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Cirrhosis in children

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https://www.readbyqxmd.com/read/28532447/clinical-characteristics-of-disseminated-cryptococcosis-in-previously-healthy-children-in-china
#1
Li-Wei Gao, An-Xia Jiao, Xi-Rong Wu, Shun-Ying Zhao, Yun Ma, Gang Liu, Ju Yin, Bao-Ping Xu, Kun-Ling Shen
BACKGROUND: Disseminated cryptococcosis is a rare and fatal disease, and limited data exist regarding it in children. This study aimed to investigate the clinical characteristics of disseminated cryptococcosis in previously healthy children in China. METHODS: Hospitalized patients with disseminated cryptococcosis were enrolled during January 1996 to December 2015 in Beijing Children's Hospital, Capital Medical University, China. Data on clinical manifestations, laboratory tests, treatment, and prognosis were evaluated...
May 22, 2017: BMC Infectious Diseases
https://www.readbyqxmd.com/read/28515029/tolvaptan-utilization-in-children-with-chronic-hyponatremia-due-to-inappropriate-antidiuretic-hormone-secretion-siadh-three-case-reports-and-review-of-the-literature
#2
Gerdi Tuli, Daniele Tessaris, Luisa De Sanctis, Patrizia Matarazzo
Hyponatremia is the most common electrolyte disorder among hospitalized patients and it is sometimes considered as a poor outcome predictor. Its correction is thus indicated, even in asymptomatic patients. The conventional treatment is represented by fluid restriction in presence of euvolemia or hypervolemia; whereas loop diuretics are used in some hypervolemic conditions (cardiac heart failure, liver cirrhosis and nephrotic syndrome) and intravenous isotonic or hypertonic solution are administered in hypovolemic conditions...
May 17, 2017: Journal of Clinical Research in Pediatric Endocrinology
https://www.readbyqxmd.com/read/28507928/the-role-of-direct-acting-antivirals-in-the-treatment-of-children-with-chronic-hepatitis-c
#3
REVIEW
Christine Hong Ting Yang, Eric R Yoo, Aijaz Ahmed
In the United States, chronic infection with the hepatitis C virus (HCV) affects an estimated 0.1-2% of the pediatric population, who are consequently at risk for major complications, including cirrhosis, hepatocellular carcinoma, and death. The current standard of treatment for chronic hepatitis C (CHC) in children is pegylated-interferon-alpha (PEG-IFN) in combination with ribavirin. PEG-IFN/ribavirin therapy is approved for children ages 3 and older; however, it is often held from use until adulthood because of its extensive list of potential side effects and high likelihood of causing adverse symptoms...
March 28, 2017: Journal of Clinical and Translational Hepatology
https://www.readbyqxmd.com/read/28504497/liver-disease-and-dyslipidemia-as-a-manifestation-of-lysosomal-acid-lipase-deficiency-lal-d-clinical-and-diagnostic-aspects-and-a-new-treatment-an-update
#4
Luisa Bay, Cristina Canero Velasco, Mirta Ciocca, Andrea Cotti, Miriam Cuarterolo, Alejandro Fainboim, Eduardo Fassio, Marcela Galoppo, Federico Pinero, Paula Rozenfeld
Lysosomal acid lipase deficiency (LAL-D) is still a little recognized genetic disease with significant morbidity and mortality in children and adults. This document provides guidance on when to suspect LAL-D and how to diagnose it. It is recommended to add lysosomal acid lipase deficiency to the List of differential diagnoses of sepsis, oncological diseases, storage diseases, persistent diarrhea, chronic malnutrition, and hemophagocytic lymphohistiocytosis. It should also be considered in young patients with dyslipidemia and atherosclerosis as well as diseases associated with fatty liver and/or hepatomegaly...
