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Cirrhosis in children

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https://www.readbyqxmd.com/read/29030176/physiologically-based-pharmacokinetic-modeling-of-disposition-and-drug-drug-interactions-for-valproic-acid-and-divalproex
#1
Todd M Conner, Vahagn C Nikolian, Patrick E Georgoff, Manjunath P Pai, Hasan B Alam, Duxin Sun, Ronald C Reed, Tao Zhang
Valproic acid (VPA) is an older first-line antiepileptic drug with a complex pharmacokinetic (PK) profile, currently under investigation for several novel neurologic and non-neurologic indications. Our study objective was to design and validate a mechanistic model of VPA disposition in adults and children; and evaluate its predictive performance of drug-drug interactions (DDIs). This study expands upon existing physiologically based pharmacokinetic (PBPK) models for VPA by incorporating UGT enzyme kinetics and an advanced dissolution, absorption, and metabolism (ADAM) model for extended-release (ER) formulation...
October 10, 2017: European Journal of Pharmaceutical Sciences
https://www.readbyqxmd.com/read/28986027/cystic-fibrosis-related-cirrhosis
#2
Daniel H Leung, Michael R Narkewicz
While liver involvement is common in cystic fibrosis, the major liver disorder with impact on the clinical outcome of individuals with CF is the development of multilobular cirrhosis with progression to portal hypertension. Interestingly, this is a disorder primarily of children and adolescents. We review the proposed pathogenesis, clinical presentation, diagnostic work-up, medical and surgical management, and complications of CF cirrhosis.
September 16, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28983725/liver-transplantation-for-biliary-atresia-a-systematic-review
#3
REVIEW
Mureo Kasahara, Koji Umeshita, Seisuke Sakamoto, Akinari Fukuda, Hiroyuki Furukawa, Shinji Uemoto
Biliary atresia (BA) is an idiopathic neonatal cholangiopathy characterized by progressive inflammatory obliteration of the intrahepatic or extrahepatic bile ducts. Although the Kasai operation has dramatically improved the outcomes in children with BA, most patients with BA eventually require liver transplantation (LT) even after undergoing a successful Kasai procedure. The Japanese LT Society (JLTS) was established in 1980 to characterize and follow trends in patient characteristics and the graft survival among all liver transplant patients in Japan...
October 5, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28983658/newborn-screening-for-biliary-atresia-in-the-united-states
#4
Cat Goodhue, Michael Fenlon, Kasper S Wang
Despite advances in our understanding of the pathogenesis of biliary atresia (BA), BA remains the most common cause of end-stage liver disease in children and the leading indication for pediatric liver transplantation. Age at time of Kasai portoenterostomy (KPE), performed to provide bile drainage, strongly correlates with transplant-free survival, mostly due to progression of intrahepatic fibrosis to cirrhosis. Unfortunately, challenges remain in recognizing that a jaundiced infant may have BA. To better diagnose infants with BA at an earlier age, population-based screening programs in countries such as Taiwan, Japan, and China have utilized stool color cards...
October 5, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28977113/primary-sclerosing-cholangitis-in-children-and-adolescents
#5
Eleonora Druve Tavares Fagundes, Alexandre Rodrigues Ferreira, Caroline Caldeira Hosken, Thaís Costa Nascentes Queiroz
BACKGROUND: Primary sclerosing cholangitis is a rare disease, but its prevalence has been underestimated in children and adolescents due to broad variation in clinical presentation as well as diagnostic challenges in this life period. OBJECTIVE: To evaluate children and adolescents with primary sclerosing cholangitis and to describe their clinical, laboratorial, histopathological, and cholangiography conditions. METHODS: This is an observational descriptive research that took place from 2005 to 2016 and included all the patients seen in the Outpatient Unit for Pediatric Hepatology of Hospital das Clinicas of UFMG who had been diagnosed with primary sclerosing cholangitis before the age of 18...
