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Cirrhosis in children

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https://www.readbyqxmd.com/read/27906797/non-cirrhotic-portal-fibrosis-in-pediatric-population
#1
Vikrant Sood, Bikrant Bihari Lal, Rajeev Khanna, Dinesh Rawat, Chhagan Bihari Sharma, Seema Alam
BACKGROUND: Non-cirrhotic portal fibrosis (NCPF) has been classically described as a disease of young to middle age with limited literature regarding its occurrence, onset or clinical presentation in children. We hereby present a series of 19 patients diagnosed and managed as NCPF in pediatric age group. METHODS: A retrospective review of all the patients presenting to the pediatric hepatology department (age < 18 years) and diagnosed as NCPF was done and data was evaluated...
November 30, 2016: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/27898593/coinfection-with-hiv-and-hepatitis-c-virus-in-229-children-and-young-adults-living-in-europe
#2
(no author information available yet)
OBJECTIVE: To characterize children, adolescents and young adults infected with HIV/hepatitis C virus (HCV) vertically or before age of 18 years and living in Europe regarding mode of acquisition, HCV genotype, clinical status and treatment. DESIGN: Retrospective, cross-sectional study using pooled data from 11 European paediatric HIV cohorts. METHODS: Patients aged more than 18 months and less than 25 years, with HIV/HCV acquired vertically or in childhood, were included...
January 2, 2017: AIDS
https://www.readbyqxmd.com/read/27896406/evaluation-of-rex-shunt-on-cavernous-transformation-of-the-portal-vein-in-children
#3
Ruo-Yi Wang, Jun-Feng Wang, Xiao-Gang Sun, Qian Liu, Jia-Long Xu, Qi-Gang Lv, Wei-Xiu Chen, Jin-Liang Li
BACKGROUND: Children with cavernous transformation of the portal vein (CTPV) develop severe complications from prehepatic portal hypertension, such as recurrent variceal bleeding and thrombocytopenia. In this study, we reported the results of 30 children with symptomatic CTPV that were treated by a Rex shunt. The effectiveness of this surgical approach was evaluated. METHODS: A retrospective review was performed of 30 children aged between 3 and 18 years with CTPV, who underwent a Rex shunt between 2008 and 2015...
November 28, 2016: World Journal of Surgery
https://www.readbyqxmd.com/read/27822768/pediatric-nonalcoholic-fatty-liver-disease-the-rise-of-a-lethal-disease-among-mexican-american-hispanic-children
#4
REVIEW
Monica M Betancourt-Garcia, Armando Arguelles, Jorge Montes, Ambrosio Hernandez, Manish Singh, R Armour Forse
Hispanic children of Mexican origin have a high incidence of NAFLD. Susceptibility has been linked to a combination of factors including an increasing epidemic of obesity in children and adolescents, an allele substitution in the PNPLA3 gene that reduces hepatic lipid catabolism, and an altered microbiome that may increase hepatic endotoxins. The combination of NAFLD and portal vein toxins secondary to an indigenous gut microbiome appear to lead to the early occurrence of NASH, which progresses to cirrhosis and early hepatocellular carcinoma...
November 7, 2016: Obesity Surgery
https://www.readbyqxmd.com/read/27822476/pathogenesis-of-nonalcoholic-steatohepatitis-interactions-between-liver-parenchymal-and-nonparenchymal-cells
#5
REVIEW
Nancy Magee, An Zou, Yuxia Zhang
Nonalcoholic fatty liver disease (NAFLD) is the most common type of chronic liver disease in the Western countries, affecting up to 25% of the general population and becoming a major health concern in both adults and children. NAFLD encompasses the entire spectrum of fatty liver disease in individuals without significant alcohol consumption, ranging from nonalcoholic fatty liver (NAFL) to nonalcoholic steatohepatitis (NASH) and cirrhosis. NASH is a manifestation of the metabolic syndrome and hepatic disorders with the presence of steatosis, hepatocyte injury (ballooning), inflammation, and, in some patients, progressive fibrosis leading to cirrhosis...
