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Cirrhosis children

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https://www.readbyqxmd.com/read/28905444/response-to-hepatitis-b-vaccination-in-patients-with-liver-cirrhosis
#1
REVIEW
Ioanna Aggeletopoulou, Panagiota Davoulou, Christos Konstantakis, Konstantinos Thomopoulos, Christos Triantos
Hepatitis B vaccination is strongly recommended for all infants and children but also for adults who are at risk of HBV infection. Attempts to immunize patients with liver cirrhosis have been proven relatively ineffective, and several strategies have already been used to improve the immune response in this group. The primary aim of this review is to examine, discuss, and summarize the immunogenicity of hepatitis B vaccination in patients with liver cirrhosis. MEDLINE search identified 11 studies (n = 961)...
September 14, 2017: Reviews in Medical Virology
https://www.readbyqxmd.com/read/28902093/bile-acid-synthesis-disorders-in-arabs-a-10-year-screening-study
#2
Abdulrahman Al-Hussaini, Kenneth D R Setchell, Bader AlSaleem, James E Heubi, Khurram Lone, Anne Davit-Spraul, Emmanuel Jacquemin
OBJECTIVES: Early diagnosis of bile acid synthesis disorders (BASD) is important because, untreated, these conditions can be fatal. Our objectives were to screen children with cholestasis or unexplained liver disease for BASD and in those with confirmed BASD to evaluate the effectiveness of cholic acid therapy. METHODS: A routine serum total bile acid measurement was performed on children with cholestasis, liver cirrhosis, and liver failure. Patients were screened for BASD by fast atom bombardment ionization-mass spectrometry (FAB-MS) analysis of urine, and molecular analysis confirmed diagnosis...
September 9, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28895701/management-of-cirrhotic-ascites-in-children-review-and-recommendations-part-2-electrolyte-disturbances-nonelectrolyte-disturbances-therapeutic-options
#3
David F Bes, M Cristina Fernández, Ivone Malla, Horacio A Repetto, Daniel Buamscha, Susana López, Roxana Martinitto, Miriam Cuarterolo, Fernando Alvarez
Ascites is a major complication of cirrhosis. There are several evidence-based articles and guidelines for the management of adults, but few data have been published in relation to children. In the case of a pediatric patient with cirrhotic ascites (PPCA), the following questions are raised: How are the clinical assessment and ancillary tests performed? When is ascites considered refractory? How is it treated? Should fresh plasma and platelets be infused before abdominal paracentesis to prevent bleeding? What are the hospitalization criteria? What are the indicated treatments? What complications can patients develop? When and how should hyponatremia be treated? What are the diagnostic criteria for spontaneous bacterial peritonitis? How is it treated? What is hepatorenal syndrome? How is it treated? When should albumin be infused? When should fluid intake be restricted? The recommendations made here are based on pathophysiology and suggest the preferred approach to diagnostic and therapeutic aspects, and preventive care...
October 1, 2017: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/28892178/review-article-the-diagnostic-approach-and-current-management-of-chylous-ascites
#4
REVIEW
B Lizaola, A Bonder, H D Trivedi, E B Tapper, A Cardenas
BACKGROUND: Chylous ascites is rare, accounting for less than 1% of cases. An appropriate and stepwise approach to its diagnosis and management is of key importance. AIM: To review the current diagnostic approach and management of chylous ascites. METHODS: A literature search was conducted using PubMed using the key words 'chylous', 'ascites', 'cirrhosis', 'pathophysiology', 'nutritional therapy', 'paracentesis", "transjugular intrahepatic portosystemic shunt" and "TIPSS'...
