading to biliary cirrhosis, however its cause(s) and early pathogenesis are unclear. We hypothesised that a bile acid-induced Ductular Reaction (DR) drives fibrogenesis. We evaluated the DR by cytokeratin-7 immunohistochemistry in liver biopsies, staged for fibrosis, from 60 children with CFLD and demonstrated that the DR was significantly correlated with hepatic fibrosis stage and biliary taurocholate levels. To examine the mechanisms involved in DR induction liver progenitor cells (LPCs) were treated with taurocholate and key events in DR evolution were assessed: LPC proliferation, LPC biliary differentiation and hepatic stellate cell (HSC) chemotaxis...
September 18, 2017: American Journal of Pathology
https://www.readbyqxmd.com/read/28934563/hemophagocytic-lymphohistiocytosis
#4
Hanny Al-Samkari, Nancy Berliner
Hemophagocytic lymphohistiocytosis is a life-threatening disorder characterized by unbridled activation of cytotoxic T lymphocytes, natural killer (NK) cells, and macrophages resulting in hypercytokinemia and immunemediated injury of multiple organ systems. It is seen in both children and adults and is recognized as primary (driven by underlying genetic mutations that abolish critical proteins required for normal function of cytotoxic T cells and NK cells) or secondary (resulting from a malignant, infectious, or autoimmune stimulus without an identifiable underlying genetic trigger)...
September 13, 2017: Annual Review of Pathology
https://www.readbyqxmd.com/read/28934066/characterization-of-t-cell-immune-responses-in-clinical-trials-of-the-candidate-rts-s-malaria-vaccine
#5
Philippe Moris, Erik Jongert, Robbert G van der Most
The candidate malaria vaccine RTS,S has demonstrated 45.7% efficacy over 18 months against all clinical disease in a phase-III field study of African children. RTS,S targets the circumsporozoite protein (CSP), which is expressed on the Plasmodium sporozoite during the pre-erythrocyte stage of its life-cycle; the stage between mosquito bite and liver infection. Early in the development of RTS,S, it was recognized that CSP-specific cell-mediated immunity (CMI) was required to complement CSP-specific antibody-mediated immunity...
September 21, 2017: Human Vaccines & Immunotherapeutics
https://www.readbyqxmd.com/read/28932122/gadobutrol-in-india-a-comprehensive-review-of-safety-and-efficacy
#6
REVIEW
Jan Endrikat, Nicoletta Anzalone
Gadobutrol is a gadolinium (Gd)-based contrast agent for magnetic resonance imaging (MRI). In India, gadobutrol is approved for MRI of the central nervous system (CNS), liver, kidneys, breast and for MR angiography for patients 2 years and older. The standard dose for all age groups is 0.1 mmol/kg body weight. The safety profile has been demonstrated in 42 clinical phase 2 to 4 studies (>6800 patients), 7 observational studies, and by assessing pharmacovigilance data of 29 million applications. Furthermore, studies in children, adults, and elderly and in patients with impaired liver or kidney function did not show any increased adverse event rate...
2017: Magnetic Resonance Insights
https://www.readbyqxmd.com/read/28930295/global-burden-of-nafld-and-nash-trends-predictions-risk-factors-and-prevention
#7
REVIEW
Zobair Younossi, Quentin M Anstee, Milena Marietti, Timothy Hardy, Linda Henry, Mohammed Eslam, Jacob George, Elisabetta Bugianesi
NAFLD is one of the most important causes of liver disease worldwide and will probably emerge as the leading cause of end-stage liver disease in the coming decades, with the disease affecting both adults and children. The epidemiology and demographic characteristics of NAFLD vary worldwide, usually parallel to the prevalence of obesity, but a substantial proportion of patients are lean. The large number of patients with NAFLD with potential for progressive liver disease creates challenges for screening, as the diagnosis of NASH necessitates invasive liver biopsy...
