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Chronic liver disease children

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https://www.readbyqxmd.com/read/28214296/abnormally-high-and-heterogeneous-bone-matrix-mineralization-after-childhood-solid-organ-transplantation-a-complex-pathology-of-low-bone-turnover-and-local-defects-in-mineralization
#1
Nadja Fratzl-Zelman, Helena Valta, Renata C Pereira, Barbara M Misof, Paul Roschger, Hannu Jalanko, Kathrine Wesseling-Perry, Klaus Klaushofer, Outi Mäkitie
Chronic renal, liver and heart failure in children associates with multiple skeletal complications. Increased fracture incidence often persists after transplantation and could be related to alterations in bone material properties. In the present cohort study we evaluated bone mineralization density distribution (BMDD) by quantitative backscattered electron imaging (qBEI) in 23 pediatric solid organ allograft recipients with suspected osteoporosis. We measured BMDD in the entire cross-sectional area of transiliac bone biopsies obtained from kidney (n = 9), liver (n = 9) and heart (n = 5) transplant recipients (aged 7...
February 18, 2017: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
https://www.readbyqxmd.com/read/28210784/novel-aids-therapies-based-on-gene-editing
#2
REVIEW
Kamel Khalili, Martyn K White, Jeffrey M Jacobson
HIV/AIDS remains a major public health issue. In 2014, it was estimated that 36.9 million people are living with HIV worldwide, including 2.6 million children. Since the advent of combination antiretroviral therapy (cART), in the 1990s, treatment has been so successful that in many parts of the world, HIV has become a chronic condition in which progression to AIDS has become increasingly rare. However, while people with HIV can expect to live a normal life span with cART, lifelong medication is required and cardiovascular, renal, liver, and neurologic diseases are still possible, which continues to prompt research for a cure for HIV...
February 16, 2017: Cellular and Molecular Life Sciences: CMLS
https://www.readbyqxmd.com/read/28208962/the-effect-of-nutritional-status-on-outcome-of-hospitalization-in-paediatric-liver-disease-patients
#3
Yasmeen Mansi, Shereen Abdel Ghaffar, Shaymaa Sayed, Hanaa El-Karaksy
INTRODUCTION: Liver is an important organ for metabolism. It has a major role in integrating the various biochemical pathways of metabolism. Thus, children with chronic liver disease are at high risk for developing undernutrition, with important prognostic implications. AIM: To evaluate the nutritional status of hospitalized paediatric liver disease patients and its effect on outcome. MATERIALS AND METHODS: We prospectively analysed the nutritional status of 59 consecutive patients during their first 24 hours of admission, at the Hepatology Unit, using the following indices: weight/age, height/age, weight/height, Body Mass Index (BMI), arm circumference and triceps skinfold, subcapular skinfold, and mid upper arm circumference...
December 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28194394/assessment-of-school-readiness-in-chronic-cholestatic-liver-disease-a-pilot-study-examining-children-with-and-without-liver-transplantation
#4
Anna Gold, Alaine Rogers, Elizabeth Cruchley, Stephanie Rankin, Arpita Parmar, Binita M Kamath, Yaron Avitzur, Vicky Lee Ng
Background. Assessment of school readiness evaluates physical, social-emotional, and neuropsychological domains essential for educational success. Cognitive testing of preschool aged children with chronic liver disease may guide more timely interventions and focused efforts by health care providers. Patients and Methods. Children with chronic cholestatic liver disease diagnosed as an infant and still with their native liver (NL) and children who received a liver transplant (LT) before age of 2 years underwent testing with a battery of well-validated pediatric psychometric measures...
2017: Canadian Journal of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28168067/necrotizing-liver-granuloma-abscess-and-constrictive-aspergillosis-pericarditis-with-central-nervous-system-involvement-different-remarkable-phenotypes-in-different-chronic-granulomatous-disease-genotypes
#5
Sanem Eren Akarcan, Neslihan Karaca, Guzide Aksu, Halil Bozkaya, Mehmet Fatih Ayik, Yasemin Ozdemir Sahan, Mehmet Arda Kilinc, Zafer Dokumcu, Cenk Eraslan, Emre Divarci, Hudaver Alper, Necil Kutukculer
Chronic granulomatous disease (CGD) is a primary immune deficiency causing predisposition to infections with specific microorganisms, Aspergillus species and Staphylococcus aureus being the most common ones. A 16-year-old boy with a mutation in CYBB gene coding gp91(phox) protein (X-linked disease) developed a liver abscess due to Staphylococcus aureus. In addition to medical therapy, surgical treatment was necessary for the management of the disease. A 30-month-old girl with an autosomal recessive form of chronic granulomatous disease (CYBA gene mutation affecting p22(phox) protein) had invasive aspergillosis causing pericarditis, pulmonary abscess, and central nervous system involvement...
