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Chronic liver disease children

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https://www.readbyqxmd.com/read/29680189/vitamin-d-non-sufficiency-is-prevalent-in-children-with-chronic-liver-disease-in-a-tropical-country
#1
Way Seah Lee, Muhammad Yazid Jalaludin, Shin Yee Wong, Sik Yong Ong, Hee Wei Foo, Ruey Terng Ng
BACKGROUND: To determine vitamin D status in children with chronic liver disease (CLD) in a tropical country. METHODS: Cross-sectional study in Malaysian children with CLD. Factors affecting serum vitamin D level (definition: deficient < 30 nmol/L; insufficient 30-50 nmol/L; sufficient ≥ 50 nmol/L) was analyzed. RESULTS: Of the 59 children studied (males 32, 54%; median age 6.8 ± 5.3 years), the three most common causes were biliary atresia (n = 25), autoimmune hepatitis (n = 16) and sclerosing cholangitis (n = 6)...
April 5, 2018: Pediatrics and Neonatology
https://www.readbyqxmd.com/read/29659683/review-of-100-fruit-juice-and-chronic-health-conditions-implications-for-sugar-sweetened-beverage-policy
#2
Brandon J Auerbach, Sepideh Dibey, Petra Vallila-Buchman, Mario Kratz, James Krieger
Whether or not drinking 100% fruit juice causes poor health is controversial. Although 100% fruit juice may contain as much sugar as regular soda, it provides needed nutrients to Americans' diets. We systematically reviewed the current evidence of the association of 100% fruit juice consumption and chronic health conditions in children and adults. We focused on data from systematic reviews and meta-analyses about cardiometabolic health outcomes, liver disease, and caries. Aside from increased risk of tooth decay in children and small amounts of weight gain in young children and adults, there is no conclusive evidence that consumption of 100% fruit juice has adverse health effects...
March 1, 2018: Advances in Nutrition
https://www.readbyqxmd.com/read/29610169/erythropoietic-protoporphyria-initial-diagnosis-with-cholestatic-liver-disease
#3
Amy Coffey, Daniel H Leung, Norma M Quintanilla
The porphyrias are a group of rare metabolic disorders that result from defects in heme biosynthesis. Erythropoietic protoporphyria (EPP) is the most common inherited porphyria in children and is diagnosed in most individuals after the onset of cutaneous manifestations. Hepatobiliary disease affects the minority of individuals with EPP and usually manifests in patients with an established diagnosis of EPP. We report on a classic but rare case of EPP that masqueraded as cholestasis. An 8-year-old boy was referred to the Hepatology Clinic after an abrupt onset of jaundice with a longstanding history of dermatitis...
April 2018: Pediatrics
https://www.readbyqxmd.com/read/29604537/chronic-exposure-to-low-environmental-concentrations-and-legal-aquaculture-doses-of-antibiotics-cause-systemic-adverse-effects-in-nile-tilapia-and-provoke-differential-human-health-risk
#4
Samwel M Limbu, Li Zhou, Sheng-Xiang Sun, Mei-Ling Zhang, Zhen-Yu Du
BACKGROUND: Antibiotics used globally to treat human and animal diseases exist ubiquitously in the environment at low doses because of misuse, overdose and poor absorption after ingestion, coupled with their high-water solubility and degradation resistance. However, the systemic chronic effects of exposure to low environmental concentrations of antibiotics (LECAs) and legal aquaculture doses of antibiotics (LADAs) in fish and their human health risk are currently unknown. OBJECTIVE: To investigate the in vivo chronic effects of exposure to LECAs and LADAs using oxytetracycline (OTC) and sulfamethoxazole (SMZ) in Nile tilapia (Oreochromis niloticus) and their human health risk...
March 27, 2018: Environment International
https://www.readbyqxmd.com/read/29596181/chapter-6-0-history-of-paediatric-hepatology-in-espghan
#5
Martin Burdelski
Hepatology played an important role in ESPGAN from the beginning. In 1989, the Hepatology Committee was started. In the early 1990s H for hepatology was included in ESPGHAN. Hepatology Summer schools were held from 1995 and later monothematic conferences met the needs of many young ESPGHAN members. The role of ESPGHAN members in the progress of diagnostic and therapeutic measures in hepatitis B and C will be elucidated (Chapter 6.1) as well as the role of other ESPGHAN members in the understanding of immunological hepatic disorders of childhood (Chapter 6...
