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Cirrhosis in children

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https://www.readbyqxmd.com/read/29446799/autoimmune-hepatitis-in-children
#1
Saumya Pathak, Deepak Kamat
Autoimmune hepatitis (AIH) is an immune-mediated, inflammatory liver disease. Clinical presentation of AIH in children is highly variable. It can present acutely, chronically, or silently. There are two main types of AIH-type 1 and type 2, which are differentiated and defined by the presence of specific autoantibodies. AIH eventually progresses to cirrhosis when left untreated, and occasionally even with treatment. AIH must be suspected and excluded in all children presenting with signs of acute, prolonged, or severe liver disease...
February 1, 2018: Pediatric Annals
https://www.readbyqxmd.com/read/29446109/systematic-review-the-natural-history-of-alpha-1-antitrypsin-deficiency-and-associated-liver-disease
#2
REVIEW
S A Townsend, R G Edgar, P R Ellis, D Kantas, P N Newsome, A M Turner
BACKGROUND: Alpha-1 antitrypsin deficiency (AATD) is estimated to affect three million people worldwide. It causes liver disease in a proportion of carriers of the PiS and PiZ allele due to the formation and retention of polymers within the endoplasmic reticulum of hepatocytes. The reason for this selective penetrance is not known. Although clinical trials are underway, liver transplantation is the only effective treatment for liver disease due to AATD. AIMS: To report the prevalence and natural history of liver disease among individuals with AATD, and assess the outcomes of liver transplantation through systematic review...
February 15, 2018: Alimentary Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/29434181/efficacy-of-hepatitis-b-vaccination-among-children-in-special-region-of-yogyakarta-indonesia-evaluation-of-humoral-and-cellular-immunity
#3
Caessar Pronocitro, Nenny Sri Mulyani, Afif Avicenna Ghufron, Yugata Halimawan Hazazi, Bambang Ardianto, Didik Setyo Heriyanto
Hepatitis B remains a global burden, with estimated 15 to 40 percents of infected individuals eventually suffer from liver cirrhosis, liver failure, and hepatocellular carcinoma. Vaccination aims to form anti-HBs antibody with protective titer to prevent infection. CD4 T cell lymphocytes are known to play a major role in establishing immunity after vaccination. This study aimed to investigate protective titer rate among Indonesian children in Special Region of Yogyakarta following hepatitis B vaccination and correlation between anti-HBs titer and CD4 count...
January 17, 2018: Kobe Journal of Medical Sciences
https://www.readbyqxmd.com/read/29349944/investigation-of-clinical-and-pathological-relationships-between-adult-and-pediatric-type-nash-in-korean-children
#4
Jungmin Yoon, Byung Han Park, Ja Hye Kim, Jin Hwa Moon, Young Ho Lee, Se Min Jang, Yong Joo Kim
BACKGROUND: Histologically, nonalcoholic steatohepatitis (NASH) is categorized into adult-type (type 1) and pediatric-type (type 2). The origination of the histological difference between the two types and how they differ clinically remain uncertain. We aimed to understand the incidence and clinical characteristics of the two types of NASH in Korean children, and to investigate the association between their pathological type and clinical characteristics, using anthropometric and laboratory data...
January 29, 2018: Journal of Korean Medical Science
https://www.readbyqxmd.com/read/29341979/wilson-s-disease-in-children-a-position-paper-by-the-hepatology-committee-of-the-european-society-for-paediatric-gastroenterology-hepatology-and-nutrition
#5
Piotr Socha, Wojciech Janczyk, Anil Dhawan, Ulrich Baumann, Lorenzo D'Antiga, Stuart Tanner, Raffaele Iorio, Pietro Vajro, Roderick Houwen, Björn Fischler, Antal Dezsofi, Nedim Hadzic, Loreto Hierro, Jörg Jahnel, Valérie McLin, Valerio Nobili, Francoise Smets, Henkjan J Verkade, Dominique Debray
BACKGROUND: Clinical presentations of Wilson's disease (WD) in childhood ranges from asymptomatic liver disease to cirrhosis or acute liver failure, whereas neurological and psychiatric symptoms are rare. The basic diagnostic approach includes serum ceruloplasmin and 24-hour urinary copper excretion. Final diagnosis of WD can be established using a diagnostic scoring system based on symptoms, biochemical tests assessing copper metabolism, and molecular analysis of mutations in the ATP7B gene...