2017: Case Reports in Immunology
https://www.readbyqxmd.com/read/28164598/analysis-of-biochemical-features-of-hepatitis-b-virus-infected-patients-in-southwest-china
#6
Yuzhu Song, Yunsong Shen, Wencheng Lu, Xueshan Xia, A-Mei Zhang
BACKGROUND: Hepatitis B is one of the most common infectious diseases in China, and it is necessary to study biochemical indicators of HBV infected patients. METHODS: Biochemical and basic features of 1765 HBV infected patients and 840 general controls were collected and analyzed from Southwest China. RESULTS: A total of 23.5% of the patients were children or young persons (less than 40 years old). The co-infected rate was only 0.3% for those who were co-infected with Hepatitis C virus (HCV) or Treponema pallidum (TPa)...
September 1, 2016: Clinical Laboratory
https://www.readbyqxmd.com/read/28107283/naspghan-clinical-practice-guideline-for-the-diagnosis-and-treatment-of-nonalcoholic-fatty-liver-disease-in-children-recommendations-from-the-expert-committee-on-nafld-econ-and-the-north-american-society-of-pediatric-gastroenterology-hepatology-and-nutrition
#7
Miriam B Vos, Stephanie H Abrams, Sarah E Barlow, Sonia Caprio, Stephen R Daniels, Rohit Kohli, Marialena Mouzaki, Pushpa Sathya, Jeffrey B Schwimmer, Shikha S Sundaram, Stavra A Xanthakos
Nonalcoholic fatty liver disease (NAFLD) is a highly prevalent chronic liver disease that occurs in the setting of insulin resistance and increased adiposity. It has rapidly evolved into the most common liver disease seen in the pediatric population and is a management challenge for general pediatric practitioners, subspecialists, and for health systems. In this guideline, the expert committee on NAFLD reviewed and summarized the available literature, formulating recommendations to guide screening and clinical care of children with NAFLD...
February 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28099338/liver-steatosis-in-children-with-chronic-hepatitis-b-and-c-prevalence-predictors-and-impact-on-disease-progression
#8
Maria Pokorska-Śpiewak, Barbara Kowalik-Mikołajewska, Małgorzata Aniszewska, Magdalena Pluta, Bożena Walewska-Zielecka, Magdalena Marczyńska
Only scarce data on liver steatosis in children with chronic hepatitis B and C (CHB and CHC) are available. The objective of this study was to evaluate the prevalence, predictors, and impact of hepatic steatosis on children with CHB and CHC. A total of 78 patients aged 11.5 ± 3.4 years were included: 30 (38%) had CHB, and 48 (62%) had CHC. Steatosis was scored on a 5-point scale, as follows: absent; minimal (≤5% hepatocytes affected), mild (6-33%), moderate (34-66%), and severe (>66%). Stepwise logistic regression was used to determine the factors associated with steatosis and moderate-to-severe steatosis...
January 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28094851/blood-transfusion-for-preventing-primary-and-secondary-stroke-in-people-with-sickle-cell-disease
#9
REVIEW
Lise J Estcourt, Patricia M Fortin, Sally Hopewell, Marialena Trivella, Winfred C Wang
BACKGROUND: Sickle cell disease is one of the commonest severe monogenic disorders in the world, due to the inheritance of two abnormal haemoglobin (beta globin) genes. Sickle cell disease can cause severe pain, significant end-organ damage, pulmonary complications, and premature death. Stroke affects around 10% of children with sickle cell anaemia (HbSS). Chronic blood transfusions may reduce the risk of vaso-occlusion and stroke by diluting the proportion of sickled cells in the circulation...
