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Cirrhosis children

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https://www.readbyqxmd.com/read/28437324/noncirrhotic-portal-fibrosis-in-pediatric-population
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
https://www.readbyqxmd.com/read/28343660/a-pilot-study-of-ultrasound-elastography-as-a-non-invasive-method-to-monitor-liver-disease-in-children-with-short-bowel-syndrome
#9
Daniel Lodwick, Molly Dienhart, Jennifer N Cooper, Bonita Fung, Joseph Lopez, Sally Smith, Patrick Warren, Jane Balint, Peter C Minneci
BACKGROUND: Our objective was to evaluate the accuracy of acoustic radiation force impulse (ARFI) elastography in determining extent of liver fibrosis in pediatric patients with short bowel syndrome (SBS). METHODS: This prospective cohort study included pediatric patients with SBS who underwent ultrasound with ARFI measurements of shear wave speed (SWS) and liver biopsy within 30days of each other between 12/2014-9/2015. The mean and median SWS were compared to the stage of fibrosis...
March 16, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28320214/progression-of-liver-disease-in-children-and-adults-with-lysosomal-acid-lipase-deficiency
#10
Barbara K Burton, Nancy Silliman, Sachin Marulkar
BACKGROUND AND OBJECTIVE: Manifestations of the autosomal recessive disorder lysosomal acid lipase deficiency (LAL-D) include hepatomegaly, elevated serum liver enzymes, and progressive liver disease. We report an analysis of time to progression from first clinical manifestation to first documentation of hepatic fibrosis, cirrhosis, or liver transplantation from an observational study of pediatric and adult patients with LAL-D (clinical trial registration: NCT01528917). METHODS: Data were analyzed from 31 patients with available biopsy data and 1 patient without biopsy data who had undergone liver transplantation...
March 21, 2017: Current Medical Research and Opinion
https://www.readbyqxmd.com/read/28302000/expanded-criteria-for-hepatocellular-carcinoma-in-liver-transplant
#11
Mehmet Haberal, Aydıncan Akdur, Gökhan Moray, Gülnaz Arslan, Figen Özçay, Haldun Selçuk, Handan Özdemir
OBJECTIVES: Hepatocellular carcinoma is the sixth most common cancer worldwide and is the third highest cause of malignancy-related death. Because of its typically late diagnosis, median survival is approximately 6 to 20 months, with 5-year survival of < 12%. Hepatocellular carcinoma typically arises in the background of cirrhosis, with liver transplant regarded as the optimal therapy for selected patients. Initially, orthotopic liver transplant was limited to patients with extensive unresectable tumors, resulting in uniformly dismal outcomes due to high tumor recurrence rates...
March 2017: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/28251116/pediatric-hepatitis-b-treatment
#12
REVIEW
Haruki Komatsu, Ayano Inui, Tomoo Fujisawa
Although the introduction of hepatitis B vaccine has been contributing to the reduction in the prevalence of hepatitis B virus (HBV) carriers worldwide, the treatment of children with chronic HBV infection is a challenge to be addressed. HBeAg seroconversion, which induces low replication of HBV, is widely accepted as the first goal of antiviral treatment in children with chronic hepatitis B. However, spontaneous HBeAg seroconversion is highly expected in children with chronic HBV infection. Therefore, the identification of children who need antiviral treatment to induce HBeAg seroconversion is essential in the management of chronic HBV infection...
February 2017: Annals of Translational Medicine
https://www.readbyqxmd.com/read/28197978/managing-cardiovascular-risk-in-lysosomal-acid-lipase-deficiency
#13
REVIEW
James J Maciejko
Lysosomal acid lipase deficiency (LAL-D) is a rare, life-threatening, autosomal recessive, lysosomal storage disease caused by mutations in the LIPA gene, which encodes for lysosomal acid lipase (LAL). This enzyme is necessary for the hydrolysis of cholesteryl ester and triglyceride in lysosomes. Deficient LAL activity causes accumulation of these lipids in lysosomes and a marked decrease in the cytoplasmic free cholesterol concentration, leading to dysfunctional cholesterol homeostasis. The accumulation of neutral lipid occurs predominantly in liver, spleen, and macrophages throughout the body, and the aberrant cholesterol homeostasis causes a marked dyslipidemia...
February 14, 2017: American Journal of Cardiovascular Drugs: Drugs, Devices, and Other Interventions
https://www.readbyqxmd.com/read/28144387/from-the-liver-to-the-heart-cardiac-dysfunction-in-obese-children-with-non-alcoholic-fatty-liver-disease
#14
EDITORIAL
Anna Di Sessa, Giuseppina Rosaria Umano, Emanuele Miraglia Del Giudice, Nicola Santoro
In the last decades the prevalence of non-alcoholic fatty liver disease (NAFLD) has increased as a consequence of the childhood obesity world epidemic. The liver damage occurring in NAFLD ranges from simple steatosis to steatohepatitis, fibrosis and cirrhosis. Recent findings reported that fatty liver disease is related to early atherosclerosis and cardiac dysfunction even in the pediatric population. Moreover, some authors have shown an association between liver steatosis and cardiac abnormalities, including rise in left ventricular mass, systolic and diastolic dysfunction and epicardial adipose tissue thickness...
