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Liver disease in children

R Jin, A Krasinskas, N-A Le, J V Konomi, J Holzberg, R Romero, M B Vos
BACKGROUND: Plasminogen activator inhibitor-1 (PAI-1) is the primary inhibitor of the endogenous fibrinolytic system and is known to be increased in obesity, insulin resistance and non-alcoholic fatty liver disease (NAFLD). We previously demonstrated that PAI-1 levels were closely related to the amount of hepatic steatosis in children. OBJECTIVES: The aim of this study was to characterize plasma PAI-1 in relationship to severity of inflammation and fibrosis, as well as to plasma lipids in children with NAFLD...
October 20, 2016: Pediatric Obesity
Abdulmoein E Al-Agha, Abrar M Alnawab, Tala M Hejazi
To determine the various etiologies of primary and secondary hyperlipidemia among children visiting the pediatric endocrine clinic. Methods: This is a retrospective, cross-sectional, cohort study conducted at King Abdulaziz University Hospital (KAUH), Jeddah, Kingdom of Saudi Arabia from January 2010 to 2015 that included 253 children aged from birth to 12 years old. Data were obtained by reviewing medical reports of patients who presented with hyperlipidemia to the clinic, and their laboratory investigation results using KAUH electronic "Phoenix" system...
November 2016: Saudi Medical Journal
Seung Man Cho, Byung-Ho Choe
This review article was requested by editor-in-chief of this journal as 'pediatric CHB treatment' for the upcoming special issue. The main objective of chronic hepatitis B (CHB) treatment is diminishing the risk of complications related to chronic liver disease. In Asia, there are already some reports about hepatocellular carcinoma (HCC) in hepatitis B virus (HBV) infected children. The key points of treatment in children with CHB infections are selection of which patients to treat and conformation of the optimal therapy time that would reduce viral resistance...
September 2016: Annals of Translational Medicine
E Ylinen, L Salmela, J Peräsaari, T Jaatinen, A Tenca, O Vapalahti, M Färkkilä, H Jalanko, K-L Kolho
AIM: The human leukocyte antigen (HLA) allele and haplotype frequencies of the Finnish population are unique because of the restricted and homogenous gene population. There are no published data on HLA genotype associations in paediatric autoimmune liver diseases in Scandinavia. This study characterised the HLA genotypes of children with autoimmune liver or biliary disease in Finland. METHODS: The study cohort comprised 19 paediatric patients (13 female) aged 3-15 years treated for autoimmune liver or biliary disease at the Children's Hospital, Helsinki University Hospital, between 2000 and 2011 and followed up for 4...
October 19, 2016: Acta Paediatrica
Shivaram Prasad Singh, Pradeep Kumar Padhi, Jimmy Narayan, Ayaskanta Singh, Girish Kumar Pati, Preetam Nath, Prasant Kumar Parida, Sunil Mishra
AIM: The aim of this study is to estimate the socioeconomic impact of alcohol use on patients with alcoholic liver disease (ALD) and their families. METHODS: The demographic and socioeconomic data were collected from hospitalized ALD patients and attendants using a self designed non validated questionnaire and analyzed. RESULTS: Study subjects included 100 consecutive ALD patients (all males). Sixty percent were between 30 and 50 years. Most were married (96 %), literate (63 %), either businessmen (37 %) or employed (30 %) and belonged to middle socioeconomic class...
October 19, 2016: Indian Journal of Gastroenterology: Official Journal of the Indian Society of Gastroenterology
Giovanni Vitale, Giulia Simonetti, Martina Pirillo, Gianfranco Taruschio, Pietro Andreone
Bile Salt Export Pump (BSEP) Deficiency disease, including Progressive Familial Intrahepatic Cholestasis type 2 (PFIC2), is a rare disease, usually leading within the first ten years to portal hypertension, liver failure, hepatocellular carcinoma. Often liver transplantation is needed. Sodium 4-phenylbutyrate (4-PB) seems to be a potential therapeutic compound for PFIC2. Psychiatric side effects in the adolescent population are little known and little studied since the drug used to treat children and infants...
September 2016: Psychiatry Investigation
Takaki Emura, Kenji Hosoda, Shota Harai, Noboru Oyachi, Takeyuki Suzuki, Ken Takada, Koji Kobayashi, Hisatake Ikeda
BACKGROUND: Massive gastrointestinal bleeding in children, mostly caused by esophageal varices secondary to chronic liver disease, is uncommon. Dieulafoy lesion in the gastrointestinal tract is a rare but important cause of gastrointestinal bleeding; massive bleeding from this lesion can be fatal unless adequate treatment is promptly initiated. We report a case of gastric Dieulafoy lesion in a 2-year old successfully treated with endoscopic hemoclipping. CASE PRESENTATION: A 2-year-old Japanese boy was admitted to our department with sudden massive hematemesis...
