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Cirrhosis in children

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https://www.readbyqxmd.com/read/29196283/ringed-telangiectasias-an-unusual-presentation-of-telangiectasia-macularis-eruptiva-perstans
#1
Laila Siddique, Hadjh Ahrns, Elizabeth Seiverling
Telangiectasia macularis eruptiva perstans (TMEP) is a rare form of cutaneous mastocytosis. While most cutaneous mastocytoses occur in children and are asymptomatic, TMEP occurs predominantly in adults and is associated with systemic manifestations, requiring medical management. TMEP is typically characterised by scattered red-brown macules on the trunk and extremities, but must be differentiated from other telangiectatic conditions such as scleroderma, hereditary haemorrhagic telangiectasia and telangiectasias secondary to cirrhosis...
November 30, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/29193603/quality-of-life-in-adolescents-with-hepatitis-c-treated-with-sofosbuvir-and-ribavirin
#2
Zobair M Younossi, Maria Stepanova, Kathleen B Schwarz, Stefan Wirth, Philip Rosenthal, Regino Gonzalez-Peralta, Karen Murray, Linda Henry, Sharon Hunt
Chronic HCV infection has been associated with impairment of health-related quality of life (HRQL) in both adults and pediatric patients. Our aim was to assess the HRQL of HCV-positive children treated with SOF+RBV. The data for this post-hoc analysis was collected in a phase 2 open-label multinational study that evaluated safety and efficacy of sofosbuvir (400mg/day) plus ribavirin (weight-based up to 1400mg/day) for 12 or 24 weeks in adolescents with chronic HCV (GS-US-334-1112). Patients and their parents/guardians completed the PedsQL-4...
November 28, 2017: Journal of Viral Hepatitis
https://www.readbyqxmd.com/read/29183423/outcomes-of-kasai-hepatoportoenterostomy-in-children-with-biliary-atresia-in-johannesburg-south-africa
#3
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
https://www.readbyqxmd.com/read/29128061/the-genetics-of-pediatric-nonalcoholic-fatty-liver-disease
#4
REVIEW
Nidhi P Goyal, Jeffrey B Schwimmer
Nonalcoholic fatty liver disease (NAFLD) is the leading cause of chronic liver disease in children. Severe fibrosis and cirrhosis are potential consequences of pediatric NAFLD and can occur within a few years of diagnosis. Observations suggest that genetics may be a strong modifying factor in the presentation, severity, and natural history of the disease. There is increasing interest in determining at-risk populations based on genetics in the hope of finding genotypes that correlate to NAFLD phenotype. Ultimately, the hope is to be able to tailor therapeutics to genetic predispositions and decrease disease morbidity in children with NAFLD...
February 2018: Clinics in Liver Disease
https://www.readbyqxmd.com/read/29126168/endocrine-disruptors-and-developmental-origins-of-nonalcoholic-fatty-liver-disease
#5
Lindsey S Treviño, Tiffany A Katz
Nonalcoholic fatty liver disease (NAFLD) is a growing epidemic worldwide, particularly in countries that consume a Western diet, and can lead to life-threatening conditions such as cirrhosis and hepatocellular carcinoma (HCC). With increasing prevalence of NAFLD in both children and adults, an understanding of the factors that promote NAFLD development and progression is crucial. Environmental agents, including endocrine-disrupting chemicals (EDCs), that have been linked to other diseases may play a role in NAFLD development...
November 6, 2017: Endocrinology
https://www.readbyqxmd.com/read/29115027/clinical-features-of-pediatric-autoimmune-hepatitis-in-japan-a-nationwide-survey
#6
Tsuyoshi Sogo, Atsushi Takahashi, Ayano Inui, Tomoo Fujisawa, Hiromasa Ohira, Hajime Takikawa
AIM: The purpose of this study was to determine the characteristics of children with autoimmune hepatitis (AIH) in Japan. METHODS: Questionnaires, which asked about patients newly diagnosed with AIH from 2009 to 2013 were sent to hospitals certified as training facilities for pediatrics in January 2015. RESULTS: A total of 35 patients were enrolled. The median age at diagnosis was 10 years (ranging from 3 months to 15 years), and the male-to-female ratio was 2:3...
November 8, 2017: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/29108191/-an-interpretation-of-the-aasld-practice-guideline-on-the-diagnosis-and-management-of-nonalcoholic-fatty-liver-disease-in-2017
#7
Y M Nan, N Fu, W C Li, L L Kong, X W Yuan, S Y Zhang, L D Liu, Y Lu, L Y Cui
The American Association for the Study of Liver Diseases (AASLD) updated and published the Practice Guidance for the Diagnosis and Management of Nonalcoholic Fatty Liver Disease (NAFLD) in July 2017, which provides recommendations for the accurate diagnosis, treatment, and effective prevention of NAFLD. Related metabolic diseases should be considered during the initial evaluation of patients suspected of NAFLD. Noninvasive diagnostic techniques including transient elastography, magnetic resonance elastography, and serum biochemical models should be used to evaluate the development and progression of liver fibrosis in patients with NAFLD...
September 20, 2017: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://www.readbyqxmd.com/read/29059700/favorable-response-to-immunosuppressive-combination-therapy-with-mizoribine-and-azathioprine-in-children-with-primary-sclerosing-cholangitis
#8
Hitoshi Tajiri, Yoh Zen, Tomoko Takano, Stephen Brooks
Primary sclerosing cholangitis (PSC), with no curative intervention, can progress to end-stage liver disease. Mizoribine, a purine antimetabolite, has never been used for the management of PSC. To evaluate the role of mizoribine with azathioprine we undertook a preliminary clinical study in children with PSC. Children with PSC and autoimmune features were simultaneously treated with mizoribine and azathioprine. Ursodeoxycholic acid or mesalazine were not regulated. The primary end-points of our study included improvement of aspartate aminotransferase, alanine aminotransferase, and γ-glutamyltransferase...
October 23, 2017: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/29030176/physiologically-based-pharmacokinetic-modeling-of-disposition-and-drug-drug-interactions-for-valproic-acid-and-divalproex
#9
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
#10
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.
November 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
#11
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...
December 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28983658/newborn-screening-for-biliary-atresia-in-the-united-states
#12
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...
December 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28977113/primary-sclerosing-cholangitis-in-children-and-adolescents
#13
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
#14
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
#15
Katarzyna N Pozniak, Michael A Pearen, Tamara N Pereira, Cynthia S M Kramer, Priyakshi Kalita-De Croft, Sujeevi K Nawaratna, Manuel A Fernandez-Rojo, Geoffrey N Gobert, Janina E E 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 hypothesized that a bile acid-induced ductular reaction (DR) drives fibrogenesis. The DR was evaluated by cytokeratin-7 immunohistochemistry in liver biopsies, staged for fibrosis, from 60 children with CFLD, and it 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 19, 2017: American Journal of Pathology
https://www.readbyqxmd.com/read/28905444/response-to-hepatitis-b-vaccination-in-patients-with-liver-cirrhosis
#16
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
#17
Abdulrahman A Al-Hussaini, Kenneth D R Setchell, Badr AlSaleem, James E Heubi, Khurram Lone, Anne Davit-Spraul, Emmanuel Jacquemin
OBJECTIVES: Early diagnosis of bile acid synthesis disorders (BASDs) 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...
December 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
#18
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
#19
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
#20
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: Clinical and experimental hepatology
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