January 17, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28092152/issues-in-nutrition-dietary-considerations-in-select-chronic-conditions
#10
Margaret E Thompson, Mary Barth Noel
Chronic diseases that affect the gastrointestinal tract also tend to affect nutrition. The incidence of chronic liver disease is increasing. As the prevalence of obesity rises, so do the incidences of nonalcoholic fatty liver disease and nonalcoholic steatohepatitis. Patients with chronic liver disease usually have some degree of malnutrition. In the absence of encephalopathy, patients with chronic liver disease should consume more protein than that in the average diet. There is some controversy about whether diet plays a role in the development of inflammatory bowel disease...
January 2017: FP Essentials
https://www.readbyqxmd.com/read/28044976/-kidsetransplant-a-serious-game-for-liver-transplanted-children
#11
Ana-Maria Calinescu, Valérie McLin, Stéphane Spahni, Thomas Boggini, Mirana Randriambelonoro, Roxane Jaquier-Grant, Michèle Steiner, Antoine Geissbuhler, Barbara E Wildhaber
Serious games are increasingly used in pediatric care, especially to educate young patients with chronic diseases, to improve their understanding of the disease and develop independence in disease management, as it can ultimately improve clinical results. This is also true for liver transplanted children and adolescents, who are not only challenged by their therapeutic burden, but who need to face transition to adult care, often without remembering their transplantation, which was performed, most of the time, very early in their life...
December 2016: Médecine Sciences: M/S
https://www.readbyqxmd.com/read/28040394/long-term-hematological-visceral-and-growth-outcomes-in-children-with-gaucher-disease-type-3-treated-with-imiglucerase-in-the-international-collaborative-gaucher-group-gaucher-registry
#12
Amal El-Beshlawy, Anna Tylki-Szymanska, Ashok Vellodi, Nadia Belmatoug, Gregory A Grabowski, Edwin H Kolodny, Julie L Batista, Gerald F Cox, Pramod K Mistry
In Gaucher disease (GD), deficiency of lysosomal acid β-glucosidase results in a broad phenotypic spectrum that is classified into three types based on the absence (type 1 [GD1]) or presence and severity of primary central nervous system involvement (type 2 [GD2], the fulminant neuronopathic form, and type 3 [GD3], the milder chronic neuronopathic form). Enzyme replacement therapy (ERT) with imiglucerase ameliorates and prevents hematological and visceral manifestations in GD1, but data in GD3 are limited to small, single-center series...
December 6, 2016: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/28040302/omega-3-fatty-acids-as-a-treatment-for-non-alcoholic-fatty-liver-disease-in-children-a-systematic-review-and-meta-analysis-of-randomized-controlled-trials
#13
Lian-Hui Chen, Yong-Fen Wang, Qing-Hong Xu, Shan-Shan Chen
BACKGROUND: The most typical chronic liver disease in children and adolescents is non-alcoholic fatty liver disease (NAFLD). The dietary addition of ω-3 polyunsaturated fatty acids (PUFAs) provides a promising therapy for children with NAFLD due to its convenience and safety; however, several studies suggested contradictory results for PUFA supplementation in children. Hence, we performed a systematic review and meta-analysis to evaluate the effectiveness of PUFA supplementation in children with NAFLD...
December 23, 2016: Clinical Nutrition: Official Journal of the European Society of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/28027602/is-size-the-only-determinant-of-delayed-abdominal-closure-in-pediatric-liver-transplant
#14
Shirin Elizabeth Khorsandi, Arthur William Raven Day, Miriam Cortes, Akash Deep, Anil Dhawan, Hector Vilca-Melendez, Nigel Heaton
The aim was to determine the factors associated with the use of delayed abdominal closure in pediatric liver transplant and whether this affected outcome. From a prospectively maintained database, transplants performed in children (≤ 18 years) were identified (October 2010 - March 2015). Primary abdominal closure was defined as mass closure performed at time of transplant. Delayed abdominal closure was defined as mass closure not initially performed at the same time as transplant. 230 children underwent liver transplantation...