January 18, 2017: World Journal of Hepatology
https://www.readbyqxmd.com/read/28138183/spectrum-of-histopathological-diagnosis-in-paediatric-patients-with-liver-disorders-in-pakistan
#15
Syeda Shaheera Hashmi, Abu Bakar Hafeez Bhatti, Munir Iqbal Malik, Atif Rana, Humaira Nasir, Faisal Saud Dar, Ejaz Ahmed Khan
OBJECTIVE: To document spectrum of paediatric liver disorders confirmed on liver biopsy. METHODS: The retrospective review of patients was conducted at Shifa International Hospital, Islamabad, Pakistan, and comprised data of all children who underwent ultrasound-guided percutaneous liver biopsy between December 2008 and June 2015. Frequency of individual diagnosis was assessed and compared on gender basis. SPSS 16 was used for statistical analysis. RESULTS: There were medical records of 74 patients in the study with an overall median age of 6...
February 2017: JPMA. the Journal of the Pakistan Medical Association
https://www.readbyqxmd.com/read/28089231/-mother-to-child-transmission-of-hepatitis-b-virus-despite-postexposure-prophylaxis-a-review-of-the-literature-and-description-of-11%C3%A2-observations
#16
B Biot, N Laverdure, F Lacaille, A Lachaux
Chronic hepatitis B virus (HBV) infection leads to a risk of developing cirrhosis and hepatocellular carcinoma. In France, where the prevalence of HBV is low, mother-to-child transmission is the cause of chronic infection in more than one-third of cases. After exposure, the risk of chronic infection is the highest for newborns (90 %). The World Health Organization implemented a global immunization program in 1991, applied in France in 1994. A significant number of children are infected each year, however, and failure of postexposure prophylaxis is reported in 4-10 % of newborns...
February 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28079234/evaluation-of-endoscopic-secondary-prophylaxis-in-children-and-adolescents-with-esophageal-varices
#17
Júlio Rocha Pimenta, Alexandre Rodrigues Ferreira, Eleonora Druve Tavares Fagundes, Paulo Fernando Souto Bittencourt, Alice Mendes Moura, Simone Diniz Carvalho
BACKGROUND: - Bleeding of esophageal varices is the main cause of morbidity and mortality in children and adults with portal hypertension and there are few studies involving secondary prophylaxis in children and adolescents. OBJECTIVE: - To evaluate the efficacy of endoscopic secondary prophylaxis in prevention of upper gastrointestinal bleeding in children and adolescents with esophageal varices. METHODS: - This is a prospective analysis of 85 patients less than 18 years of age with or without cirrhosis, with portal hypertension...
January 2017: Arquivos de Gastroenterologia
https://www.readbyqxmd.com/read/27997679/the-safety-and-effectiveness-of-ledipasvir-sofosbuvir-in-adolescents-12-to-17-years-old-with-hepatitis-c-virus-genotype-1-infection
#18
William F Balistreri, Karen F Murray, Philip Rosenthal, Sanjay Bansal, Lin Chuan-Hao, Kathryn Kersey, Benedetta Massetto, Yanni Zhu, Bittoo Kanwar, Polina German, Evguenia Svarovskaia, Diana M Brainard, Jessica Wen, Regino P Gonzalez-Peralta, Maureen M Jonas, Kathleen Schwarz
No all-oral, direct-acting antiviral regimens have been approved for children with chronic hepatitis C virus (HCV) infection. We conducted a Phase 2, multi-center, open-label study to evaluate the efficacy and safety of ledipasvir-sofosbuvir in adolescents with chronic HCV genotype 1 infection. One hundred patients ages 12 to 17 years received a combination tablet of 90 mg ledipasvir and 400 mg sofosbuvir once daily for 12 weeks. On the 10(th) day following initiation of dosing, 10 patients underwent an intensive pharmacokinetic evaluation of the concentrations of sofosbuvir, ledipasvir, and the sofosbuvir metabolite GS-331007...
December 20, 2016: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/27975111/an-overview-of-cirrhosis-in-children
#19
REVIEW
Jonathan Cordova, Hilary Jericho, Ruba K Azzam
Cirrhosis is the end result of nearly all forms of progressive liver disease. The diffuse hepatic process can be characterized as a state of inflammation progressing to fibrosis and resulting in nodular regeneration, ultimately leading to disorganized liver architecture and function. The underlying etiology of cirrhosis in children may often differ from adults owing to specific disease processes that manifest in childhood, including biliary atresia, galactosemia, and neonatal hepatitis. Although basic management strategies in children are similar to those in adults, the care given to children with cirrhosis must keep the child's growth and development of paramount importance...
December 1, 2016: Pediatric Annals
https://www.readbyqxmd.com/read/27975110/viral-hepatitis-in-children-a-through-e
#20
REVIEW
Ankur Chugh, Maryann Maximos, Meryl Perlman, Regino P Gonzalez-Peralta
Hepatitis is defined as inflammation of the liver. This inflammation can be acute and self-limited, chronic (leading to cirrhosis and an increased risk for hepatocellular carcinoma), or fulminant (requiring lifesaving liver transplantation). Although there are many causes of hepatitis, this article focuses on the main childhood viral hepatidities: types A, B, C, D, and E. This review discusses the main characteristics of each virus, including salient epidemiology, clinical characteristics, diagnosis, treatment, and prevention strategies...
December 1, 2016: Pediatric Annals
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