September 11, 2017: Alimentary Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/28856289/recommendations-for-the-treatment-of-hepatitis-b-in-2017
#5
REVIEW
Robert Flisiak, Waldemar Halota, Jerzy Jaroszewicz, Jacek Juszczyk, Piotr Małkowski, Małgorzata Pawłowska, Anna Piekarska, Krzysztof Simon, Krzysztof Tomasiewicz, Marta Wawrzynowicz-Syczewska
The therapeutic goal which is currently unfrequent but realistic in HBV infected patients is sustained HBsAg clearance. It is preceded by the loss or significant suppression of HBV replication and leads to inhibition of the progression of liver fibrosis, normalization of biochemical indicators of liver damage, reduction in the risk of hepatocellular carcinoma, prolongation of survival, prevention of HBV infection in the transplanted organ in post-transplant patients, enhancement of the quality of life, inhibition or reversal of extrahepatic changes associated with HBV infection, and halting of the spread of HBV infections...
June 2017: Clin Exp Hepatol
https://www.readbyqxmd.com/read/28834223/liver-and-systemic-hemodynamics-in-cirrhotic-children-impact-on-the-surgical-management-in-pediatric-living-donor-liver-transplantation
#6
Catherine de Magnée, Francis Veyckemans, Thierry Pirotte, Renaud Menten, Dana Dumitriu, Philippe Clapuyt, Karlien Carbonez, Catherine Barrea, Thierry Sluysmans, Christine Sempoux, Isabelle Leclercq, Francis Zech, Xavier Stephenne, Raymond Reding
BACKGROUND AND AIMS: Cirrhosis in adults is associated with modifications of systemic and liver hemodynamics, whereas little is known about the pediatric population. The aim of this work was to investigate whether alterations of hepatic and systemic hemodynamics were correlated with cirrhosis severity in children. The impact of hemodynamic findings on surgical management in pediatric living donor liver transplantation was evaluated. METHODS: Liver and systemic hemodynamics were studied prospectively in 52 children (median age= 1 year; 33 biliary atresia)...
August 17, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/28804516/the-role-of-sebelipase-alfa-in-the-treatment-of-lysosomal-acid-lipase-deficiency
#7
REVIEW
Angelika L Erwin
Lysosomal acid lipase deficiency (LALD) is a lysosomal storage disorder (LSD) characterized either by infantile onset with fulminant clinical course and very poor prognosis or childhood/adult-onset disease with an attenuated phenotype. The disorder is often misdiagnosed or remains undiagnosed in children and adults due to a rather unspecific clinical presentation with dyslipidemia and steatohepatitis. Until recently, no good treatment options were available for LALD. Despite supportive and symptomatic therapies, death occurred before 1 year of age in patients with infantile-onset disease and patients with childhood/adult-onset LALD suffered from significant complications, such as liver cirrhosis, requiring liver transplantation and early-onset cardiovascular disease...
July 2017: Therapeutic Advances in Gastroenterology
https://www.readbyqxmd.com/read/28800087/imaging-features-of-non-alcoholic-fatty-liver-disease-in-children-and-adolescents
#8
REVIEW
Michele Di Martino, Kameliya Koryukova, Mario Bezzi, Carlo Catalano
Non-invasive diagnosis and quantification of liver steatosis is important to overcome limits of liver biopsy, in order to follow up patients during their therapy and to establish a reference standard that can be used in clinical trials and longitudinal studies. Imaging offers several methods in this setting: ultrasound, which is the cheapest technique and easy to perform; magnetic resonance spectroscopy (MRS), which reflects the real content of triglycerides in a specific volume; and proton density fat fraction (PDFF) magnetic resonance, which is a simple method that reflects the distribution of the fat in the whole liver...
August 11, 2017: Children
https://www.readbyqxmd.com/read/28774413/management-of-hepatitis-b-in-special-populations
#9
REVIEW
Kali Zhou, Norah Terrault
Special populations infected with chronic HBV include those with decompensated cirrhosis, coinfections (HIV, HCV, HDV), hemodialysis and renal failure, immunosuppressed including transplant patients, children and women in pregnancy. These populations differ in their natural history and risk for liver-related complications, the indications for anti-HBV therapy as well as the recommendations regarding the HBV drugs used, duration of therapy and anticipated endpoints. Reflecting the special populations with substantive changes in management in recent years, this review focuses on HBV-HIV coinfected patients, immunosuppressed patients at risk for reactivation, liver transplant recipients and pregnant women...