October 19, 2016: Journal of Medical Case Reports
Emily Etchell, Lauriane Jugé, Alice Hatt, Ralph Sinkus, Lynne E Bilston
Purpose To determine if healthy hepatic mechanical properties differ between pediatric and adult subjects at magnetic resonance (MR) elastography. Materials and Methods Liver shear moduli in 24 healthy pediatric participants (13 children aged 5-14 years [seven boys, six girls] and 11 adolescents aged 15-18 years [six boys, five girls]) and 10 healthy adults (aged 22-36 years [five men, five women]) were obtained with 3-T MR elastography at 28, 56, and 84 Hz. Relationships between shear moduli and age were assessed with Spearman correlations...
October 17, 2016: Radiology
Emanuele Nicastro, Sara Giovannozzi, Paola Stroppa, Anna Paola Callegaro, Alessandra Tebaldi, Claudio Farina, Michele Colledan, Lorenzo DʼAntiga
BACKGROUND: Most pediatric liver transplantation (LT) centers administer long courses of prophylaxis against Cytomegalovirus (CMV) without evidence of benefit and with significant drug exposure and costs. We aimed at evaluating overall outcomes, direct and putative indirect effects of CMV, possible impact of viremia and risk factors for CMV infection in pediatric LT recipients managed with ganciclovir-based preemptive therapy (PET). METHODS: The records of all the children who underwent LT between 2008 and 2014 were retrospectively analyzed...
October 17, 2016: Transplantation
Hilary Jericho, Naire Sansotta, Stefano Guandalini
OBJECTIVE: To evaluate the effectiveness of the GFD on extra-intestinal symptoms in pediatric and adult celiac populations at the University of Chicago (UofC). METHODS: We conducted a retrospective chart review of the UofC Celiac Center clinic charts from January 2002 to October 2014. Demographics, serologic testing, intestinal biopsies, and extra-intestinal symptoms at presentation, 12, 24, and greater than 24 months were recorded. Extra-intestinal symptoms included: abnormal liver enzymes, arthralgia/arthritis, dermatitis herpetiformis (DH), alopecia, fatigue, headache, anemia, stomatitis, myalgias, psychiatric disorders, rashes, seizures, neuropathy, short stature, delayed puberty, osteoporosis and infertility...
October 13, 2016: Journal of Pediatric Gastroenterology and Nutrition
Jacqueline Jossen, Rachel Annunziato, Hee-Sung Kim, Jaime Chu, Ronen Arnon
OBJECTIVES: Autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC) are progressive immune-mediated inflammatory diseases that may require liver transplant (LT). Outcomes in children undergoing LT for these diseases are poorly studied in the Pediatric End Stage Liver Disease (PELD) era. We aimed to characterize the outcome of LT in children with AIH and PSC. METHODS: Children ≤18 years with PSC or AIH who had a first, isolated LT from 2002-2012 were identified from the UNOS database...
October 13, 2016: Journal of Pediatric Gastroenterology and Nutrition
Xiaopeng Zhu, Hua Bian, Xin Gao
Nonalcoholic fatty liver disease (NAFLD) is a globally observed metabolic disease with high prevalence both in adults and children. However, there is no efficient medication available yet. Increased evidence indicates that berberine (BBR), a natural plant product, has beneficial effects on NAFLD, though the mechanisms are not completely known. In this review, we briefly summarize the pathogenesis of NAFLD and factors that influence the progression of NAFLD, and focus on the potential mechanisms of BBR in the treatment of NAFLD...
October 14, 2016: Molecules: a Journal of Synthetic Chemistry and Natural Product Chemistry
Young Hee Joung, Se Hee Park, Ki-Beom Moon, Jae-Heung Jeon, Hye-Sun Cho, Hyun-Soon Kim
Disease prevention through vaccination is considered to be the greatest contribution to public health over the past century. Every year more than 100 million children are vaccinated with the standard World Health Organization (WHO)-recommended vaccines including hepatitis B (HepB). HepB is the most serious type of liver infection caused by the hepatitis B virus (HBV), however, it can be prevented by currently available recombinant vaccine, which has an excellent record of safety and effectiveness. To date, recombinant vaccines are produced in many systems of bacteria, yeast, insect, and mammalian and plant cells...