2016: BioMed Research International
https://www.readbyqxmd.com/read/27818588/update-on-occult-hepatitis-b-virus-infection
#6
REVIEW
Manoochehr Makvandi
The event of mutations in the surface antigen gene of hepatitis B virus (HBV) results in undetectable hepatitis B surface antigen with positive/negative anti-hepatitis B core (anti-HBc) antibody status in serum and this phenomenon is named occult hepatitis B infection (OBI). The presence of anti-HBc antibody in serum is an important key for OBI tracking, although about 20% of OBI cases are negative for anti-HBc antibody. The diagnosis of OBI is mainly based on polymerase chain reaction (PCR) and real-time PCR assays...
October 21, 2016: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/27799810/novel-treatment-options-for-lysosomal-acid-lipase-deficiency-critical-appraisal-of-sebelipase-alfa
#7
REVIEW
Kim Su, Emma Donaldson, Reena Sharma
Lysosomal acid lipase deficiency (LAL-D) is a rare disorder of cholesterol metabolism with an autosomal recessive mode of inheritance. The absence or deficiency of the LAL enzyme gives rise to pathological accumulation of cholesterol esters in various tissues. A severe LAL-D phenotype manifesting in infancy is associated with adrenal calcification and liver and gastrointestinal involvement with characteristic early mortality. LAL-D presenting in childhood and adulthood is associated with hepatomegaly, liver fibrosis, cirrhosis, and premature atherosclerosis...
2016: Application of Clinical Genetics
https://www.readbyqxmd.com/read/27796468/giant-hepatic-regenerative-nodules-in-alagille-syndrome
#8
Jordan B Rapp, Richard D Bellah, Carolina Maya, Bruce R Pawel, Sudha A Anupindi
BACKGROUND: Children with Alagille syndrome undergo surveillance radiologic examinations as they are at risk for developing cirrhosis and hepatocellular carcinoma. There is limited literature on the imaging of liver masses in Alagille syndrome. We report the ultrasound (US) and magnetic resonance imaging (MRI) appearances of incidental benign giant hepatic regenerative nodules in this population. OBJECTIVE: To describe the imaging findings of giant regenerative nodules in patients with Alagille syndrome...
October 28, 2016: Pediatric Radiology
https://www.readbyqxmd.com/read/27795726/serum-c-reactive-protein-in-children-with-liver-disease-and-ascites
#9
Gholamreza Kalvandi, Naser Honar, Bita Geramizadeh, Maryam Ataollahi, Asghar Rahmani, Hazhir Javaherizadeh
BACKGROUND: The diagnosis of peritonitis as a complication of cirrhosis is an important clinical problem. OBJECTIVES: The aim of this study was to evaluate serum C-reactive protein levels as a diagnostic factor for spontaneous bacterial peritonitis (SBP) in child patients with liver disease. METHODS: In this study, 150 children diagnosed with liver disease and ascites upon admission to Nemazee Teaching Hospital (Shiraz, Iran) were examined...
August 2016: Hepatitis Monthly
https://www.readbyqxmd.com/read/27748053/hepatocellular-carcinoma-in-children-and-young-patients-with-chronic-hbv-infection-and-the-usefulness-of-alpha-fetoprotein-assessment
#10
Hitoshi Tajiri, Tomoko Takano, Hideo Tanaka, Kosuke Ushijima, Ayano Inui, Yoko Miyoshi, Keiichi Ozono, Daiki Abukawa, Takeshi Endo, Stephen Brooks, Yasuhito Tanaka
The aims of the study were to elucidate the clinical characteristics of patients who developed hepatocellular carcinoma (HCC) related to persistent HBV infection since childhood and to investigate usefulness of assessing alpha-fetoprotein (AFP) in this population. A nationwide multicenter survey of children with chronic HBV infection was performed. Among 548 patients, 15 patients developed HCC at the median age of 15 years (range 9-36), including 13 males and 2 females. A case-control comparison showed that HBeAg seroconversion and liver cirrhosis were associated with the occurrence of HCC...
November 2016: Cancer Medicine
https://www.readbyqxmd.com/read/27746616/progressive-familial-intrahepatic-cholestasis-pfic-in-indian-children-clinical-spectrum-and-outcome
#11
Sajan Agarwal, Bikrant Bihari Lal, Dinesh Rawat, Archana Rastogi, Kishore G S Bharathy, Seema Alam
OBJECTIVE: To study the clinical and laboratory profile of children with progressive familial intrahepatic cholestasis (PFIC) and evaluate their outcome. METHODS: The study is a retrospective review of all cases diagnosed with PFIC between January 2011 and July 2015. All children underwent histopathological examination and immunostaining. Management was done as per institute's protocol. RESULTS: There were a total of 24 PFIC cases (PFIC 1-2, PFIC 2-19, PFIC 3-3)...