April 2018: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29570559/the-health-care-transition-of-youth-with-liver-disease-into-the-adult-health-system-position-paper-from-espghan-and-easl
#6
Pietro Vajro, Björn Fischler, Patrizia Burra, Dominique Debray, Antal Dezsofi, Salvatore Guercio Nuzio, Nedim Hadzic, Loreto Hierro, Joerg Jahnel, Thierry Lamireau, Patrick McKiernan, Valerie McLin, Valerio Nobili, Piotr Socha, Francoise Smets, Ulli Baumann, Henkjan J Verkade
BACKGROUND: Medical advances have dramatically improved the long-term prognosis of children and adolescents with once-fatal hepatobiliary diseases. However, there is no generally accepted optimal pathway of care for the transition from paediatric care to the adult health system. AIM: The purpose of this position paper is to propose a transition process for young people with paediatric onset hepatobiliary diseases from child-centred to adult-centred healthcare services...
March 22, 2018: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29565146/stunting-wasting-and-mid-upper-arm-circumference-status-among-children-admitted-to-nemazee-teaching-hospital
#7
Seyed Mohsen Dehghani, Hazhir Javaherizadeh, Masoomeh Heidary, Naser Honar, Maryam Ataollahi, Homa Ilkanipour, Hossein Moravej
INTRODUCTION AND AIM: The aim of this study was to evaluate nutritional status in children without prior hospital admission or evidence of chronic disease. SUBJECTS AND METHODS: The current study is a cross-sectional and observational study which was conducted for assessing the nutritional status of children. In this study, consecutive sampling was used, with a sample size about 400 children aged 6 months to 18 years at first hospital admission. All subjects were hospitalized consecutively in the Pediatric Emergency Department of the Nemazee Teaching Hospital of Shiraz University of Medical Sciences (Shiraz, Islamic Republic of Iran)...
January 10, 2018: Nutrición Hospitalaria: Organo Oficial de la Sociedad Española de Nutrición Parenteral y Enteral
https://www.readbyqxmd.com/read/29505478/retrospective-analysis-of-children-with-%C3%AE-1-antitrypsin-deficiency
#8
Atakan Comba, Fatma Demirbaş, Gönül Çaltepe, Esra Eren, Ayhan G Kalayci
BACKGROUND: α-1 Antitrypsin (AAT) deficiency is the most frequently occurring genetic liver disorder. The association among classical α-1 antitrypsin deficiency (AATD), chronic liver disease, and cirrhosis is common in adult patients but rare in children. AIM: To assess the clinical characteristics of children with AATD and to compare symptoms between homozygous and heterozygous children. MATERIALS AND METHODS: The study included 20 children who were found to have mutant Pi alleles...
March 2, 2018: European Journal of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/29504044/weekly-regimen-of-vitamin-d-supplementation-is-more-efficacious-than-stoss-regimen-for-treatment-of-vitamin-d-deficiency-in-children-with-chronic-liver-diseases
#9
Bikrant Bihari Lal, Seema Alam, Rajeev Khanna, Dinesh Rawat
There are no evidence-based recommendations on the ideal dose and regimen for supplementation of vitamin D in children with chronic liver disease (CLD). This study aimed to compare the safety and efficacy of weekly and stoss regimens for treatment of vitamin D deficiency in these children. Children between the ages of 1 to 18 years with CLD and hypovitaminosis D defined by 25-OH vitamin D (25(OH)D) < 30µg/l were included. They were randomized to receive either stoss regimen (600,000 IU on day 1) or weekly (60,000 IU weekly) regimen of vitamin D...
March 4, 2018: European Journal of Pediatrics
https://www.readbyqxmd.com/read/29497824/acute-kidney-injury-in-children-with-chronic-liver-disease
#10
Akash Deep, Romit Saxena, Bipin Jose
Acute kidney injury (AKI) is a common accompaniment in patients with liver disease. The causes, risk factors, manifestations and management of AKI in these patients vary according to the liver disease in question (acute liver failure, acute-on-chronic liver failure, post-liver transplantation or metabolic liver disease). There are multiple causes of AKI in patients with liver disease-pre-renal, acute tubular necrosis, post-renal, drug-induced renal failure and hepatorenal syndrome (HRS). Definitions of AKI in liver failure are periodically revised and updated, but pediatric definitions have still to see the light of the day...
March 1, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29495923/treatment-of-hepatitis-c-virus-infection-for-adults-and-children-updated-swedish-consensus-guidelines-2017
#11
Martin Lagging, Rune Wejstål, Ann-Sofi Duberg, Soo Aleman, Ola Weiland, Johan Westin
AIM: Following the approval of two new therapeutic combinations within the European Union in 2017, the former Swedish recommendations for the treatment of hepatitis C virus (HCV) infection from 2016 were deemed in need of updating. MATERIALS AND METHODS: An expert meeting to this end was held in Stockholm, Sweden in October 2017. RESULTS AND CONCLUSIONS: An interferon-free combination of direct-acting antiviral agents is now recommended for all patients with chronic HCV infection, regardless of liver fibrosis stage, in order to limit morbidity and spread of the disease...
