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Cirrhosis in children

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...
October 17, 2016: Cancer Medicine
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
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
Mathieu Duché, Béatrice Ducot, Oanez Ackermann, Florent Guérin, Emmanuel Jacquemin, Olivier Bernard
BACKGROUND AND 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
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
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
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...
September 14, 2016: Nature Reviews. Gastroenterology & Hepatology
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
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...
August 30, 2016: Journal of Pediatrics
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
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
Shuling Chen, Bing Liao, Zhihai Zhong, Yanling Zheng, Baoxian Liu, Quanyuan Shan, Xiaoyan Xie, Luyao Zhou
To explore an effective noninvasive tool for monitoring liver fibrosis of children with biliary atresia (BA) is important but evidences are limited. This study is to investigate the predictive accuracy of supersonic shearwave elastography (SSWE) in liver fibrosis for postoperative patients with BA and to compare it with aspartate aminotransferase to platelet ratio index (APRI) and fibrosis-4 (FIB-4). 24 patients with BA received SSWE and laboratory tests before scheduled for liver biopsy. Spearman rank coefficient and receiver operating characteristic (ROC) were used to analyze data...
2016: Scientific Reports
S S Zhu, Y Dong, L M Wang, Z Q Xu, D W Chen, Y Gan, F C Wang, J G Yan, L L Cao, P Wang, M Zhang, H F Zhang
OBJECTIVE: To investigate the liver pathological characteristics and the clearance rate of hepatitis B surface antigen (HBsAg) with antiviral treatment for 1 to 7 years old children with heptitis B e antigen(HBeAg)-negative chronic hepatitis B. METHOD: A total of 49 cases with HBeAg-negative chronic hepatitis B were treated with interferon (IFN) or IFN treatment for 6 months added with lamivudine, and were followed up for at least 6 months.Retrospective analysis was performed on the liver pathological characteristics, the efficacy of antivirus treatment and its influencing factors of the HBsAg clearance rate in the cases from June 2006 to December 2013 in the 302 Hospital of People's Liberation Army...
August 2016: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
Pamela L Valentino, Shanna Wiggins, Sarah Harney, Roshan Raza, Christine K Lee, Maureen M Jonas
OBJECTIVES: Data regarding pediatric PSC natural history are limited. We describe a large pediatric PSC cohort with longitudinal follow-up. METHODS: Records review of pediatric PSC patients diagnosed between 1984 and 2014. RESULTS: N = 120 (63% M) aged 1-21y (median 14y) at diagnosis. 27% (31/113) had ASC (autoimmune sclerosing cholangitis), 24% had exclusive small duct PSC, METAVIR stage was F3-F4 in 41%. 81% of PSC patients had IBD; most had ulcerative/indeterminate colitis (72/97), typically pancolitis (40/72)...
August 6, 2016: Journal of Pediatric Gastroenterology and Nutrition
Júlio Rocha Pimenta, Alexandre Rodrigues Ferreira, Eleonora Druve Tavares Fagundes, Thaís Costa Nascentes Queiroz, Regiane Aparecida Nascimento Baptista, Eduardo Guimarães de Araújo Moreira, Camilo Brandão de Resende, Paulo Fernando Souto Bittencourt, Simone Diniz Carvalho, José Andrade Franco Neto, Francisco José Penna
OBJECTIVE: Bleeding of esophageal varices is the main cause of morbidity and mortality in children with portal hypertension. It is important to understand the factors related with a bleeding episode to evaluate more effective primary prophylaxis. This study aims to describe the endoscopic and laboratory findings associated with upper gastrointestinal bleeding (UGIB) secondary to esophageal varices. METHOD: A cross-sectional study with 103 children and adolescents with cirrhosis, divided into a group that had experienced an episode of upper UGIB (35 patients) and a group without a history of UGIB (68 patients), was carried out...
August 4, 2016: Journal of Pediatric Gastroenterology and Nutrition
K Venugopal Menon, Tamer Malak Moawad Sorour
BACKGROUND: The prevalence and characteristics of spontaneous spondylodiscitis in the Middle East is unknown. There seems to be an emerging dominance of pyogenic infections over Tuberculosis and Brucellosis. METHODS: Retrospective observational study based on electronic case records of 4 years from a tertiary reference facility in Oman. Case records, microbiology reports, blood reports, imaging studies, histopathology and surgical reports of all spinal infection cases were studied...
August 2, 2016: World Neurosurgery
Mohammed Hasosah, Alaa Bokhari, Ashraf Alsahafi, Ghassan Sukkar, Abdullah Alzaben
Patients with type-1 diabetes are predisposed to develop a spectrum of liver diseases, which includes fatty liver, steatohepatitis and cirrhosis. The association of hepatitis A infection with type-1 diabetes is extremely rare. To the best of our knowledge, our case is the first case report in children. We described a nine year-old girl who had type-1 diabetes and developed hepatitis A virus infection without complications. A review of the literature is also provided.
April 26, 2016: Clinics and Practice
Alison E Butler, Richard A Schreiber, Natalie Yanchar, Sherif Emil, Jean-Martin Laberge
Biliary atresia is the most common cause of end-stage liver disease and liver cirrhosis in children, and the leading indication for liver transplantation in the paediatric population. There is no cure for biliary atresia; however, timely diagnosis and early infant age at surgical intervention using the Kasai portoenterostomy optimize the prognosis. Late referral is a significant problem in Canada and elsewhere. There is also a lack of standardized care practices among treating centres in this country. Biliary atresia registries currently exist across Europe, Asia and the United States...
April 2016: Paediatrics & Child Health
Patrick R Blackburn, Raymond D Hickey, Rebecca A Nace, Nasra H Giama, Daniel L Kraft, Andrew J Bordner, Roongruedee Chaiteerakij, Jennifer B McCormick, Maja Radulovic, Rondell P Graham, Michael S Torbenson, Silvia Tortorelli, C Ronald Scott, Noralane M Lindor, Dawn S Milliner, Devin Oglesbee, Wafa'a Al-Qabandi, Markus Grompe, Dimitar K Gavrilov, Mounif El-Youssef, Karl J Clark, Paldeep S Atwal, Lewis R Roberts, Eric W Klee, Stephen C Ekker
Tyrosinemia type I (TYRSN1, TYR I) is caused by fumarylacetoacetate hydrolase (FAH) deficiency and affects approximately one in 100,000 individuals worldwide. Pathogenic variants in FAH cause TYRSN1, which induces cirrhosis and can progress to hepatocellular carcinoma (HCC). TYRSN1 is characterized by the production of a pathognomonic metabolite, succinylacetone (SUAC) and is included in the Recommended Uniform Screening Panel for newborns. Treatment intervention is effective if initiated within the first month of life...
October 2016: Human Mutation
Maria Inês A Wilasco, Carolina Uribe-Cruz, Daniele Santetti, Bianca Pfaffenseller, Cristina T L Dornelles, Themis R da Silveira
BACKGROUND: The nutritional status in patients with cirrhosis is not so easy to assess properly. Considering the relationship between brain-derived neurotrophic factor (BDNF) and energy homeostasis, the main aim of this study was to evaluate the concentration of BDNF in children and adolescents with cirrhosis due to biliary atresia (BA) and correlate it with their nutritional status. METHODS: Fifty-three children and adolescents with cirrhosis due to BA and 33 healthy controls were enrolled in this study...
2016: Annals of Nutrition & Metabolism
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