February 2018: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29316057/-a-promising-medium-term-follow-up-of-pediatric-sclerosing-cholangitis-mild-phenotype-or-early-diagnosis
#6
F Ferrari, G Ranucci, M Aloi, L Della Volpe, F Viola, E Miele, S Cucchiara, R Iorio
BACKGROUND AND AIMS: Sclerosing cholangitis (SC) is a chronic cholestatic liver disease whose diagnosis is being increasingly recognized in childhood. Long-term course and prognosis of pediatric SC is poorly described. METHODS: We reviewed data of pediatric SC patients, followed in two referral centers during a period of up to 20 years. We aimed to evaluate long-term outcomes according to SC phenotype. RESULTS: Among 45 patients (median age: 10...
January 9, 2018: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/29286012/hepatitis-a-b-c-and-hiv-seroprevalence-among-syrian-refugee-children-admitted-to-outpatient-clinics
#7
Şükran Köse, Ilker Ödemiş, Didem Çelik, Bengü Gireniz Tatar, Ilkay Akbulut, Dilek Yilmaz Çiftdoğan
Viral hepatitis is the most common cause of serious health problems such as liver cirrhosis and hepatocellular carcinoma (HCC). Leading to immunodeficiency disorders through different mechanisms, Human Immunodeficiency Virus (HIV) causes the development of severe secondary infections. Hepatitis A (HAV) is thought to spread by the faecal-oral route, while Hepatitis B (HBV), Hepatitis C (HCV) and HIV are mostly transmitted vertically during childhood. In our study, we aimed to determine the seroprevalence of HAV, HBV, HCV and HIV among Syrian refugee children who were admitted to outpatient clinics...
December 1, 2017: Le Infezioni in Medicina
https://www.readbyqxmd.com/read/29240010/well-preserved-renal-function-in-children-with-untreated-chronic-liver-disease
#8
Ulla B Berg, Antal Németh
OBJECTIVES: Based on studies with hepatorenal syndrome, it is widely regarded that renal function is impacted in chronic liver disease. Therefore we investigated renal function in our children with chronic liver disease. METHODS: In a retrospective study of 277 children with chronic liver disease, renal function was investigated as glomerular filtration rate (GFR) and effective renal plasma flow (ERPF), measured as clearance of inulin and para-amino hippuric acid or clearance of iohexol...
December 12, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29196283/ringed-telangiectasias-an-unusual-presentation-of-telangiectasia-macularis-eruptiva-perstans
#9
Laila Siddique, Hadjh Ahrns, Elizabeth Seiverling
Telangiectasia macularis eruptiva perstans (TMEP) is a rare form of cutaneous mastocytosis. While most cutaneous mastocytoses occur in children and are asymptomatic, TMEP occurs predominantly in adults and is associated with systemic manifestations, requiring medical management. TMEP is typically characterised by scattered red-brown macules on the trunk and extremities, but must be differentiated from other telangiectatic conditions such as scleroderma, hereditary haemorrhagic telangiectasia and telangiectasias secondary to cirrhosis...
November 30, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/29193603/quality-of-life-in-adolescents-with-hepatitis-c-treated-with-sofosbuvir-and-ribavirin
#10
Zobair M Younossi, Maria Stepanova, Kathleen B Schwarz, Stefan Wirth, Philip Rosenthal, Regino Gonzalez-Peralta, Karen Murray, Linda Henry, Sharon Hunt
Chronic HCV infection has been associated with impairment of health-related quality of life (HRQL) in both adults and pediatric patients. Our aim was to assess the HRQL of HCV-positive children treated with SOF+RBV. The data for this post-hoc analysis was collected in a phase 2 open-label multinational study that evaluated safety and efficacy of sofosbuvir (400mg/day) plus ribavirin (weight-based up to 1400mg/day) for 12 or 24 weeks in adolescents with chronic HCV (GS-US-334-1112). Patients and their parents/guardians completed the PedsQL-4...