March 1, 2018: Infectious Diseases
https://www.readbyqxmd.com/read/29483797/vascular-toxicity-of-urea-a-new-old-player-in-the-pathogenesis-of-chronic-renal-failure-induced-cardiovascular-diseases
#12
REVIEW
Ida Giardino, Maria D'Apolito, Michael Brownlee, Angela Bruna Maffione, Anna Laura Colia, Michele Sacco, Pietro Ferrara, Massimo Pettoello-Mantovani
Chronic kidney disease in children is an irreversible process that may lead to end-stage renal disease. The mortality rate in children with end-stage renal disease who receive dialysis increased dramatically in the last decade, and it is significantly higher compared with the general pediatric population. Furthermore, dialysis and transplant patients, who have developed end-stage renal disease during childhood, live respectively far less as compared with age/race-matched populations. Different reports show that cardiovascular disease is the leading cause of death in children with end-stage renal disease and in adults with childhood-onset chronic kidney disease, and that children with chronic kidney disease are in the highest risk group for the development of cardiovascular disease...
December 2017: Türk Pediatri Arşivi
https://www.readbyqxmd.com/read/29478492/frailty-in-children-with-liver-disease-a-prospective-multicenter-study
#13
Eberhard Lurz, Claudia Quammie, Michael Englesbe, Estella M Alonso, Henry C Lin, Evelyn K Hsu, Katryn N Furuya, Nitika A Gupta, Veena L Venkat, James F Daniel, Mike A Leonis, Tamir Miloh, Grzegorz W Telega, Jason Yap, Jerome Menendez, Linda S Book, Ryan W Himes, Shikha S Sundaram, Rulan Parekh, Chris Sonnenday, John Bucuvalas, Vicky L Ng, Binita M Kamath
OBJECTIVE: To assess frailty, a measure of physiologic declines in multiple organ systems, in children with chronic liver disease using a novel pediatric frailty tool. STUDY DESIGN: We performed a prospective cross-sectional multicenter study at 17 liver transplantation (LT) centers. 71 children (5-17 years of age), 36 with compensated chronic liver disease (CCLD) and 35 with end-stage liver disease (ESLD) and listed for LT, were assessed for frailty using validated pediatric tools to assess the 5 classic Fried Frailty Criteria-slowness, weakness, exhaustion, diminished physical activity, and shrinkage...
March 2018: Journal of Pediatrics
https://www.readbyqxmd.com/read/29478248/histopathology-of-460-liver-allografts-removed-at-retransplantation-a-shift-in-disease-patterns-over-27-years
#14
Lara Neves Souza, Rodrigo Bronze de Martino, Alberto Sanchez-Fueyo, Mohamed Rela, Anil Dhawan, John O'Grady, Nigel Heaton, Alberto Quaglia
BACKGROUND AND AIM: Approximately 10%-19% of liver transplant recipients develop irreversible graft failure requiring retransplantation. We reviewed the histology of failed grafts removed at retransplantation in our center over 27 years. METHODS: Two hundred and seventy-six adults and 118 children underwent retransplantation from 1987 to 2014, receiving 321 and 139 liver grafts, respectively. We analyzed graft histology, recipient demographics, indications and time interval to retransplantation...
February 25, 2018: Clinical Transplantation
https://www.readbyqxmd.com/read/29471012/pediatric-fatty-liver-disease-pefld-all-is-not-nafld-pathophysiological-insights-and-approach-to-management
#15
REVIEW
Robert Hegarty, Maesha Deheragoda, Emer Fitzpatrick, Anil Dhawan
The recognition of a pattern of steatotic liver injury where histology mimicked alcoholic liver disease but alcohol consumption was denied, led to the identification of non-alcoholic fatty liver disease (NAFLD). Non-alcoholic fatty liver disease has since become the most common chronic liver disease in adults owing to the global epidemic of obesity. However, in pediatrics, the term NAFLD seems incongruous: alcohol consumption is largely not a factor and inherited metabolic disorders (IMD) can mimic or co-exist with a diagnosis of NAFLD...