September 2016: Journal of Clinical and Experimental Hepatology
https://www.readbyqxmd.com/read/27677164/co-infection-with-hiv-and-hcv-in-229-children-and-young-adults-living-in-europe
#12
Claire Thorne, Anna Turkova, Giuseppe Indolfi, Elisabetta Venturini, Carlo Giaquinto
OBJECTIVE: To characterise children, adolescents and young adults infected with HIV/HCV vertically or before age 18 years and living in Europe regarding mode of acquisition, HCV genotype, clinical status and treatment. DESIGN: Retrospective, cross-sectional study using pooled data from 11 European paediatric HIV cohorts METHODS:: Patients aged > 18 months and < 25 years, with HIV/HCV acquired vertically or in childhood, were included. Anonymised individual-patient data were collected using a standard protocol and modified HIV Cohorts Data Exchange Protocol...
September 24, 2016: AIDS
https://www.readbyqxmd.com/read/27663417/portal-hypertension-in-children-high-risk-varices-primary-prophylaxis-and-consequences-of-bleeding
#13
Mathieu Duché, Béatrice Ducot, Oanez Ackermann, Florent Guérin, Emmanuel Jacquemin, Olivier Bernard
BACKGROUND & AIMS: Primary prophylaxis of bleeding is debated for children with portal hypertension because of the limited number of studies on its safety and efficacy, the lack of a known endoscopic pattern carrying a high-risk of bleeding for all causes, and the assumption that the mortality of a first bleed is low. We report our experience with these issues. METHODS: From 1989 to 2014, we managed 1300 children with portal hypertension. Endoscopic features were recorded; high-risk varices were defined as: grade 3 esophageal varices, grade 2 varices with red wale markings, or gastric varices...
September 20, 2016: Journal of Hepatology
https://www.readbyqxmd.com/read/27640355/infantile-cirrhosis-growth-impairment-and-neurodevelopmental-anomalies-associated-with-deficiency-of-ppp1r15b
#14
Saeed Mohammad, Lynne A Wolfe, Petra Stöbe, Saskia Biskup, Mark S Wainwright, Hector Melin-Aldana, Padmini Malladi, Maximilian Muenke, William A Gahl, Peter F Whitington
OBJECTIVE: To assess the utility of whole-exome sequencing (WES) in a sibling pair with undetermined liver disease and describe the phenotype associated with mutations discovered therein. STUDY DESIGN: Next-generation WES was performed on 2 siblings (S1 and S2) who were born to nonconsanguineous parents of European extraction. Both siblings developed cirrhosis of indeterminate etiology and required liver transplantation; S1 at 7 months and S2 at 22 months. RESULTS: Sequencing of germline DNA identified compound heterozygous mutations in PPP1R15B resulting in increased levels of phosphorylated eukaryotic translation initiation factor 2α...
September 15, 2016: Journal of Pediatrics
https://www.readbyqxmd.com/read/27629842/predictors-of-liver-disease-severity-in-children-with-chronic-hepatitis-b
#15
Maria Pokorska-Śpiewak, Barbara Kowalik-Mikołajewska, Małgorzata Aniszewska, Magdalena Pluta, Bożena Walewska-Zielecka, Magdalena Marczyńska
BACKGROUND: Evaluation of the liver histology is essential for the management of chronic hepatitis B (CHB) in children. OBJECTIVES: The aim of this study was to analyze the histopathological features in children with CHB and compare them with clinical and laboratory data. MATERIAL AND METHODS: The study comprised 30 treatment-naïve children (mean age: 12.8 ± 2.4; mean duration of infection: 11.7 ± 2.5 years; 16/30 HBeAg-positive and 14/30 HBeAg-negative), who underwent a liver biopsy due to CHB...