November 28, 2017: Journal of Viral Hepatitis
https://www.readbyqxmd.com/read/29183423/outcomes-of-kasai-hepatoportoenterostomy-in-children-with-biliary-atresia-in-johannesburg-south-africa
#11
T De Maayer, S G Lala, J Loveland, G Okudo, R Mohanlal, C Hajinicolaou
BACKGROUND: Without timely surgical intervention, most children with biliary atresia (BA) are not expected to live beyond 2 years of age. The initial intervention, the Kasai hepatoportoenterostomy (KPE), aims to achieve biliary drainage. Liver transplantation (LT) is performed if jaundice fails to clear or when biliary cirrhosis occurs. In under-resourced South African (SA) academic state hospitals, KPE procedures are the standard of care for the majority of children with BA, but LT is becoming more routinely available...
November 6, 2017: South African Medical Journal, Suid-Afrikaanse Tydskrif Vir Geneeskunde
https://www.readbyqxmd.com/read/29128061/the-genetics-of-pediatric-nonalcoholic-fatty-liver-disease
#12
REVIEW
Nidhi P Goyal, Jeffrey B Schwimmer
Nonalcoholic fatty liver disease (NAFLD) is the leading cause of chronic liver disease in children. Severe fibrosis and cirrhosis are potential consequences of pediatric NAFLD and can occur within a few years of diagnosis. Observations suggest that genetics may be a strong modifying factor in the presentation, severity, and natural history of the disease. There is increasing interest in determining at-risk populations based on genetics in the hope of finding genotypes that correlate to NAFLD phenotype. Ultimately, the hope is to be able to tailor therapeutics to genetic predispositions and decrease disease morbidity in children with NAFLD...
February 2018: Clinics in Liver Disease
https://www.readbyqxmd.com/read/29126168/endocrine-disruptors-and-developmental-origins-of-nonalcoholic-fatty-liver-disease
#13
Lindsey S Treviño, Tiffany A Katz
Nonalcoholic fatty liver disease (NAFLD) is a growing epidemic worldwide, particularly in countries that consume a Western diet, and can lead to life-threatening conditions such as cirrhosis and hepatocellular carcinoma (HCC). With increasing prevalence of NAFLD in both children and adults, an understanding of the factors that promote NAFLD development and progression is crucial. Environmental agents, including endocrine-disrupting chemicals (EDCs), that have been linked to other diseases may play a role in NAFLD development...
November 6, 2017: Endocrinology
https://www.readbyqxmd.com/read/29115027/clinical-features-of-pediatric-autoimmune-hepatitis-in-japan-a-nationwide-survey
#14
Tsuyoshi Sogo, Atsushi Takahashi, Ayano Inui, Tomoo Fujisawa, Hiromasa Ohira, Hajime Takikawa
AIM: The purpose of this study was to determine the characteristics of children with autoimmune hepatitis (AIH) in Japan. METHODS: Questionnaires, which asked about patients newly diagnosed with AIH from 2009 to 2013 were sent to hospitals certified as training facilities for pediatrics in January 2015. RESULTS: A total of 35 patients were enrolled. The median age at diagnosis was 10 years (ranging from 3 months to 15 years), and the male-to-female ratio was 2:3...
November 8, 2017: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/29108191/-an-interpretation-of-the-aasld-practice-guideline-on-the-diagnosis-and-management-of-nonalcoholic-fatty-liver-disease-in-2017
#15
Y M Nan, N Fu, W C Li, L L Kong, X W Yuan, S Y Zhang, L D Liu, Y Lu, L Y Cui
The American Association for the Study of Liver Diseases (AASLD) updated and published the Practice Guidance for the Diagnosis and Management of Nonalcoholic Fatty Liver Disease (NAFLD) in July 2017, which provides recommendations for the accurate diagnosis, treatment, and effective prevention of NAFLD. Related metabolic diseases should be considered during the initial evaluation of patients suspected of NAFLD. Noninvasive diagnostic techniques including transient elastography, magnetic resonance elastography, and serum biochemical models should be used to evaluate the development and progression of liver fibrosis in patients with NAFLD...
