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Liver disease in children

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https://www.readbyqxmd.com/read/28240175/clinical-considerations-of-focal-drug-delivery-in-cancer-treatment
#1
Jamie Harris, Bill Chiu
According to the US Center for Disease Control, cancer deaths are the second most common cause of mortality in both adults and children. Definitive treatment of solid tumors involves surgical resection with or without systemic chemotherapy and radiation. The advent of local drug delivery presents a unique treatment modality that can offer substantial benefits in cancer management. Local drug delivery offers targeted drug delivery to cancer tissues while minimizing side effects of the medications. Three main phases in solid tumor management exist for the treating physician: initial diagnosis with tissue biopsy, surgical resection with or without chemotherapy, and management of metastatic disease...
February 24, 2017: Current Drug Delivery
https://www.readbyqxmd.com/read/28238808/hypophosphatasia-an-overview-for-2017
#2
Michael P Whyte
Hypophosphatasia (HPP) is the inborn-error-of-metabolism that features low serum alkaline phosphatase (ALP) activity (hypophosphatasemia) caused by loss-of-function mutation(s) of the gene that encodes the tissue-nonspecific isoenzyme of ALP (TNSALP). Autosomal recessive or autosomal dominant inheritance from among >300 TNSALP (ALPL) mutations largely explains HPP's remarkably broad-ranging severity. TNSALP is a cell-surface homodimeric phosphohydrolase richly expressed in the skeleton, liver, kidney, and developing teeth...
February 24, 2017: Bone
https://www.readbyqxmd.com/read/28237697/heart-transplantation-in-adults-with-congenital-heart-disease
#3
REVIEW
Lucile Houyel, Ngoc-Tram To-Dumortier, Yannick Lepers, Jérôme Petit, Régine Roussin, Mohamed Ly, Emmanuel Lebret, Elie Fadel, Jürgen Hörer, Sébastien Hascoët
With the advances in congenital cardiac surgery and postoperative care, an increasing number of children with complex congenital heart disease now reach adulthood. There are already more adults than children living with a congenital heart defect, including patients with complex congenital heart defects. Among these adults with congenital heart disease, a significant number will develop ventricular dysfunction over time. Heart failure accounts for 26-42% of deaths in adults with congenital heart defects. Heart transplantation, or heart-lung transplantation in Eisenmenger syndrome, then becomes the ultimate therapeutic possibility for these patients...
February 22, 2017: Archives of Cardiovascular Diseases
https://www.readbyqxmd.com/read/28230607/effects-of-probiotics-on-nonalcoholic-fatty-liver-disease-in-obese-children-and-adolescents
#4
Fatemeh Famouri, Zainab Shariat, Mahin Hashemipour, Mojtaba Keikha, Roya Kelishadi
OBJECTIVES: This study aims to evaluate the effects of some probiotics on sonographic and biochemical nonalcoholic fatty liver disease (NAFLD). METHODS: This randomized triple-blind trial was conducted among 64 obese children with sonographic NAFLD. They were randomly allocated to receive probiotic capsule (containing Lactobacillus acidophilus ATCC B3208, 3 × 10 colony forming units [CFU]; Bifidobacterium lactis DSMZ 32269, 6 × 10 CFU; Bifidobacterium bifidum ATCC SD6576, 2 × 10 CFU; Lactobacillus rhamnosus DSMZ 21690, 2 × 10 CFU) or placebo for 12 weeks...
March 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28230078/unravelling-the-metabolic-impact-of-sbs-associated-microbial-dysbiosis-insights-from-the-piglet-short-bowel-syndrome-model
#5
Prue M Pereira-Fantini, Sean G Byars, James Pitt, Susan Lapthorne, Fiona Fouhy, Paul D Cotter, Julie E Bines
Liver disease is a major source of morbidity and mortality in children with short bowel syndrome (SBS). SBS-associated microbial dysbiosis has recently been implicated in the development of SBS-associated liver disease (SBS-ALD), however the pathological implications of this association have not been explored. In this study high-throughput sequencing of colonic content from the well-validated piglet SBS-ALD model was examined to determine alterations in microbial communities, and concurrent metabolic alterations identified in urine samples via targeted mass spectrometry approaches (GC-MS, LC-MS, FIA-MS) further uncovered impacts of microbial disturbance on metabolic outcomes in SBS-ALD...