June 1, 2017: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/28502444/nonalcoholic-fatty-liver-disease-in-children-hepatic-and-extrahepatic-complications
#5
REVIEW
Praveen Kumar Conjeevaram Selvakumar, Mohammad Nasser Kabbany, Valerio Nobili, Naim Alkhouri
Nonalcoholic fatty liver disease (NAFLD) is considered the hepatic manifestation of metabolic syndrome and has become the most common form of chronic liver disease in children and adolescents. The histologic spectrum of NAFLD is broad ranging, from the relatively benign form of simple steatosis to the aggressive form of nonalcoholic steatohepatitis, eventually leading to fibrosis and cirrhosis. NAFLD has also been recognized as an independent risk factor for extrahepatic complications, such as cardiovascular disease, type 2 diabetes mellitus, sleep disorders, and osteoporosis...
June 2017: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/28497759/long-term-challenges-and-perspectives-of-pre-adolescent-liver-disease
#6
REVIEW
Nedim Hadžić, Ulrich Baumann, Pat McKiernan, Valerie McLin, Valerio Nobili
Chronic liver disease is a growing problem that has substantial effects on public health. Many paediatric liver conditions are precursors of adult chronic liver disease, cirrhosis, and hepatocellular carcinoma. Clinical management of Wilson's disease, autoimmune liver disease, and chronic biliary disorders, such as biliary atresia, which remains the most common paediatric chronic liver disease and indication for liver transplantation, is similar in children and adults. In the past 10 or so years, paediatric hepatology has expanded into neighbouring clinical areas, such as metabolic liver diseases and systemic conditions with liver involvement...
June 2017: Lancet. Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28489830/-clinical-and-laboratory-characteristics-of-patients-with-pulmonary-hypertension-and-pulmonary-vascular-complications-hospitalized-at-the-instituto-nacional-de-salud-del-ni%C3%A3-o
#7
Alexis Jose Ormeño Julca, Carlos Melchor Alvarez Murillo, Pedro Miguel Amoretti Alvino, Angel Aladino Florian Florian, Rosa Aurora Castro Johanson, Maria Danisa Celi Perez, Olga Rocío Huamán Prado
The hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PPHN) are distinct pulmonary vascular complications of portal hypertension (PHT) and are associated with increased morbidity and mortality. OBJECTIVES: To describe the clinical and laboratory characteristics of patients with pulmonary hypertension and pulmonary vascular complications hospitalized at the Instituto Nacional de Salud del Niño. MATERIALS AND METHODS: We included patients with HTP hospitalized from January 2012 to June 2013 and that during its evolution progressed with SHP or HTPP...
January 2017: Revista de Gastroenterología del Perú: órgano Oficial de la Sociedad de Gastroenterología del Perú
https://www.readbyqxmd.com/read/28473309/hepatitis-c-virus-in-children-the-global-picture
#8
REVIEW
Etienne Sokal, Pilar Nannini
Chronic hepatitis C virus (HCV) infection is a major cause of cirrhosis, hepatocellular carcinoma and end-stage liver disease. One hundred thirty million to 150 million people have chronic HCV infection, among them 11 million are younger than 15 years of age. This review summarises the epidemiology and characteristics of HCV infection in children, and highlights the role of the new upcoming therapies in HCV-related liver complications.
May 4, 2017: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/28468285/immune-tolerant-chronic-hepatitis-b-the-unrecognized-risks
#9
REVIEW
Patrick T F Kennedy, Samuel Litwin, Grace E Dolman, Antonio Bertoletti, William S Mason
Chronic infection with hepatitis B virus (HBV) progresses through multiple phases, including immune tolerant, immune active, immune control, and, in a subset of patients who achieve immune control, reactivation. The first, the immune tolerant phase, is considered to be prolonged in duration but essentially benign in nature, lacking long-term consequences, and thus not recommended for antiviral therapy. This review challenges the notion that the immune tolerant phase is truly benign and considers the possibility that events during this phase may contribute significantly to cirrhosis, hepatocellular carcinoma (HCC), and the premature death of 25% of HBV carriers worldwide...
April 29, 2017: Viruses
https://www.readbyqxmd.com/read/28452094/imaging-characteristics-of-liver-langerhans-cell-histiocytosis-in-children
#10
Hui Liu, Xueying Long, Xiaoyi Wang, Wenzheng Li, Yigang Pei
BACKGROUND: Hepatic involvement of Langerhans cell histiocytosis (LCH) in children is important not only for prognostic purposes, but also as a guide for therapeutic strategies. The purpose of this study was to evaluate the imaging characteristics of hepatic LCH by computed tomography (CT) and magnetic resonance imaging (MRI) in children. METHODS: Imaging features of 17 children with hepatic LCH diagnosed by physical examination, laboratory and histopathological tests were analyzed retrospectively...
