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Chronic liver disease children

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
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
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
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
Sylva Skalova, Stepan Kutilek
Transient hyperphosphatasemia of infancy and early childhood (THI) is characterized by transiently increased activity of serum alkaline phosphatase (S-ALP), predominantly its bone or liver isoform, in children under five years of age. There are no signs of metabolic bone disease or hepatopathy corresponding with the increased S-ALP. THI is benign disorder, rather laboratory than clinical disorder, which is usually accidentally detected in both healthy and sick children. When encountered in a child with either chronic bone, liver or kidney disease, it might concern the physician...
July 2016: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
Sofía Verdaguer D, Juan Cristóbal Gana A
BACKGROUND: There is a paucity of good quality research about the diagnosis of esophageal varices and the prophylaxis and treatment of variceal bleeding in pediatric patients with portal hypertension There is little consensus and practically no evidence-based approach about the management of these patients. AIM: To describe the behavior and preferences of pediatric gastroenterologists in Chile in the management of portal hypertension in children. MATERIAL AND METHODS: An online survey was sent to Chilean pediatric gastroenterologists, with questions evaluating the physicians’ approaches to screening of esophageal varices in children with portal hypertension, and their preferred methods of prophylaxis and initial management of variceal bleeding...
July 2016: Revista Médica de Chile
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
Ryan W Himes, Sarah E Barlow, Kevin Bove, Norma M Quintanilla, Rachel Sheridan, Rohit Kohli
Lysosomal acid lipase deficiency (LAL-D) is a classic lysosomal storage disorder characterized by accumulation of cholesteryl ester and triglyceride. Although it is associated with progressive liver injury, fibrosis, and end-stage liver disease in children and adolescents, LAL-D frequently presents with nonspecific signs that overlap substantially with other, more common, chronic conditions like nonalcoholic fatty liver disease (NAFLD), metabolic syndrome, and certain inherited dyslipidemias. We present 2 children with NAFLD who achieved clinically significant weight reduction through healthy eating and exercise, but who failed to have the anticipated improvements in aminotransferases and γ-glutamyl transferase...
September 13, 2016: Pediatrics
Hartono Gunardi, Melanie Y Iskandar, Turyadi, Susan I Ie, Pramita G Dwipoerwantoro, Rino A Gani, David H Muljono
BACKGROUND: HBV-infected patients are potential sources of intra-familial transmission. We studied HBV transmission and molecular characteristics within families of HBV-related chronic liver disease (CLD) patients. METHODS: Family members [index cases (ICs), spouses, and 1-18-year-old children] of HBV-related CLD patients were tested for HBsAg, anti-HBc, and anti-HBs. HBsAg-positive subjects were tested for HBeAg/anti-HBe. Anti-HBc-positive children together with their family members were further investigated for HBV DNA...
September 13, 2016: Hepatology International
Alexis Strickler, Natalia Vásquez, Leonardo Maggi, Juvenal Hernández, Ximena Hidalgo
Human toxocarosis is a chronic larval parasitosis listed as one of the five most important neglected diseases by the CDC. The larvae can spread systemically and migrate to different tissues including liver and gallbladder. Acalculous acute cholecystitis (AAC) is a rare disease in children. The diagnosis is based on clinical parameters and imaging criteria. It has been reported in relation to sepsis, shock, trauma, burns, severe systemic diseases, congenital anomalies, infections and also in healthy children...
June 2016: Revista Chilena de Infectología: órgano Oficial de la Sociedad Chilena de Infectología
Antonella Mosca, Claudia Della Corte, Maria Rita Sartorelli, Francesca Ferretti, Francesco Nicita, Andrea Vania, Valerio Nobili
Non-alcoholic fatty liver disease (NAFLD) is the most common chronic liver disease in children and adolescents, due to the increased worldwide incidence of obesity among children. It is now clear enough that of diet high in carbohydrates and simple sugars are associated with hepatic steatosis and non-alcoholic steatohepatitis (NASH). Several studies have shown that an increased consumption of simple sugars is also positively associated with overweight and obesity, and related co-morbidities, such as type 2 diabetes, metabolic syndrome and NAFLD...
August 26, 2016: Eating and Weight Disorders: EWD
Alice Thébaut, Dalila Habes, Frédéric Gottrand, Christine Rivet, Joseph Cohen, Dominique Debray, Emmanuel Jacquemin, Emmanuel Gonzales
BACKGROUNDS/AIM: Pruritus is a severe symptom accompanying chronic cholestasis. It can be debilitating and difficult to control. In children, first-line treatments are ursodeoxycholic acid and rifampicin. Refractory pruritus may require invasive therapies including liver transplantation. Clinical trials based on small samples of adult patients suggest that serotonin reuptake inhibitors can improve pruritus in cholestatic or uremic disease. We performed a prospective, multicenter study to assess efficiency and safety of the serotonin reuptake inhibitor sertraline in treating children with refractory cholestatic pruritus...