February 23, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28229097/pediatric-hypovitaminosis-d-molecular-perspectives-and-clinical-implications
#6
Rafiu Ariganjoye
Vitamin D, a secosteroid, is essential for the development and maintenance of healthy bone in both the adult and pediatric populations. Low level of 25-hydroxy vitamin D (25-(OH)-D) is highly prevalent in children worldwide and has been linked to various adverse health outcomes including rickets, osteomalacia, osteomalacic myopathy, sarcopenia, and weakness, growth retardation, hypocalcemia, seizure and tetany, autism, cardiovascular diseases, diabetes mellitus, cancers (prostate, colon, breast), infectious diseases (viral, tuberculosis), and autoimmune diseases, such as multiple sclerosis and Hashimoto's thyroiditis...
2017: Global Pediatric Health
https://www.readbyqxmd.com/read/28225568/liver-histology-and-diffusion-weighted-mri-in-children-with-nonalcoholic-fatty-liver-disease-a-magnet-study
#7
Paul Manning, Paul Murphy, Kang Wang, Jonathan Hooker, Tanya Wolfson, Michael S Middleton, Kimberly P Newton, Cynthia Behling, Hannah I Awai, Janis Durelle, Melissa N Paiz, Jorge E Angeles, Diana De La Pena, J Allen McCutchan, Jeffrey B Schwimmer, Claude B Sirlin
PURPOSE: To determine potential associations between histologic features of pediatric nonalcoholic fatty liver disease (NAFLD) and estimated quantitative magnetic resonance diffusion-weighted imaging (DWI) parameters. MATERIALS AND METHODS: This prospective, cross-sectional study was performed as part of the Magnetic Resonance Assessment Guiding NAFLD Evaluation and Treatment (MAGNET) ancillary study to the Nonalcoholic Steatohepatitis Clinical Research Network (NASH CRN)...
February 22, 2017: Journal of Magnetic Resonance Imaging: JMRI
https://www.readbyqxmd.com/read/28220684/living-donor-liver-transplantation-during-the-first-three-months-of-life
#8
Mureo Kasahara, Seisuke Sakamoto, Kengo Sasaki, Hajime Uchida, Toshihiro Kitajima, Takanobu Shigeta, Soichi Narumoto, Yoshihiro Hirata, Akinari Fukuda
BACKGROUND: Living donor liver transplantation is now an established technique for treating children with end-stage liver disease. Few data exist about liver transplantation for exclusively young infants, especially infants of <3 months of age. We report our single-center experience with 12 cases in which living donor liver transplantation (LT) was performed during the first 3 months of life and compare the results with those of older infants who underwent LT. All of the patients were treated at the National Center of Child Health and Development, Tokyo, Japan...
February 21, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/28219060/steady-state-therapy-with-azithromycin-or-low-dose-prednisolone-in-paediatric-cystic-fibrosis-patients-inflammatory-markers-and-disease-progression
#9
Galina Shmarina, Alexander Pukhalsky, Lucine Avakian, Sergey Semykin, Daria Pukhalskaya, Vladimir Alioshkin
BACKGROUND: Anti-inflammatory therapy is a logical approach to slowing the inevitable lung function deterioration in cystic fibrosis (CF) patients. This study's aim was to evaluate inflammatory markers and disease progression in paediatric CF patients chronically treated with azithromycin or low-dose prednisolone. METHODS: The study included 204 patients with CF and 100 healthy controls; 102 CF patients were treated with basic therapy only (without anti-inflammatory treatment; WAT), and 102 individuals received basic therapy along with azithromycin (n = 59) or low-dose prednisolone (n = 43)...
February 21, 2017: International Archives of Allergy and Immunology
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
#10
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/28214020/serum-uric-acid-concentrations-and-fructose-consumption-are-independently-associated-with-nash-in-children-and-adolescents
#11
Antonella Mosca, Valerio Nobili, Rita De Vito, Annalisa Crudele, Eleonora Scorletti, Alberto Villani, Anna Alisi, Christopher D Byrne
BACKGROUND & AIMS: Recent research has suggested that dietary fructose intake may increase serum uric acid (UA) concentrations. Both UA concentration and fructose consumption maybe also increase in NAFLD. It is not known whether dietary fructose consumption and UA concentration are independently associated with non-alcoholic steatohepatitis (NASH). Our aim was to investigate the factors associated with NASH in children and adolescents with proven NAFLD, and to test whether UA concentrations and fructose consumption are independently associated with NASH...