April 28, 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/28446162/prevalence-and-risk-factors-for-non-alcoholic-fatty-liver-in-children-and-youth-with-obesity
#11
Carolina Jimenez-Rivera, Stasia Hadjiyannakis, Jorge Davila, Julie Hurteau, Mary Aglipay, Nick Barrowman, Kristi B Adamo
BACKGROUND: Non- Alcoholic Fatty Liver (NAFL) is a spectrum of liver diseases (LD) that ranges from benign fatty infiltration of the liver to cirrhosis and hepatic failure. Hepatic ultrasound (US) and serum alanine aminotransferase (ALT) are often used as markers of NAFL. Our aim is to describe prevalence of NAFL and associated findings on ultrasound (US) and biochemical parameters in a population of children and adolescents with obesity at the Children's Hospital of Eastern Ontario. METHODS: Children with Obesity (BMI >95th percentile) ages 8-17 years presenting to the Endocrinology and Gastroenterology clinics, without underlying LD were prospectively recruited from 2009 to 2012...
April 26, 2017: BMC Pediatrics
https://www.readbyqxmd.com/read/28444987/platelet-count-spleen-length-and-platelet-count-to-spleen-length-ratio-for-the-diagnosis-of-oesophageal-varices-in-people-with-chronic-liver-disease-or-portal-vein-thrombosis
#12
REVIEW
Agostino Colli, Juan Cristóbal Gana, Jason Yap, Thomasin Adams-Webber, Natalie Rashkovan, Simon C Ling, Giovanni Casazza
BACKGROUND: Current guidelines recommend screening of people with oesophageal varices via oesophago-gastro-duodenoscopy at the time of diagnosis of hepatic cirrhosis. This requires that people repeatedly undergo unpleasant invasive procedures with their attendant risks, although half of these people have no identifiable oesophageal varices 10 years after the initial diagnosis of cirrhosis. Platelet count, spleen length, and platelet count-to-spleen length ratio are non-invasive tests proposed as triage tests for the diagnosis of oesophageal varices...
April 26, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28437324/noncirrhotic-portal-fibrosis-in-pediatric-population
#13
Vikrant Sood, Bikrant B Lal, Rajeev Khanna, Dinesh Rawat, Chhagan Bihari, Seema Alam
OBJECTIVES: Noncirrhotic 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 were evaluated...
May 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28436333/epidemiology-of-hepatitis-c-infection-in-pakistan-current-estimate-and-major-risk-factors
#14
Aiman Arshad, Usman Ali Ashfaq
In Pakistan, hepatitis C virus (HCV) is a major healthcare problem, with acute and chronic infections responsible for liver damage, cirrhosis, and hepatocellular carcinoma. Under the Human Development Index of the United Nations, Pakistan is ranked 134th of 174 countries due to its poor educational and health standards. This study was designed to study HCV and its genotype prevalence in different cities and provinces of Pakistan and describe the major routes of HCV transmission. Literature searches were performed in PubMed, Mendeley, and Google Scholar...