September 20, 2017: Nature Reviews. Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28925053/extracorporeal-membrane-oxygenation-can-save-lives-in-children-with-heart-or-lung-failure-after-liver-transplantation
#8
Sandrine Jean, Christophe Chardot, Mehdi Oualha, Carmen Capito, Olivier Bustarret, Philippe Pouard, Sylvain Renolleau, Florence Lacaille, Laurent Dupic
The risk of cardiac or lung failure after liver transplantation (LT) is significant. In rare cases, the usual intensive care techniques fail to maintain organ oxygenation with a risk of multiorgan dysfunction. Although extracorporeal membrane oxygenation (ECMO) is a difficult and risky procedure, it can be proposed as life-saving. Four children with either acute pulmonary (three) or cardiac (one) failure after LT, and the criteria that decided the use of ECMO (level of ventilation and results, dosage of inotropic drugs, cardiac ultrasound, blood lactate) were retrospectively reported...
September 2017: Artificial Organs
https://www.readbyqxmd.com/read/28921939/extreme-hepatic-resections-for-the-treatment-of-advanced-hepatoblastoma-are-planned-close-margins-an-acceptable-approach
#9
Adriana Fonseca, Abha Gupta, Furqan Shaikh, Raveena Ramphal, Vicky Ng, Ian McGilvray, J Ted Gerstle
BACKGROUND: Orthotopic liver transplantation (OLT) is considered the standard for children with hepatoblastoma (HB) in whom complete surgical resection is not possible. However, OLT is not always available or feasible. OBJECTIVE: To describe the outcome of children with HB who were initially deemed unresectable and underwent complex hepatectomy with planned close margins, and ultimately avoided OLT. METHODS: Demographic data, surgical and pathologic details, and survival information were collected from children treated for HB between January 2010 to December 2015...
September 17, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28918383/liver-transplantation-in-children-state-of-the-art-and-future-perspectives
#10
REVIEW
Rohit Kohli, Miriam Cortes, N D Heaton, Anil Dhawan
In this review, we provide a state of the art of liver transplantation in children, as the procedure is now carried out for more than 30 years and most of our paediatric colleagues are managing these patients jointly with liver transplant centres. Our goal for this article is to enhance the understanding of the liver transplant process that a child and his family goes through while explaining the surgical advances and the associated complications that could happen in the immediate or long-term follow-up. We have deliberately introduced the theme that 'liver transplant is a disease' and 'not a cure', to emphasise the need for adherence with immunosuppression, a healthy lifestyle and lifelong medical follow-up...
September 16, 2017: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/28917237/screening-for-nutritional-risk-in-hospitalized-children-with-liver-disease
#11
Tiantian Song, Ying Mu, Xue Gong, Wenyan Ma, Li Li
BACKGROUND AND OBJECTIVES: Malnutrition is a major contributor to morbidity and mortality from pediatric liver disease. We investigated the prevalence of both malnutrition and high nutritional risk in hospitalized children with liver disease as well as the rate of in-hospital nutritional support. METHODS AND STUDY DESIGN: A total of 2,874 hospitalized children and adolescents with liver disease aged 1 to 17 years (inclusive) were enrolled. Malnutrition was screened by anthropometric measures (height-for-age, weight-for-height, weight-for-age, and BMI- for-age z-scores)...
2017: Asia Pacific Journal of Clinical Nutrition
https://www.readbyqxmd.com/read/28914695/autoimmune-acute-liver-failure-and-seronegative-autoimmune-liver-disease-in-children-are-they-different-from-classical-disease
#12
Vikas Jain, Anshu Srivastava, Surender K Yachha, Niraj Kumari, Rachana Kathuria, Moinak S Sarma, Ujjal Poddar, Narendra Krishnani
OBJECTIVES: Presentation as autoimmune acute liver failure (AI-ALF) and seronegative autoimmune liver disease (SN-AILD) represents two uncommon variants of AILD. We compared the clinical profile and outcome of AI-ALF with autoimmune-non-acute liver failure (AI-non-ALF) and also SN-AILD with seropositive autoimmune liver disease (SP-AILD). MATERIALS AND METHODS: Children managed as AI-ALF and AI-non-ALF including SN-AILD and SP-AILD were enrolled and compared. AI-non-ALF was diagnosed by simplified diagnostic criteria and AI-ALF by Pediatric Acute Liver Failure Study Group criteria with positive autoantibody, exclusion of other etiologies, elevated immunoglobulin G and histology when available...
