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Cirrhosis in children

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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
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
https://www.readbyqxmd.com/read/27966327/effect-of-methylprednisolone-injection-on-interleukin-4-and-interferon-gamma-expression-following-hepatitis-b-vaccination-in-mice
#13
Peter Ughachukwu, Paul Okonkwo, Prince Unekwe, John Ogama
BACKGROUND/AIM: The prevalence of hepatitis B infection is high worldwide with liver cirrhosis and hepatocellular carcinoma as important complications. Immunosuppression, especially from corticosteroids, is often cited as a cause of poor immune response and there is documented evidence of irrational administration of glucocorticoids to children and adults. Decreased expression of interleukin-4 and interferon-gamma is an indication of poor humoral and cellular immune responses, respectively...
November 17, 2016: Turkish Journal of Medical Sciences
https://www.readbyqxmd.com/read/27957245/can-platelet-count-spleen-diameter-ratio-be-used-for-cirrhotic-children-to-predict-esophageal-varices
#14
Oya Balci Sezer, Deniz Çelik, Nihal Tutar, Figen Özçay
AIM: To determine the laboratory and radiologic parameters, including the platelet count (PC)-to-spleen diameter (SD) ratio as a non-invasive marker that may predict the presence of esophageal varices (EV) in children with cirrhosis. METHODS: Eighty-nine patients with cirrhosis, but without a history of variceal bleeding were prospectively included. The children were grouped into 6-12 and 12-18 years of age groups. These groups were also divided into 2 sub-groups (presence and absence of EV)...
November 28, 2016: World Journal of Hepatology
https://www.readbyqxmd.com/read/27941191/diagnostic-and-therapeutic-management-of-children-with-lysosomal-acid-lipase-deficiency-lal-d-review-of-the-literature-and-own-experience
#15
Aldona Wierzbicka-Rucińska, Wojciech Jańczyk, Agnieszka Ługowska, Dariusz Lebensztejn, Piotr Socha
Lysosomal acid lipase deficiency may present at any age (in infants, children and adults). Its presenting features commonly include elevated serum transaminase activity levels, hypercholesterolemia, fatty liver, progressive liver fibrosis, and cirrhosis. Nonspecific clinical manifestations can lead to a delay in the diagnosis of both children and adults. The early development of fibrosis and cirrhosis suggests that the lysosomal accumulation of cholesterol esters and triglycerides in the liver is a potent inducer of fibrosis...
2016: Developmental Period Medicine
https://www.readbyqxmd.com/read/27938558/-a-retrospective-study-on-hbsag-clearance-rate-after-antiviral-therapy-in-children-with-hbeag-positive-chronic-hepatitis-b-aged-1-7-years
#16
S S Zhu, Y Dong, Z Q Xu, L M Wang, D W Chen, Y Gan, F C Wang, J G Yan, L L Cao, P Wang, H F Zhang
Objective: To investigate the HBsAg clearance rate after antiviral therapy in children with HBeAg-positive chronic hepatitis B (CHB) aged 1-7 years. Methods: A retrospective analysis was performed for the HBsAg clearance rate in 293 children who were hospitalized in 302 Hospital of PLA from June 2006 to December 2013, met the inclusion criteria, received antiviral therapy, and were followed up for at least 6 months after the withdrawal of antiviral therapy. The t-test or the rank sum test was applied according to the distribution of continuous data, and the chi-square test was used for comparison of categorical data...
October 20, 2016: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://www.readbyqxmd.com/read/27935710/quantification-of-atp7b-protein-in-dried-blood-spots-by-peptide-immuno-srm-as-a-potential-screen-for-wilson-s-disease
#17
Sunhee Jung, Jeffrey R Whiteaker, Lei Zhao, Han-Wook Yoo, Amanda G Paulovich, Si Houn Hahn
Wilson's Disease (WD), a copper transport disorder caused by a genetic defect in the ATP7B gene, has been a long time strong candidate for newborn screening (NBS), since early interventions can give better results by preventing irreversible neurological disability or liver cirrhosis. Several previous pilot studies measuring ceruloplasmin (CP) in infants or children showed that this marker alone was insufficient to meet the universal screening for WD. WD results from mutations that cause absent or markedly diminished levels of ATP7B...
December 9, 2016: Journal of Proteome Research
https://www.readbyqxmd.com/read/27931582/liver-transplantation-for-hepatitis-b-in-early-adulthood-analysis-of-the-united-network-for-organ-sharing-database
#18
G Rifai, A Anani, I A Hanouneh, B Mohamad, A Matloob, R Lopez, N N Zein, N Alkhouri
BACKGROUND: Chronic hepatitis B virus (HBV) infection has a mild course in most children that may delay initiation of treatment even when indicated. Unfortunately, a small number of cases can progress rapidly to cirrhosis, which may require liver transplantation (LT) in early adulthood. The aim of this study was to assess the characteristics of HBV-positive young adults who received LT and to evaluate post-transplant outcomes including patient and graft survival and differences between pre- and post-implementation of Model for End-stage Liver Disease (MELD) prioritization...
December 2016: Transplantation Proceedings
https://www.readbyqxmd.com/read/27931577/liver-transplantation-in-polish-children-with-%C3%AE-1-antitrypsin-deficiency-a-single-center-experience
#19
A Bakula, J Pawlowska, O Niewiadomska, I Jankowska, M Teisseyre, P Kaliciński, P Socha
BACKGROUND: α1-Antitrypsin deficiency (ATD) is the most common genetic cause of liver injury in young children. Asymptomatic hepatitis is observed in most patients. However, the course of liver disease due to ATD is unpredictable, and some children develop liver cirrhosis. Liver transplantation (Ltx) dramatically improves their outcome and in some cases is required in the first years of life. The aim of the study was to evaluate the course of the disease in children with ATD treated with Ltx in a single center...
December 2016: Transplantation Proceedings
https://www.readbyqxmd.com/read/27906797/non-cirrhotic-portal-fibrosis-in-pediatric-population
#20
Vikrant Sood, Bikrant Bihari Lal, Rajeev Khanna, Dinesh Rawat, Chhagan Bihari Sharma, Seema Alam
BACKGROUND: Non-cirrhotic 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 was evaluated...
November 30, 2016: Journal of Pediatric Gastroenterology and Nutrition
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