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Cirrhosis in children

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https://www.readbyqxmd.com/read/28735109/design-and-rationale-for-a-real-world-observational-cohort-of-patients-with-nonalcoholic-fatty-liver-disease-the-target-nash-study
#1
A Sidney Barritt, Norman Gitlin, Samuel Klein, Anna S Lok, Rohit Loomba, Laura Malahias, Margaret Powell, Miriam B Vos, L Michael Weiss, Kenneth Cusi, Brent A Neuschwander-Tetri, Arun Sanyal
Nonalcoholic fatty liver disease (NAFLD) is highly prevalent and can lead to cirrhosis, hepatocellular carcinoma, and end-stage liver disease. NAFLD comprises the spectrum from simple steatosis (nonalcoholic fatty liver, NAFL), to steatosis with inflammation (nonalcoholic steatohepatitis, NASH). Current primary therapy recommended for NAFLD is weight loss induced by lifestyle modification. The difficulty in achieving this has led to robust pharmacological therapy development. While new drugs may show efficacy in selected phase II/III clinical trial populations, their real-world effectiveness is unknown...
July 19, 2017: Contemporary Clinical Trials
https://www.readbyqxmd.com/read/28699603/infants-with-extrahepatic-biliary-atresia-effect-of-follow-up-on-the-survival-rate-at-ege-university-medical-school-transplantation-center
#2
Miray Karakoyun, Maşallah Baran, Caner Turan, Murat Kılıç, Orkan Ergun, Sema Aydoğdu
BACKGROUND/AIMS: Biliary atresia (BA) is the main cause of neonatal cholestasis and the primary reason for infant liver transplants worldwide. It is an obliterative cholangiopathy observed only in children and caused by progressive inflammation and fibrosis of the bile duct. We collaborated with a liver transplantation center to investigate the effects of follow-up in patients with BA. MATERIALS AND METHODS: Medical records of 99 patients who were diagnosed with BA and monitored at our center from 1990 to 2002 (27 patients) and from 2003 to 2015 (72 patients) were analyzed retrospectively...
July 2017: Turkish Journal of Gastroenterology: the Official Journal of Turkish Society of Gastroenterology
https://www.readbyqxmd.com/read/28689548/-non-alcoholic-fatty-liver-disease-in-children-and-adolescents
#3
Jessica Björklund, Tea Lund Laursen, Konstantin Kazankov, Karen Louise Thomsen, Stephen Hamilton-Dutoit, Elisabeth Stenbøg, Henning Grønbæk
Non-alcoholic fatty liver disease (NAFLD) is characterized by liver fat accumulation and non-alcoholic steatohepatitis (NASH) with inflammation and fibrosis, which may lead to cirrhosis also in childhood. NAFLD/NASH in children are related to obesity and the metabolic syndrome, and incidence and prevalence are expected to increase. Children having liver steatosis and elevated liver enzymes are most often asymptomatic, and a liver biopsy is necessary for correct diagnosis and staging. The treatment should focus on lifestyle changes, as pharmacological therapy needs further evaluation...
July 3, 2017: Ugeskrift for Laeger
https://www.readbyqxmd.com/read/28675359/the-romanian-national-program-for-liver-transplantation-852-procedures-in-815-patients-over-17-years-2000-2017-a-continuous-evolution-to-success
#4
Irinel Popescu, Mihnea Ionescu, Vladislav Braşoveanu, Doina Hrehoreţ, Narcis Copca, Cristian Lupaşcu, Florin Botea, Bogdan Dorobanţu, Sorin Alexandrescu, Mihai Grigorie, Emil Matei, Radu Zamfir, Vasile Lungu, Dana Tomescu, Gabriela Droc, Daniela Ungureanu, Ruxandra Fota, Graţiela Manga, Mihai Popescu, Laura Popa, Liana Gheorghe, Speranţa Iacob, Corina Pietrăreanu, Mariana Mihailă, Laurenţiu Mic, Sanda Constantinescu, Cristian Gheorghe, Bogdan Cotruta, Ioana Lupescu, Mugur Grasu, Mirela Boroş, Radu Dumitru, Mihai Toma, Liliana Paslaru, Laura Vlad, Ileana Constantinescu, Ileana Dima, Vlad Herlea, Gabriel Becheanu, Cătălin Pecheanu, Daniela Sasalovici
Background: Liver transplantation (LT) has become an established treatment for end-stage liver disease, with more than 20.000 procedures yearly worldwide. The aim of this study was to analyze the results of Romanian National Program of LT. Methods: Between April 2000 and April 2017, 817 pts received 852 LTs in Romania. Male/female ratio was 487/330, while adult/pediatric ratio was 753/64, with a mean age of 46 years (median 50 yrs; range 7 months - 68 yrs). Main LT indications were HBV cirrhosis (230 pts; 28...