October 2, 2017: Arquivos de Gastroenterologia
https://www.readbyqxmd.com/read/28957984/health-related-quality-of-life-in-adolescent-patients-with-hepatitis-c-genotype-1-treated-with-sofosbuvir-and-ledipasvir
#6
Zobair M Younossi, Maria Stepanova, William Balistreri, Kathleen Schwarz, Karen F Murray, Philip Rosenthal, Sanjay Bansal, Sharon Hunt
OBJECTIVE: To assess the impact of treatment with ledipasvir/sofosbuvir (LDV/SOF) on the health-related quality of life (HRQL) of pediatric patients with chronic hepatitis C virus (HCV) infection. METHODS: Adolescents (12-17 years) with HCV were treated with LDV/SOF (90 mg/400 mg daily) for 12 weeks. HRQL was assessed using the PedsQLv4.0-SF15 completed by the children and caregivers before, during, and after treatment. RESULTS: We included 100 adolescents with HCV genotype 1 infection [14...
September 27, 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
#7
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/28905444/response-to-hepatitis-b-vaccination-in-patients-with-liver-cirrhosis
#8
REVIEW
Ioanna Aggeletopoulou, Panagiota Davoulou, Christos Konstantakis, Konstantinos Thomopoulos, Christos Triantos
Hepatitis B vaccination is strongly recommended for all infants and children but also for adults who are at risk of HBV infection. Attempts to immunize patients with liver cirrhosis have been proven relatively ineffective, and several strategies have already been used to improve the immune response in this group. The primary aim of this review is to examine, discuss, and summarize the immunogenicity of hepatitis B vaccination in patients with liver cirrhosis. MEDLINE search identified 11 studies (n = 961)...
September 14, 2017: Reviews in Medical Virology
https://www.readbyqxmd.com/read/28902093/bile-acid-synthesis-disorders-in-arabs-a-10-year-screening-study
#9
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/28895701/management-of-cirrhotic-ascites-in-children-review-and-recommendations-part-2-electrolyte-disturbances-nonelectrolyte-disturbances-therapeutic-options
#10
David F Bes, M Cristina Fernández, Ivone Malla, Horacio A Repetto, Daniel Buamscha, Susana López, Roxana Martinitto, Miriam Cuarterolo, Fernando Alvarez
Ascites is a major complication of cirrhosis. There are several evidence-based articles and guidelines for the management of adults, but few data have been published in relation to children. In the case of a pediatric patient with cirrhotic ascites (PPCA), the following questions are raised: How are the clinical assessment and ancillary tests performed? When is ascites considered refractory? How is it treated? Should fresh plasma and platelets be infused before abdominal paracentesis to prevent bleeding? What are the hospitalization criteria? What are the indicated treatments? What complications can patients develop? When and how should hyponatremia be treated? What are the diagnostic criteria for spontaneous bacterial peritonitis? How is it treated? What is hepatorenal syndrome? How is it treated? When should albumin be infused? When should fluid intake be restricted? The recommendations made here are based on pathophysiology and suggest the preferred approach to diagnostic and therapeutic aspects, and preventive care...
October 1, 2017: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/28892178/review-article-the-diagnostic-approach-and-current-management-of-chylous-ascites
#11
REVIEW
B Lizaola, A Bonder, H D Trivedi, E B Tapper, A Cardenas
BACKGROUND: Chylous ascites is rare, accounting for less than 1% of cases. An appropriate and stepwise approach to its diagnosis and management is of key importance. AIM: To review the current diagnostic approach and management of chylous ascites. METHODS: A literature search was conducted using PubMed using the key words 'chylous', 'ascites', 'cirrhosis', 'pathophysiology', 'nutritional therapy', 'paracentesis", "transjugular intrahepatic portosystemic shunt" and "TIPSS'...