February 19, 2018: Journal of Hepatology
https://www.readbyqxmd.com/read/29456414/high-prevalence-of-hepatitis-b-antibody-loss-and-a-case-report-of-de-novo-hepatitis-b-virus-infection-in-a-child-after-living-donor-liver-transplantation
#16
Palittiya Sintusek, Nawarat Posuwan, Piyaporn Wanawongsawad, Suttiruk Jitraruch, Yong Poovorawan, Voranush Chongsrisawat
AIM: To assess the seroprevalence of hepatitis B virus (HBV) immunity among previously vaccinated pediatric liver transplant recipients and present a case report of de novo hepatitis B infection after liver transplantation. METHODS: This study focused on children with chronic liver diseases who received primary hepatitis B immunization and had a complete dataset of anti-HBs before and after liver transplantation between May 2001 and June 2017. Medical records were retrospectively reviewed for potential factors relating to HBV immunity loss...
February 14, 2018: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/29451227/statural-growth-and-prevalence-of-endocrinopathies-in-relation-to-liver-iron-content-lic-in-adult-patients-with-beta-thalassemia-major-btm-and-sickle-cell-disease-scd
#17
Mohamed A Yassin, Ashraf T Soliman, Vincenzo De Sanctis, Mohammad Aj Abdula, Lubna M Riaz, Firdous F Ghori, Anil Yousaf, Abdulqadir J Nashwan, Sandara Abusamaan, Abbas Moustafa, Samah Kohla, Dina S Soliman
Despite regular blood transfusion and iron chelation therapy, growth impairment and pubertal delay are commonly seen in children and adolescents with transfusion-dependent Beta thalassaemia major (BTM) and sickle cell disease (SCD). We evaluated growth parameters and endocrine disorders in relation to the liver iron concentration (LIC) assessed by the Ferriscan® method in a cohort of adults with SCD (n =40) and BTM (n = 52) receiving blood transfusions and iron chelation therapy since early childhood. Before transfusion, hemoglobin concentration had not been less than 9 g/dl in the past 12 years; subcutaneous daily desferrioxamine was administered for all of them since early childhood (2- 5 years of age)...
February 16, 2018: Acta Bio-medica: Atenei Parmensis
https://www.readbyqxmd.com/read/29446799/autoimmune-hepatitis-in-children
#18
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/29423877/nhlrc2-variants-identified-in-patients-with-fibrosis-neurodegeneration-and-cerebral-angiomatosis-finca-characterisation-of-a-novel-cerebropulmonary-disease
#19
Johanna Uusimaa, Riitta Kaarteenaho, Teija Paakkola, Hannu Tuominen, Minna K Karjalainen, Javad Nadaf, Teppo Varilo, Meri Uusi-Mäkelä, Maria Suo-Palosaari, Ilkka Pietilä, Anniina E Hiltunen, Lloyd Ruddock, Heli Alanen, Ekaterina Biterova, Ilkka Miinalainen, Annamari Salminen, Raija Soininen, Aki Manninen, Raija Sormunen, Mika Kaakinen, Reetta Vuolteenaho, Riitta Herva, Päivi Vieira, Teija Dunder, Hannaleena Kokkonen, Jukka S Moilanen, Heikki Rantala, Lawrence M Nogee, Jacek Majewski, Mika Rämet, Mikko Hallman, Reetta Hinttala
A novel multi-organ disease that is fatal in early childhood was identified in three patients from two non-consanguineous families. These children were born asymptomatic but at the age of 2 months they manifested progressive multi-organ symptoms resembling no previously known disease. The main clinical features included progressive cerebropulmonary symptoms, malabsorption, progressive growth failure, recurrent infections, chronic haemolytic anaemia and transient liver dysfunction. In the affected children, neuropathology revealed increased angiomatosis-like leptomeningeal, cortical and superficial white matter vascularisation and congestion, vacuolar degeneration and myelin loss in white matter, as well as neuronal degeneration...
February 8, 2018: Acta Neuropathologica
https://www.readbyqxmd.com/read/29404465/nonalcoholic-fatty-liver-disease-and-the-ongoing-role-of-liver-biopsy-evaluation
#20
REVIEW
Elizabeth M Brunt
Nonalcoholic fatty liver disease (NAFLD) is one of the most common underlying causes of chronically elevated liver tests and liver disease in adults and children worldwide and may be strongly suspected if not diagnosed by ever evolving and available serologic and imaging-based noninvasive tests. However, the definitive diagnosis of the most progressive form of NAFLD, nonalcoholic steatohepatitis, and the identification of fibrosis stage still require liver biopsy evaluation as noninvasive testing has not replaced some of the specifics or the totality of information obtainable from liver biopsy...
July 2017: Hepatology communications
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