December 27, 2016: Liver Transplantation
https://www.readbyqxmd.com/read/28006876/health-related-quality-of-life-after-pediatric-liver-transplantation-a-systematic-review
#15
REVIEW
Arpita Parmar, Shannon Marie Vandriel, Vicky Lee Ng
With improved survival rates after pediatric liver transplantation (LT), attention is targeting improving the health-related quality of life (HRQOL) as an outcome metric. We conducted a systematic review of the literature to examine HRQOL after pediatric LT, focusing on assessment tools, and factors associated with HRQOL.A literature search was conducted through PubMed, Web of Science, OVID, and Google Scholar for all studies matching the eligibility criteria between January 2004 and September 2016. Titles and abstracts were screened independently by two authors and consensus for included studies was achieved through discussion...
December 22, 2016: Liver Transplantation
https://www.readbyqxmd.com/read/27957786/impaired-intention-to-treat-survival-after-listing-for-liver-transplantation-in-children-with-biliary-atresia-compared-to-other-chronic-liver-diseases-20%C3%A2-years-experience-from-the-nordic-countries
#16
S Malenicka, B-G Ericzon, M H Jørgensen, H Isoniemi, T H Karlsen, M Krantz, V Naeser, M Olausson, A Rasmussen, K Rönnholm, T Sanengen, T Scholz, B Fischler, A Nemeth
Biliary atresia (BA) is the most common indication for LT in children. We investigated whether this diagnosis per se, compared to other chronic liver diseases (OCLD), had an influence on patient survival. Data from 421 Scandinavian children, 194 with BA and 227 with OCLD, listed for LT between 1990 and 2010 were analyzed. The intention-to-treat survival and influencing risk factors were studied. Patients with BA had higher risk of death after listing than patients with OCLD. The youngest (<1 year) and smallest (<10 kg) children with the highest bilirubin (>510 μmol/L), highest INR (>1...
December 13, 2016: Pediatric Transplantation
https://www.readbyqxmd.com/read/27931582/liver-transplantation-for-hepatitis-b-in-early-adulthood-analysis-of-the-united-network-for-organ-sharing-database
#17
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/27906803/naspghan-clinical-practice-guideline-for-the-diagnosis-and-treatment-of-nonalcoholic-fatty-liver-disease-in-children
#18
Miriam B Vos, Stephanie H Abrams, Sarah E Barlow, Sonia Caprio, Stephen R Daniels, Rohit Kohli, Marialena Mouzaki, Pushpa Sathya, Jeffrey B Schwimmer, Shikha S Sundaram, Stavra A Xanthakos
Nonalcoholic fatty liver disease (NAFLD) is a highly prevalent chronic liver disease that occurs in the setting of insulin resistance and increased adiposity. It has rapidly evolved into the most common liver disease seen in the pediatric population and is a management challenge for general pediatric practitioners, subspecialists and for health systems. In this guideline, the expert committee on NAFLD (ECON) reviewed and summarized the available literature, formulating recommendations to guide screening and clinical care of children with NAFLD...
November 30, 2016: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/27898169/cross-sectional-analysis-of-progressive-familial-intrahepatic-cholestasis-in-puerto-rican-children
#19
Dellys M Soler, Antonio I Del Valle, David Fernandez-Lube, Benjamin L Shneider
OBJECTIVE: Specific inherited disorders may be more common in island communities. Prior case reports suggest that cholestatic liver diseases may constitute a group of these inherited disorders in Puerto Rico. A cross-sectional survey of liver diseases in children was conducted to assess this hypothesis. METHODS: A cross-sectional analysis was performed in patients with chronic cholestasis at "Hospital Pediátrico Universitario" in San Juan, Puerto Rico. Ten potential participants with high gamma-glutamyl transpeptidase (GGTP) cholestasis were identified...
December 2016: Puerto Rico Health Sciences Journal
https://www.readbyqxmd.com/read/27862714/de-novo-inflammatory-bowel-disease-after-pediatric-kidney-or-liver-transplant
#20
Melissa A Fernandes, Hillary J Braun, Kim Evason, Sue Rhee, Emily R Perito
A subset of children who receive a liver and/or kidney transplant develop de novo inflammatory bowel disease-like chronic intestinal inflammation, not explained by infection or medications, following transplant. We have conducted a single-center, retrospective case series describing the unique clinical and histologic features of this IBD-like chronic intestinal inflammation following solid organ transplant. At our center, nine of 327 kidney or liver recipients developed de novo IBD following transplant (six liver, two kidney, one liver-kidney)...
February 2017: Pediatric Transplantation
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