November 2017: Alimentary Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/28856289/recommendations-for-the-treatment-of-hepatitis-b-in-2017
#12
REVIEW
Robert Flisiak, Waldemar Halota, Jerzy Jaroszewicz, Jacek Juszczyk, Piotr Małkowski, Małgorzata Pawłowska, Anna Piekarska, Krzysztof Simon, Krzysztof Tomasiewicz, Marta Wawrzynowicz-Syczewska
The therapeutic goal which is currently unfrequent but realistic in HBV infected patients is sustained HBsAg clearance. It is preceded by the loss or significant suppression of HBV replication and leads to inhibition of the progression of liver fibrosis, normalization of biochemical indicators of liver damage, reduction in the risk of hepatocellular carcinoma, prolongation of survival, prevention of HBV infection in the transplanted organ in post-transplant patients, enhancement of the quality of life, inhibition or reversal of extrahepatic changes associated with HBV infection, and halting of the spread of HBV infections...
June 2017: Clin Exp Hepatol
https://www.readbyqxmd.com/read/28834223/liver-and-systemic-hemodynamics-in-cirrhotic-children-impact-on-the-surgical-management-in-pediatric-living-donor-liver-transplantation
#13
Catherine de Magnée, Francis Veyckemans, Thierry Pirotte, Renaud Menten, Dana Dumitriu, Philippe Clapuyt, Karlien Carbonez, Catherine Barrea, Thierry Sluysmans, Christine Sempoux, Isabelle Leclercq, Francis Zech, Xavier Stephenne, Raymond Reding
BACKGROUND AND AIMS: Cirrhosis in adults is associated with modifications of systemic and liver hemodynamics, whereas little is known about the pediatric population. The aim of this work was to investigate whether alterations of hepatic and systemic hemodynamics were correlated with cirrhosis severity in children. The impact of hemodynamic findings on surgical management in pediatric living donor liver transplantation was evaluated. METHODS: Liver and systemic hemodynamics were studied prospectively in 52 children (median age= 1 year; 33 biliary atresia)...
August 17, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/28804516/the-role-of-sebelipase-alfa-in-the-treatment-of-lysosomal-acid-lipase-deficiency
#14
REVIEW
Angelika L Erwin
Lysosomal acid lipase deficiency (LALD) is a lysosomal storage disorder (LSD) characterized either by infantile onset with fulminant clinical course and very poor prognosis or childhood/adult-onset disease with an attenuated phenotype. The disorder is often misdiagnosed or remains undiagnosed in children and adults due to a rather unspecific clinical presentation with dyslipidemia and steatohepatitis. Until recently, no good treatment options were available for LALD. Despite supportive and symptomatic therapies, death occurred before 1 year of age in patients with infantile-onset disease and patients with childhood/adult-onset LALD suffered from significant complications, such as liver cirrhosis, requiring liver transplantation and early-onset cardiovascular disease...
July 2017: Therapeutic Advances in Gastroenterology
https://www.readbyqxmd.com/read/28800087/imaging-features-of-non-alcoholic-fatty-liver-disease-in-children-and-adolescents
#15
REVIEW
Michele Di Martino, Kameliya Koryukova, Mario Bezzi, Carlo Catalano
Non-invasive diagnosis and quantification of liver steatosis is important to overcome limits of liver biopsy, in order to follow up patients during their therapy and to establish a reference standard that can be used in clinical trials and longitudinal studies. Imaging offers several methods in this setting: ultrasound, which is the cheapest technique and easy to perform; magnetic resonance spectroscopy (MRS), which reflects the real content of triglycerides in a specific volume; and proton density fat fraction (PDFF) magnetic resonance, which is a simple method that reflects the distribution of the fat in the whole liver...