May 2017: Chirurgia
https://www.readbyqxmd.com/read/28627213/nonalcoholic-fatty-liver-disease-nafld-pathophysiology-in-obese-children-and-adolescents-update
#5
Silvana Neves Ferraz de Assunção, Ney Christian Boa Sorte, Crésio Dantas Alves, Patricia S Almeida Mendes, Carlos Roberto Brites Alves, Luciana Rodrigues Silva
OBJECTIVE: Although the nonalcoholic fatty liver disease was first identified in 1980, it presents multifactorial and unclear pathophysiology. In this review, we intend to update the pathophysiological mechanisms of a high morbidity and mortality associated disease that is affecting obese children worldwide. DATA SOURCES: The PubMed and the Cochrane Library databases were used in the search strategy for articles related to nonalcoholic fatty liver disease and published in the last three decades...
June 5, 2017: Nutrición Hospitalaria: Organo Oficial de la Sociedad Española de Nutrición Parenteral y Enteral
https://www.readbyqxmd.com/read/28602606/trends-in-mortality-burden-of-hepatocellular-carcinoma-cirrhosis-and-fulminant-hepatitis-before-and-after-roll-out-of-the-first-pilot-vaccination-program-against-hepatitis-b-in-peru-an-analysis-of-death-certificate-data
#6
Max Carlos Ramírez-Soto, Gutia Ortega-Cáceres, César Cabezas
The first pilot vaccination program against hepatitis B in Peru was implemented in the hyperendemic Abancay province in 1991. To assess the impact of vaccination on mortality rates of hepatitis B-related hepatocellular carcinoma (HCC), cirrhosis, and fulminant hepatitis, we compared mortality trends before (1960-1990) and after (1991-2012) roll-out of the vaccination program, using death certificate data from the Municipalidad Provincial de Abancay. Our results showed that, following program roll-out, the overall mortality rates (per 100,000 population) decreased from 9...
July 5, 2017: Vaccine
https://www.readbyqxmd.com/read/28595203/parameters-of-ventricular-repolarization-in-patients-with-autoimmune-hepatitis
#7
Burak Açar, Mahmut Yüksel, Çağrı Yayla, Özgür Kırbaş, Sefa Ünal, Ahmet Göktuğ Ertem, Koray Demirtaş, Mustafa Kaplan, Meral Akdoğan, Sabite Kaçar, Ertuğrul Kayaçetin, Sinan Aydoğdu
OBJECTIVE: Autoimmune hepatitis (AIH) is a liver disorder that affects both children and adults. It is characterized by inflammatory liver histology, elevated transaminase level, circulating nonorgan-specific autoantibodies, and increased level of immunoglobulin G in the absence of known etiology. Ventricular repolarization has been evaluated using T wave and QT interval measurements in patients with hepatic cirrhosis. Ventricular repolarization may be defined using QT interval, QT dispersion, and T wave measurements...
June 2017: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
https://www.readbyqxmd.com/read/28587303/the-role-of-lipid-and-lipoprotein-metabolism-in-non-alcoholic-fatty-liver-disease
#8
REVIEW
Francesco Massimo Perla, Maurizia Prelati, Michela Lavorato, Daniele Visicchio, Caterina Anania
Due to the epidemic of obesity across the world, nonalcoholic fatty liver disease (NAFLD) has become one of the most prevalent chronic liver disorders in children and adolescents. NAFLD comprises a spectrum of fat-associated liver conditions that can result in end-stage liver disease and the need for liver transplantation. Simple steatosis, or fatty liver, occurs early in NAFLD and may progress to nonalcoholic steatohepatitis, fibrosis and cirrhosis with increased risk of hepatocellular carcinoma. The mechanism of the liver injury in NAFLD is currently thought to be a "multiple-hit process" where the first "hit" is an increase in liver fat, followed by multiple additional factors that trigger the inflammatory activity...