October 13, 2016: International Journal of Molecular Sciences
Fatemeh Famouri, Zainab Shariat, Mahin Hashemipour, Mojtaba Keikha, Roya Kelishadi
OBJECTIVES: This trial aims to evaluate the effects of some probiotics on sonographic and biochemical non-alcoholic fatty liver disease (NAFLD). METHODS: This randomized triple-blind trial was conducted among 64 obese children with sonographic NAFLD. They were randomly allocated to receive probiotic capsule (containing Lactobacillus acidophilus ATCC B3208, 3 × 10 colony forming units, CFU; Bifidobacterium lactis DSMZ 32269, 6 × 10 CFU; B bifidum ATCC SD6576, 2 × 10 CFU; L rhamnosus DSMZ 21690, 2 × 10 CFU) or placebo for 12 weeks...
October 13, 2016: Journal of Pediatric Gastroenterology and Nutrition
Frank W DiPaola, Kurt R Schumacher, Caren S Goldberg, Joshua Friedland-Little, Aishwarya Parameswaran, Jonathan R Dillman
OBJECTIVES: Assess liver stiffness using ultrasound point shear wave elastography (US P-SWE) in children before and after the Fontan operation. METHODS: Eighteen children undergoing the Fontan operation were prospectively enrolled. Eight US P-SWE measurements were obtained from the right hepatic lobe before surgery, and at multiple postoperative time points. Temporally related inferior vena cava pressure (IVC) data was collected from medical records, when available...
October 17, 2016: European Radiology
Peter Witters, Dominic Hughes, Palaniswamy Karthikeyan, Somashekara Ramakrishna, Mark Davenport, Anil Dhawan, Tassos Grammatikopoulos
BACKGROUND AND AIMS: Variceal haemorrhage can be a life-threatening complication of chronic liver disease in children. There is limited evidence about the optimal prophylactic management and selection criteria of children who will benefit from upper gastrointestinal endoscopy. METHODS: Children presenting in our centre with suspected portal hypertension or gastrointestinal bleeding and undergoing their first oesophagogastroduodenoscopy between 2005-2012 were included...
October 4, 2016: Journal of Pediatric Gastroenterology and Nutrition
Emmanuel Gonzales, Tim Ulinski, Dalila Habes, Georges Deschênes, Véronique Frémeaux-Bacchi, Albert Bensman
BACKGROUND: Rational options for the treatment of end-stage renal disease (ESRD) due to atypical hemolytic uremic syndrome (aHUS) in children are still open to discussion. In the case of human complement factor H (CFH) deficiency, the choice is either kidney transplantation in combination with eculizumab, a humanized anti-C5 monoclonal antibody, or a combined liver-kidney transplantation. CASE-DIAGNOSIS/TREATMENT: A child with a homozygous CFH deficiency underwent a successful liver-kidney transplantation...
October 15, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Max Bolun Liu, Thuy Bui Huong, Xuyen Hoang, Lan Doan, Shauna Trinh, Hoa Pham Anh Nguyen, Hai Thanh Le, Ai-Xuan Holterman
BACKGROUND: Biliary atresia is an idiopathic, neonatal liver disease of the bile ducts. The natural evolution of biliary atresia is known in developed countries. This study describes the clinical course of biliary atresia in Vietnam, a developing country. METHODS: Chart reviews were undertaken of patients treated with or without the Kasai procedure between January 2010 and July 2013 at a children's hospital in Vietnam. RESULTS: Of 287 children with biliary atresia, 149 (52%) were treated without the Kasai procedure and 138 (48%) were treated with the Kasai procedure...
October 12, 2016: Surgery
Riad Lutfi, Kamal Abulebda, Mara E Nitu, Jean P Molleston, Molly A Bozic, Girish Subbarao
Pediatric acute liver failure is rare but life-threatening illness that occurs in children without preexisting liver disease. The rarity of the disease, along with its severity and heterogeneity, presents unique clinical challenges to the physicians providing care for pediatric patients with acute liver failure. In this review, practical clinical approaches to the care of critically ill children with acute liver failure are discussed with an organ system specific approach. The underlying pathophysiological processes, major areas of uncertainty, and approaches to the critical care management of pediatric acute liver failure are also reviewed...
October 12, 2016: Journal of Pediatric Gastroenterology and Nutrition
Sunil V Pawar, Vinay G Zanwar, Ajay S Choksey, Ashok R Mohite, Samit S Jain, Ravindra G Surude, Qais Q Contractor, Pravin M Rathi, Ravi U Verma, Premlata K Varthakavi
:  Background and rationale. Nonalcoholic fatty liver disease (NAFLD) is the most common cause of pediatric liver disease in western countries. Its prevalence in Indian subcontinent is not well studied. MATERIAL AND METHODS: In a school based cross sectional study we have screened overweight and obese children in the age group of 11 to 15 years for NAFLD. Ultrasonography, elevated serum transaminases, fibroscan were used for defining NAFLD. Dietary habits, blood pressure, serum lipid profile, blood counts and insulin resistance were recorded...
November 2016: Annals of Hepatology
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