August 11, 2017: Children
https://www.readbyqxmd.com/read/28774413/management-of-hepatitis-b-in-special-populations
#16
REVIEW
Kali Zhou, Norah Terrault
Special populations infected with chronic HBV include those with decompensated cirrhosis, coinfections (HIV, HCV, HDV), hemodialysis and renal failure, immunosuppressed including transplant patients, children and women in pregnancy. These populations differ in their natural history and risk for liver-related complications, the indications for anti-HBV therapy as well as the recommendations regarding the HBV drugs used, duration of therapy and anticipated endpoints. Reflecting the special populations with substantive changes in management in recent years, this review focuses on HBV-HIV coinfected patients, immunosuppressed patients at risk for reactivation, liver transplant recipients and pregnant women...
June 2017: Best Practice & Research. Clinical Gastroenterology
https://www.readbyqxmd.com/read/28764309/death-rate-of-dental-anaesthesia
#17
REVIEW
Hamed Mortazavi, Maryam Baharvand, Yaser Safi
Death was the most important side effect of anaesthesia in dentistry. In this article we reviewed more than 20 studies with adequate data focusing on death associated with dental procedures since 1955 and found 218 deaths out of 71,435,282 patients (3 deaths per 1,000,000 persons) with the mortality rate of 1:327,684. In addition, mortality rate per million has dropped to half (6.2 per 1,000,000 vs. 3 per 1,000,000) since 1955 till the last report in 2012 without any sex predilection. In children, most cases died in the age of two to five years...
June 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28752463/semiquantitation-of-monomer-and-polymer-alpha-1-antitrypsin-by-centrifugal-separation-and-assay-by-western-blot-of-soluble-and-insoluble-components
#18
Keith S Blomenkamp, Jeffrey H Teckman
Alpha-1 antitrypsin (a1AT) deficiency, in its classical form, is an autosomal recessive disease associated with an increased risk of liver disease in adults and children, and with lung disease in adults. The vast majority of liver disease is associated with homozygosity for the Z mutant allele, also called PiZZ. This homozygous allele synthesizes large quantities of a1AT mutant Z protein in the liver, but the mutant protein also folds improperly during biogenesis. As a result, approximately 85% of the molecules are retained within the hepatocytes instead of being appropriately secreted...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28752441/pathophysiology-of-alpha-1-antitrypsin-deficiency-liver-disease
#19
Jeffrey H Teckman, Keith S Blomenkamp
Classical alpha-1 antitrypsin (a1AT) deficiency is an autosomal recessive disease associated with an increased risk of liver disease in adults and children, and with lung disease in adults (Teckman and Jain, Curr Gastroenterol Rep 16(1):367, 2014). The vast majority of the liver disease is associated with homozygosity for the Z mutant allele, the so-called PIZZ. These homozygous individuals synthesize large quantities of a1AT mutant Z protein in the liver, but the mutant protein folds improperly during biogenesis and approximately 85% of the molecules are retained within the hepatocytes rather than appropriately secreted...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28737869/management-of-cirrhotic-ascites-in-children-review-and-recommendations-part-1-pathophysiology-diagnostic-evaluation-hospitalization-criteria-treatment-nutritional-management
#20
REVIEW
David F Bes, M Cristina Fernández, Ivone Malla, Horacio A Repetto, Daniel Buamsch, Susana López, Roxana Martinitto, Miriam Cuarterolo, Fernando Álvarez
Ascites is a major complication of cirrhosis. There are several evidence-based articles and guidelines for the management of adults, but few data have been published in relation to children. In the case of pediatric patients with cirrhotic ascites (PPCA), the following questions are raised: How are the clinical assessment and ancillary tests performed? When is ascites considered refractory? How is it treated? Should fresh plasma and platelets be infused before abdominal paracentesis to prevent bleeding? What are the hospitalization criteria? What are the indicated treatments? What complications can patients develop? When and how should hyponatremia be treated? What are the diagnostic criteria for spontaneous bacterial peritonitis? How is it treated? What is hepatorenal syndrome? How is it treated? When should albumin be infused? When should fluid intake be restricted? The recommendations made here are based on pathophysiology and suggest the preferred approach to its diagnostic and therapeutic aspects, and preventive care...
August 1, 2017: Archivos Argentinos de Pediatría
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