September 20, 2017: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://www.readbyqxmd.com/read/29059700/favorable-response-to-immunosuppressive-combination-therapy-with-mizoribine-and-azathioprine-in-children-with-primary-sclerosing-cholangitis
#16
Hitoshi Tajiri, Yoh Zen, Tomoko Takano, Stephen Brooks
Primary sclerosing cholangitis (PSC), with no curative intervention, can progress to end-stage liver disease. Mizoribine, a purine antimetabolite, has never been used for the management of PSC. To evaluate the role of mizoribine with azathioprine we undertook a preliminary clinical study in children with PSC. Children with PSC and autoimmune features were simultaneously treated with mizoribine and azathioprine. Ursodeoxycholic acid or mesalazine were not regulated. The primary end-points of our study included improvement of aspartate aminotransferase, alanine aminotransferase, and γ-glutamyltransferase...
October 23, 2017: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/29030176/physiologically-based-pharmacokinetic-modeling-of-disposition-and-drug-drug-interactions-for-valproic-acid-and-divalproex
#17
Todd M Conner, Vahagn C Nikolian, Patrick E Georgoff, Manjunath P Pai, Hasan B Alam, Duxin Sun, Ronald C Reed, Tao Zhang
Valproic acid (VPA) is an older first-line antiepileptic drug with a complex pharmacokinetic (PK) profile, currently under investigation for several novel neurologic and non-neurologic indications. Our study objective was to design and validate a mechanistic model of VPA disposition in adults and children; and evaluate its predictive performance of drug-drug interactions (DDIs). This study expands upon existing physiologically based pharmacokinetic (PBPK) models for VPA by incorporating UGT enzyme kinetics and an advanced dissolution, absorption, and metabolism (ADAM) model for extended-release (ER) formulation...
January 1, 2018: European Journal of Pharmaceutical Sciences
https://www.readbyqxmd.com/read/28986027/cystic-fibrosis-related-cirrhosis
#18
Daniel H Leung, Michael R Narkewicz
While liver involvement is common in cystic fibrosis, the major liver disorder with impact on the clinical outcome of individuals with CF is the development of multilobular cirrhosis with progression to portal hypertension. Interestingly, this is a disorder primarily of children and adolescents. We review the proposed pathogenesis, clinical presentation, diagnostic work-up, medical and surgical management, and complications of CF cirrhosis.
November 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28983725/liver-transplantation-for-biliary-atresia-a-systematic-review
#19
REVIEW
Mureo Kasahara, Koji Umeshita, Seisuke Sakamoto, Akinari Fukuda, Hiroyuki Furukawa, Shinji Uemoto
Biliary atresia (BA) is an idiopathic neonatal cholangiopathy characterized by progressive inflammatory obliteration of the intrahepatic or extrahepatic bile ducts. Although the Kasai operation has dramatically improved the outcomes in children with BA, most patients with BA eventually require liver transplantation (LT) even after undergoing a successful Kasai procedure. The Japanese LT Society (JLTS) was established in 1980 to characterize and follow trends in patient characteristics and the graft survival among all liver transplant patients in Japan...
December 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28983658/newborn-screening-for-biliary-atresia-in-the-united-states
#20
Cat Goodhue, Michael Fenlon, Kasper S Wang
Despite advances in our understanding of the pathogenesis of biliary atresia (BA), BA remains the most common cause of end-stage liver disease in children and the leading indication for pediatric liver transplantation. Age at time of Kasai portoenterostomy (KPE), performed to provide bile drainage, strongly correlates with transplant-free survival, mostly due to progression of intrahepatic fibrosis to cirrhosis. Unfortunately, challenges remain in recognizing that a jaundiced infant may have BA. To better diagnose infants with BA at an earlier age, population-based screening programs in countries such as Taiwan, Japan, and China have utilized stool color cards...
December 2017: Pediatric Surgery International
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