September 13, 2017: European Journal of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28912414/polish-experience-with-liver-transplantation-and-post-transplant-outcomes-in-children-with-urea-cycle-disorders
#13
Edyta Szymańska, Piotr Kaliciński, Joanna Pawłowska, Sylwia Szymańska, Maciej Pronicki, Marek Stefanowicz, Joanna Teisseyre, Dorota Broniszczak, Dariusz Rokicki
BACKGROUND Liver transplantation (LT) is recommended for various metabolic diseases, including urea cycle disorders (UCDs). The aim of this study was to determine indications and outcomes of LT for UCDs in the tertiary reference Children's Hospital in Warsaw, Poland. MATERIAL AND METHODS Medical charts of children with UCD who underwent LT between 2008 and July 2016 were retrospectively reviewed. The following parameters were analyzed: symptoms at time of diagnosis, age at diagnosis, age at transplantation, graft characteristics and survival, postsurgical complications, and biochemical and laboratory results before and after transplantation...
September 15, 2017: Annals of Transplantation: Quarterly of the Polish Transplantation Society
https://www.readbyqxmd.com/read/28902093/bile-acid-synthesis-disorders-in-arabs-a-10-year-screening-study
#14
Abdulrahman Al-Hussaini, Kenneth D R Setchell, Bader AlSaleem, James E Heubi, Khurram Lone, Anne Davit-Spraul, Emmanuel Jacquemin
OBJECTIVES: Early diagnosis of bile acid synthesis disorders (BASD) is important because, untreated, these conditions can be fatal. Our objectives were to screen children with cholestasis or unexplained liver disease for BASD and in those with confirmed BASD to evaluate the effectiveness of cholic acid therapy. METHODS: A routine serum total bile acid measurement was performed on children with cholestasis, liver cirrhosis, and liver failure. Patients were screened for BASD by fast atom bombardment ionization-mass spectrometry (FAB-MS) analysis of urine, and molecular analysis confirmed diagnosis...
September 9, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28895096/palivizumab-prophylaxis-against-respiratory-syncytial-virus-infection-in-children-with-immunocompromised-conditions-or-down-syndrome-a-multicenter-post-marketing-surveillance-in-japan
#15
Tomoko Kashiwagi, Yukiko Okada, Ken Nomoto
OBJECTIVE: The aim of this study was to assess the safety and effectiveness of palivizumab for the prevention of lower respiratory tract infection (LRI) caused by respiratory syncytial virus (RSV) in children with immunocompromised conditions or Down syndrome. METHODS: In this multicenter, post-marketing surveillance study (December 2013 to December 2015), children aged ≤24 months with immunocompromised conditions or Down syndrome (without hemodynamically significant congenital heart disease) receiving palivizumab immunoprophylaxis during two RSV seasons were observed until 30 days after the final palivizumab injection...
September 11, 2017: Paediatric Drugs
https://www.readbyqxmd.com/read/28892185/prevalence-comorbidities-and-mortality-of-toxic-shock-syndrome-in-united-states-children-and-adults
#16
Mark A Strom, Derek Y Hsu, Jonathan I Silverberg
Toxic Shock Syndrome (TSS) is a superantigen-mediated illness characterized by rash, hypotension and multi-organ dysfunction. The predictors of TSS and related morbidity and mortality are poorly defined. We analyzed data on 61,959,084 hospitalizations from the 2003-2012 Nationwide Inpatient Sample, a 20% stratified sample of US hospitalizations. ICD-9-CM coding was used to identify 4,491 hospitalizations with a diagnosis of TSS. Incidence, in-hospital mortality rate, comorbidities, length of stay and costs of care due to TSS were determined...