June 6, 2017: Children
https://www.readbyqxmd.com/read/28570148/an-official-american-thoracic-society-workshop-report-obesity-and-metabolism-an-emerging-frontier-in-lung-health-and-disease
#9
Benjamin T Suratt, Niki D J Ubags, Deepa Rastogi, Kelan G Tantisira, Benjamin J Marsland, Irina Petrache, Janice B Allen, Jason H T Bates, Fernando Holguin, Meredith C McCormack, Evangelos D Michelakis, Stephen M Black, Manu Jain, Ana L Mora, Viswanathan Natarajan, Yury I Miller, Michael B Fessler, Konstantin G Birukov, Ross S Summer, Stephanie A Shore, Anne E Dixon
The world is in the midst of an unprecedented epidemic of obesity. This epidemic has changed the presentation and etiology of common diseases. For example, steatohepatitis, directly attributable to obesity, is now the most common cause of cirrhosis in the United States. Type 2 diabetes is increasingly being diagnosed in children. Pulmonary researchers and clinicians are just beginning to appreciate the impact of obesity and altered metabolism on common pulmonary diseases. Obesity has recently been identified as a major risk factor for the development of asthma and for acute respiratory distress syndrome...
June 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/28567493/epidemiologic-features-of-348-children-with-hepatitis-c-virus-infection-over-a-30-year-period-a-nationwide-survey-in-japan
#10
Tatsuki Mizuochi, Tomoko Takano, Tadahiro Yanagi, Kosuke Ushijima, Mitsuyoshi Suzuki, Yoko Miyoshi, Yoshinori Ito, Ayano Inui, Hitoshi Tajiri
BACKGROUND: Although the epidemiology of hepatitis C virus (HCV) infection among children may be rapidly changing, few reports have characterized large nationwide cohorts of children with HCV infection. We, therefore, sought to clarify the epidemiology and natural history of HCV infection in Japanese children born over the last three decades. METHODS: Sixty-five pediatric centers retrospectively and prospectively recruited consecutive, otherwise-healthy HCV-infected children born during 1986 to 2015...
May 31, 2017: Journal of Gastroenterology
https://www.readbyqxmd.com/read/28556542/long-term-outcomes-of-de-novo-autoimmune-hepatitis-in-pediatric-liver-transplant-recipients
#11
Udeme D Ekong, Patrick McKiernan, Mercedes Martinez, Steven Lobritto, Deirdre Kelly, Vicky L Ng, Estella M Alonso, Yaron Avitzur
The long-term course and outcome of DAIH is unknown. A retrospective multicenter study assessing associations and long-term consequences of DAIH developing in a transplanted allograft is presented. Children with DAIH were followed from diagnosis until death, re-LT, or transfer of care and for a minimum of 1 year. A total of 31 patients of 1833 (1.7%) LT were identified; 29 followed for a median of 7.1 years (range, 1.6-15); 52% had no rejection preceding diagnosis of DAIH. Transaminases fell following treatment with steroids and antimetabolites (ALT 108 vs 39 U/L (P=...
May 29, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28543053/sofosbuvir-and-ribavirin-in-adolescents-12-to-17-years-old-with-hepatitis-c-virus-genotype-2-or-3-infection
#12
Stefan Wirth, Philip Rosenthal, Regino P Gonzalez-Peralta, Maureen M Jonas, William F Balistreri, Lin Chuan-Hao, Winita Hardikar, Kathryn Kersey, Benedetta Massetto, Bittoo Kanwar, Diana M Brainard, Jiang Shao, Evguenia Svarovskaia, Brian Kirby, Ronen Arnon, Karen F Murray, Kathleen B Schwarz
BACKGROUND & AIMS: Children with chronic hepatitis C virus (HCV) infection have limited treatment options. We evaluated the all-oral combination of sofosbuvir and ribavirin in adolescents aged 12-17 with HCV genotype 2 or 3. METHODS: Fifty-two patients received sofosbuvir 400mg once daily and weight-based ribavirin twice daily for 12 (genotype 2) or 24 (genotype 3) weeks. The pharmacokinetics of sofosbuvir and its metabolite GS-331007 were evaluated by intensive plasma sampling at day 7 in the first 10 patients enrolled, and by sparse sampling in all patients throughout treatment...
May 22, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28532447/clinical-characteristics-of-disseminated-cryptococcosis-in-previously-healthy-children-in-china
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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.
July 2017: Archives of Disease in Childhood
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