July 2016: Advances in Clinical and Experimental Medicine: Official Organ Wroclaw Medical University
https://www.readbyqxmd.com/read/27625189/screening-and-diagnosis-of-hbv-in-low-income-and-middle-income-countries
#16
Jean-Pierre Allain, Ohene Opare-Sem
HBV testing and diagnosis of HBV-related liver disease in low-income and middle-income countries differs substantially from that in developed countries in terms of access to resources and expensive technologies requiring highly specialized staff. For identification and classification of HBV infection, genomic amplification methods to detect and quantify HBV DNA are often nonexistent or available only in central laboratories of major cities. When samples from peripheral locations do arrive, delays in receiving results generate loss to follow-up...
November 2016: Nature Reviews. Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/27605415/yap-subcellular-localization-and-hippo-pathway-transcriptome-analysis-in-pediatric-hepatocellular-carcinoma
#17
Michael J LaQuaglia, James L Grijalva, Kaly A Mueller, Antonio R Perez-Atayde, Heung Bae Kim, Ghazaleh Sadri-Vakili, Khashayar Vakili
Pediatric hepatocellular carcinoma (HCC) is a rare tumor which is associated with an extremely high mortality rate due to lack of effective chemotherapy. Recently, the Hippo pathway and its transcriptional co-activator Yes-associated protein (YAP) have been shown to play a role in hepatocyte proliferation and development of HCC in animal models. Therefore, we sought to examine the activity of YAP and the expression of Hippo pathway components in tumor and non-neoplastic liver tissue from 7 pediatric patients with moderately differentiated HCC...
2016: Scientific Reports
https://www.readbyqxmd.com/read/27590612/hepatocellular-carcinoma-risk-in-alaska-native-children-and-young-adults-with-hepatitis-b-virus-retrospective-cohort-analysis
#18
Prabhu P Gounder, Lisa R Bulkow, Mary Snowball, Susan Negus, Philip R Spradling, Brian J McMahon
OBJECTIVES: To evaluate the hepatocellular carcinoma (HCC) risk in Alaska Native children and young adults with hepatitis B virus (HBV). STUDY DESIGN: Retrospective analysis of a population-based cohort of Alaska Native persons with HBV followed during 1982-2012. All individuals with HBV were offered HCC screening regardless of age using alpha-fetoprotein every 6 months; persons with an elevated alpha-fetoprotein or persons at high-risk for HCC, such as cirrhosis, family history of HCC, were offered ultrasound...
November 2016: Journal of Pediatrics
https://www.readbyqxmd.com/read/27547235/assessment-of-vitamin-d-status-in-a-group-of-egyptian-children-with-non-alcoholic-fatty-liver-disease-multicenter-study
#19
Amal Mohamed Ahmed, Maha Abdel Ghany, Gehan Lotfy Abdel Hakeem, Aya Kamal, Rania Khattab, Asmaa Abdalla, Laila El Morsi Abou El Fotoh, Abdel Azeem El Mazary, Madiha Abdalla Sayed, Ashraf Mohamed Abdel Fadil
BACKGROUND: Nonalcoholic fatty liver disease (NAFLD) is one of the health problems with great burden on the liver that may end with liver cirrhosis and hepatocellular carcinoma. The aim of this work was to assess serum vitamin D level in nonalcoholic fatty liver disease children. METHODS: This cross sectional case control study involved 47 patients with nonalcoholic fatty liver disease selected while recruiting the pediatric hepatology clinics. Their ages ranged from 5-15 years and were compared with 23 healthy age and sex matched children...
2016: Nutrition & Metabolism
https://www.readbyqxmd.com/read/27529964/-invasive-campylobacter-jejuni-coli-infections-9-case-reports-at-a-single-center-between-2000-and-2015-and-a-review-of-literature-describing-japanese-patients
#20
Keiji Tasaka, Kousaku Matsubara, Hiroyuki Nigami, Aya Iwata, Kenichi Isome, Go Yamamoto
There have been few coherent reports on extraintestinal infection or bacteremia caused by Campylobacter jejuni (C. jejuni) or C. coli in Japan. To clarify the clinical and microbiological characteristics of invasive infections caused by these two species, we retrospectively analyzed the records of patients from whom these pathogens had been isolated from sterile sites between 2000 and 2015. During this study period, we identified 9 patients. The clinical syndrome of all of these patients was bacteremia. Three patients had underlying diseases with both liver cirrhosis and malignant neoplasm, and all of these patients were aged 60 years or older...
May 2016: Kansenshōgaku Zasshi. the Journal of the Japanese Association for Infectious Diseases
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