June 2017: Best Practice & Research. Clinical Gastroenterology
https://www.readbyqxmd.com/read/28764309/death-rate-of-dental-anaesthesia
#10
REVIEW
Hamed Mortazavi, Maryam Baharvand, Yaser Safi
Death was the most important side effect of anaesthesia in dentistry. In this article we reviewed more than 20 studies with adequate data focusing on death associated with dental procedures since 1955 and found 218 deaths out of 71,435,282 patients (3 deaths per 1,000,000 persons) with the mortality rate of 1:327,684. In addition, mortality rate per million has dropped to half (6.2 per 1,000,000 vs. 3 per 1,000,000) since 1955 till the last report in 2012 without any sex predilection. In children, most cases died in the age of two to five years...
June 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28752463/semiquantitation-of-monomer-and-polymer-alpha-1-antitrypsin-by-centrifugal-separation-and-assay-by-western-blot-of-soluble-and-insoluble-components
#11
Keith S Blomenkamp, Jeffrey H Teckman
Alpha-1 antitrypsin (a1AT) deficiency, in its classical form, is an autosomal recessive disease associated with an increased risk of liver disease in adults and children, and with lung disease in adults. The vast majority of liver disease is associated with homozygosity for the Z mutant allele, also called PiZZ. This homozygous allele synthesizes large quantities of a1AT mutant Z protein in the liver, but the mutant protein also folds improperly during biogenesis. As a result, approximately 85% of the molecules are retained within the hepatocytes instead of being appropriately secreted...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28752441/pathophysiology-of-alpha-1-antitrypsin-deficiency-liver-disease
#12
Jeffrey H Teckman, Keith S Blomenkamp
Classical alpha-1 antitrypsin (a1AT) deficiency is an autosomal recessive disease associated with an increased risk of liver disease in adults and children, and with lung disease in adults (Teckman and Jain, Curr Gastroenterol Rep 16(1):367, 2014). The vast majority of the liver disease is associated with homozygosity for the Z mutant allele, the so-called PIZZ. These homozygous individuals synthesize large quantities of a1AT mutant Z protein in the liver, but the mutant protein folds improperly during biogenesis and approximately 85% of the molecules are retained within the hepatocytes rather than appropriately secreted...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28737869/management-of-cirrhotic-ascites-in-children-review-and-recommendations-part-1-pathophysiology-diagnostic-evaluation-hospitalization-criteria-treatment-nutritional-management
#13
REVIEW
David F Bes, M Cristina Fernández, Ivone Malla, Horacio A Repetto, Daniel Buamsch, Susana López, Roxana Martinitto, Miriam Cuarterolo, Fernando Álvarez
Ascites is a major complication of cirrhosis. There are several evidence-based articles and guidelines for the management of adults, but few data have been published in relation to children. In the case of pediatric patients with cirrhotic ascites (PPCA), the following questions are raised: How are the clinical assessment and ancillary tests performed? When is ascites considered refractory? How is it treated? Should fresh plasma and platelets be infused before abdominal paracentesis to prevent bleeding? What are the hospitalization criteria? What are the indicated treatments? What complications can patients develop? When and how should hyponatremia be treated? What are the diagnostic criteria for spontaneous bacterial peritonitis? How is it treated? What is hepatorenal syndrome? How is it treated? When should albumin be infused? When should fluid intake be restricted? The recommendations made here are based on pathophysiology and suggest the preferred approach to its diagnostic and therapeutic aspects, and preventive care...
August 1, 2017: Archivos Argentinos de Pediatría
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
#14
A S 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...
October 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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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