August 24, 2016: Journal of Pediatric Gastroenterology and Nutrition
D H Leung, A Narang, C G Minard, G Hiremath, J A Goss, R Shepherd
Young children <2 years of age with chronic end-stage liver disease (YC2) are a uniquely vulnerable group listed for liver transplantation, characterized by a predominance of biliary atresia (BA). To investigate wait-list mortality, associated risk factors and outcomes of YC2, we evaluated UNOS registry data from April 2003-March 2013 for YC2 listed for deceased donor transplant (BA=994, other chronic liver disease, CLD=221). Overall, wait-list mortality among YC2 was 12.4% and post-transplant mortality was 8%, accounting for an overall post-listing mortality of 19...
August 19, 2016: Liver Transplantation
Vratislav Smolka, Eva Karaskova, Oksana Tkachyk, Kvetoslava Aiglova, Jiri Ehrmann, Kamila Michalkova, Michal Konecny, Jana Volejnikova
BACKGROUND: Sclerosing cholangitis (SC) is a chronic cholestatic hepatobiliary disease with uncertain long-term prognosis in pediatric patients. This study aimed to evaluate long-term results in children with SC according to the types of SC. METHODS: We retrospectively followed up 25 children with SC over a period of 4-17 years (median 12). The diagnosis of SC was based on biochemical, histological and cholangiographic findings. Patients fulfilling diagnostic criteria for probable or definite autoimmune hepatitis at the time of diagnosis were defined as having autoimmune sclerosing cholangitis (ASC); other patients were included in a group of primary sclerosing cholangitis (PSC)...
August 2016: Hepatobiliary & Pancreatic Diseases International: HBPD INT
Behairy E Behairy, Hanaa A El-Araby, Hasan H Abd El Kader, Nermin A Ehsan, Menan E Salem, Haidy M Zakaria, Mohammed A Khedr
UNLABELLED:  Background. T-cell populations regulate the balance of immune responses. The CD (Cluster of differentiation) 4+CD25+ regulatory T cells (Tregs) are crucial for maintaining negative control of various immune responses. There are different T-cell subpopulations with regulatory functions, as natural killer T cells, CD8+ and CD28. The forkhead box P3 (FOXP3) regulates Treg development and is required for its suppressive function. AIM: To evaluate the hepatic expression of the intrahepatic Tregs, Ig (immunoglobulin) G and IgM plasma cells in autoimmune hepatitis (AIH) and other chronic liver diseases (CLDs)...
September 2016: Annals of Hepatology
Phillip C Bost, Mark J Strynar, Jessica L Reiner, Jerry A Zweigenbaum, Patricia L Secoura, Andrew B Lindstrom, Janice A Dye
: Perfluoroalkyl substances (PFAS), such as perfluorooctane sulfonate (PFOS) and perfluorooctanoic acid (PFOA), are persistent, globally distributed, anthropogenic compounds. The primary source(s) for human exposure are not well understood although within home exposure is likely important since many consumer products have been treated with different PFAS, and people spend much of their lives indoors. Herein, domestic cats were used as sentinels to investigate potential exposure and health linkages...
July 29, 2016: Environmental Research
Archie Ramaswami, Danya J Rosen, Jaime Chu, Birte Wistinghausen, Ronen Arnon
Deferesirox (DFX), an oral chelating agent, is used to treat chronic iron overload in several hematological diseases such as β-thalassemia, sickle cell disease, and myelodysplastic anemia. DFX is generally well tolerated with the exception of gastrointestinal disturbances and rash, although cases of renal toxicity, as well as acute and chronic liver failure, have been reported in adults and children. Here we describe a 3-year-old girl with β-thalassemia undergoing treatment with DFX who presented with acute liver failure and Fanconi's syndrome...
July 29, 2016: Journal of Pediatric Hematology/oncology
Kimberly P Newton, Jiayi Hou, Nancy A Crimmins, Joel E Lavine, Sarah E Barlow, Stavra A Xanthakos, Jonathan Africa, Cynthia Behling, Michele Donithan, Jeanne M Clark, Jeffrey B Schwimmer
Importance: Nonalcoholic fatty liver disease (NAFLD) is a major chronic liver disease in children in the United States and is associated with insulin resistance. In adults, NAFLD is also associated with type 2 diabetes. To our knowledge, the prevalence of type 2 diabetes in children with NAFLD is unknown. Objective: To determine the prevalence of type 2 diabetes and prediabetes in children with NAFLD and assess type 2 diabetes and prediabetes as risk factors for nonalcoholic steatohepatitis (NASH)...
October 3, 2016: JAMA Pediatrics
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