2017: Critical Reviews in Eukaryotic Gene Expression
https://www.readbyqxmd.com/read/28433100/diagnosis-of-wilson-disease
#15
Peter Ferenci
Clinical presentation of Wilson disease can vary widely; therefore diagnosis is not always straightforward. Wilson disease is not just a disease of children and young adults, but may present at any age. The key features of Wilson disease are liver disease and cirrhosis, neuropsychiatric disturbances, Kayser-Fleischer rings, and acute episodes of hemolysis, often in association with acute liver failure. Diagnosis is particularly difficult in children and in adults presenting with active liver disease. None of the available laboratory tests is perfect and may not be specific for Wilson disease...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/28433098/wilson-disease-in-children
#16
Eve A Roberts, Piotr Socha
Wilson disease (WD) is an inherited disorder mainly of hepatocellular copper disposition, due to dysfunction of the Wilson ATPase, a P1B-ATPase encoded by the gene ATP7B. In children, as in older age brackets, clinical disease is highly diverse. Although hepatic disease is the common presentation in children/adolescents, neurologic, psychiatric, and hematologic clinical presentations do occur. Very young children may have clinically evident liver disease due to WD. Early diagnosis, preferably when the child/adolescent is asymptomatic, is most likely to result in near-normal longevity with generally good health so long as the patient tolerates effective medication, is adherent to the lifelong treatment regimen, and has consistent access to the medication...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/28391883/childhood-adult-onset-lysosomal-acid-lipase-deficiency-a-serious-metabolic-and-vascular-phenotype-beyond-liver-disease-four-new-pediatric-cases
#17
Pierre Poinsot, Sophie Collardeau Frachon, Lioara Restier, André Sérusclat, Mathilde Di Filippo, Sybil Charrière, Philippe Moulin, Alain Lachaux, Noel Peretti
BACKGROUND: The childhood/adult-onset lysosomal acid lipase deficiency (LALD; late-onset LALD) is a rare genetic disease. Children present severe fatty liver disease with early cirrhosis. Before enzyme replacement therapy, statins were the standard treatment to improve the severe dyslipidemia. However, late-onset LALD should be considered as a systemic metabolic disease: chronic hyper-low-density lipoprotein and hypo-high-density lipoprotein cholesterolemia induces early atherosclerosis in addition to the liver morbidity...
January 2017: Journal of Clinical Lipidology
https://www.readbyqxmd.com/read/28367327/assessment-of-immunization-to-hepatitis-b-vaccine-among-children-under-five-years-in-rural-areas-of-taiz-yemen
#18
Fuad A A Alssamei, Najla A Al-Sonboli, Fawzi A Alkumaim, Nader S Alsayaad, Mohammed S Al-Ahdal, Tarig B Higazi, Atif A Elagib
Background. Hepatitis B virus (HBV) infection poses a major health problem worldwide. approximately 1 million deaths annually due to cirrhosis and hepatocellular carcinoma. Objectives. This study was conducted to determine the coverage rate of HBV vaccine and assess the vaccine protective response among children under five years old in rural areas of Yemen. Methods. A cross-sectional study was conducted from January to December 2015 in four districts of countryside Yemen. The target population was children aged from 6 to 59 months...
2017: Hepatitis Research and Treatment
https://www.readbyqxmd.com/read/28357379/hepatitis-b-virus-and-its-sexually-transmitted-infection-an-update
#19
REVIEW
Takako Inoue, Yasuhito Tanaka
incidence and prevalence: About 5% of the world's population has chronic hepatitis B virus (HBV) infection, and nearly 25% of carriers develop chronic hepatitis, cirrhosis, and hepatocellular carcinoma (HCC). The prevalence of chronic HBV infection in human immunodeficiency virus (HIV)-infected individuals is 5%-15%; HIV/HBV coinfected individuals have a higher level of HBV replication, with higher rates of chronicity, reactivation, occult infection, and HCC than individuals with HBV only. The prevalence of HBV genotype A is significantly higher among men who have sex with men (MSM), compared with the rest of the population...
September 5, 2016: Microbial Cell
https://www.readbyqxmd.com/read/28357028/limitations-and-opportunities-of-non-invasive-liver-stiffness-measurement-in-children
#20
REVIEW
Guido Engelmann, Jasmin Quader, Ulrike Teufel, Jens Peter Schenk
Changes in liver structure are an important issue in chronic hepatopathies. Until the end of the 20(th) century, these changes could only be determined by histological analyses of a liver specimen obtained via biopsy. The well-known limitations of this technique (i.e., pain, bleeding and the need for sedation) have precluded its routine use in follow-up of patients with liver diseases. However, the introduction of non-invasive technologies, such as ultrasound and magnetic resonance imaging, for measurement of liver stiffness as an indirect marker of fibroses has changed this situation...
March 18, 2017: World Journal of Hepatology
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