September 11, 2017: Microbiology and Immunology
https://www.readbyqxmd.com/read/28888897/hepatobiliary-disease-in-children-and-adolescents-with-cystic-fibrosis
#17
Fernanda de S Nascimento, Nelson A Sena, Tatiane da A Ferreira, Cibele D F Marques, Luciana R Silva, Edna Lúcia S de Souza
OBJECTIVES: The aims of the study were to determine the frequency of hepatobiliary disease in patients with CF and to describe the sociodemographic, clinical, and laboratory profile of these patients. METHODS: This was a retrospective, descriptive, and analytical study of 55 patients diagnosed with CF fibrosis, aged between 3 months and 21 years, followed-up from January 2008 to June 2016 in a referral center. Medical records were consulted, including sociodemographic, clinical and laboratory data, including hepatobiliary alterations, imaging studies, genetic studies, liver biopsies, and upper digestive endoscopies...
September 7, 2017: Jornal de Pediatria
https://www.readbyqxmd.com/read/28887821/serpina1-and-man1b1-polymorphisms-are-not-linked-to-severe-liver-disease-in-a-french-cohort-of-alpha-1-antitrypsin-deficiency-children
#18
Philippe Joly, Alain Lachaux, Mathias Ruiz, Lioara Restier, Abdelhouaed Belmalih, Colette Chapuis-Cellier, Alain Francina, Céline Renoux, Marion Bouchecareilh
BACKGROUND & AIMS: Fifteen to twenty percent of alpha-1 antitrypsin deficiency patients (A1ATD) have a severe liver outcome (portal hypertension - PHT) during childhood. Since they all share the same ZZSERPINA1 genotype and that environmental factors such as alcohol cannot be advanced, the presence of modifier genes is now well recognized. SNPs located on the SERPINA1 and MAN1B1 genes have already been tested in very few studies with contradictory or not replicated results. METHODS: Our genotype-phenotype correlation study, performed on 92 ZZ children, aimed at determining once and for all if SERPINA1 and MAN1B1 polymorphisms may be implied in the onset of PHT...
September 8, 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/28884036/severe-hepatopulmonary-syndrome-in-a-child-with-caroli-syndrome
#19
W De Jesus-Rojas, K McBeth, A Yadav, J M Stark, R A Mosquera, C Jon
Hepatopulmonary Syndrome (HPS) is a potential complication of chronic liver disease and is more commonly seen in the adult population. Caroli Syndrome is a rare inherited disorder characterized by intrahepatic ductal dilation and liver fibrosis that leads to portal hypertension. In children with liver disease, HPS should be considered in the differential diagnosis of prolonged, otherwise unexplained, hypoxemia. The presence of HPS can improve patient priority on the liver transplantation wait list, despite their Pediatric End-Stage Liver Disease (PELD) score...
2017: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/28882581/premature-ovarian-senescence-and-high-miscarriage-rate-impair-fertility-in-women-with-hepatitis-c-virus-infection
#20
Aimilia Karampatou, Xue Han, Loreta A Kondili, Gloria Taliani, Alessia Ciancio, Filomena Morisco, Rosina Maria Critelli, Enrica Baraldi, Veronica Bernabucci, Giulia Troshina, Maria Guarino, Simonetta Tagliavini, Federica D'Ambrosio, Laura Bristot, Laura Turco, Stefano Rosato, Stefano Vella, Tommaso Trenti, Isabella Neri, Antonio La Marca, Shivaji Manthena, Andrea S Goldstein, Savino Bruno, Yanjun Bao, Yuri Sanchez Gonzalez, Erica Villa
BACKGROUND AND AIMS: Premenopausal Hepatitis C Virus positive (HCV+) women have failing ovarian function.We thus investigated reproductive history,risk of infertility and pregnancy outcomes in HCV+ women of childbearing age. METHODS: Three different groups were studied:1) Clinical cohort:100 HCV+ women with chronic liver disease (CLD), age matched with 50 HBV+ women with CLD and with 100 healthy women consecutively observed in 3 Italian Gastroenterology Units;2) 1998 HCV+ women enrolled in the Italian Platform for the Study of Viral Hepatitis Therapies (PITER);3) 6...
September 4, 2017: Journal of Hepatology
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