February 6, 2017: Journal of Hepatology
https://www.readbyqxmd.com/read/28213931/successful-resumption-of-peritoneal-dialysis-following-living-donor-liver-transplantation-in-children-with-end-stage-renal-disease
#12
Hiroyuki Kanazawa, Akinari Fukuda, Mai Sato, Shingo Ishimori, Kengo Sasaki, Hajime Uchida, Takanobu Shigeta, Vidyadhar Padmakar Mali, Seisuke Sakamoto, Kenji Ishikura, Mureo Kasahara
Children with ESRD in need of RRT are commonly managed by PD due to difficulty with vascular access for HD and the relatively large extracorporeal blood volume required. Major abdominal surgery may result in injury to the peritoneum and consequent adhesion, thereby resulting in a reduction in the anatomical capacity and transport capability across the peritoneal membrane. Here, we report successful resumption of PD after LDLT in two pediatric patients. The causes of ESRD were PH1 and juvenile nephronophthisis, respectively...
February 18, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28213828/screening-for-non-alcoholic-fatty-liver-disease-in-children-and-adolescents-with-type-1-diabetes-mellitus-a-cross-sectional-analysis
#13
Sebastian Kummer, Dirk Klee, Gerald Kircheis, Michael Friedt, Joerg Schaper, Dieter Häussinger, Ertan Mayatepek, Thomas Meissner
: The liver is intensely involved in glucose metabolism and is thereby closely related to diabetes pathophysiology. Adult patients with type 1 diabetes mellitus (DM) are at an increased risk for non-alcoholic fatty liver disease (NAFLD). Here, we studied the prevalence of NAFLD in a cohort of children and adolescents with type 1 DM in a tertiary care paediatric diabetes centre in Germany. We screened 93 children and adolescents with type 1 DM using ultrasound, laboratory investigations, and liver stiffness measurements (Fibroscan® [FS] and acoustic radiation force imaging [ARFI])...
February 17, 2017: European Journal of Pediatrics
https://www.readbyqxmd.com/read/28212920/neonatal-lupus-follow-up-in-infants-with-anti-ssa-ro-antibodies-and-review-of-the-literature
#14
REVIEW
Antonio Alberto Zuppa, Riccardo Riccardi, Simonetta Frezza, Francesca Gallini, Rita Maria Paola Luciano, Giovanni Alighieri, Costantino Romagnoli, Sara De Carolis
Neonatal Lupus Syndrome (NLS) is a distinct clinical entity caused by transplacental passage of maternal anti-SSA/Ro antibodies (Ab). Mothers may have systemic lupus erythematosus, Sjögren syndrome, or other connective tissue disease, or may be completely healthy at the time of giving birth. NLS includes several clinical manifestations: complete congenital heart block (CCHB) and cutaneous lupus are the most common, while hepatobiliary disease, hematological manifestations and central nervous system involvement may occur...
February 14, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28210784/novel-aids-therapies-based-on-gene-editing
#15
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/28209954/-combined-echinococcosis-management-in-children
#16
R Kh Shangareyeva, A A Mirasov, V V Glazyrina, E K Timerbaeva
AIM: To analyze the results of diagnosis and treatment of combined echinococcosis. MATERIAL AND METHODS: The study included 51 children. 40 (78.4%) of them had combined lesion of liver and other organs, 29 - combined echinococcosis of liver and lungs. Echinococcosis of both lungs was observed in 14 children including lesion of liver in 6 cases. Combined echinococcosis of three organs was observed in 5 patients. RESULTS AND DISCUSSION: Combined echinococcosis in children was associated with uneven growth of parasitic cysts in various tissues...
2017: Khirurgiia
https://www.readbyqxmd.com/read/28208962/the-effect-of-nutritional-status-on-outcome-of-hospitalization-in-paediatric-liver-disease-patients
#17
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/28203453/association-of-osteoprotegerin-with-obesity-insulin-resistance-and-non-alcoholic-fatty-liver-disease-in-children
#18
Meltem Erol, Ozlem Bostan Gayret, Hikmet Tekin Nacaroglu, Ozgul Yigit, Oguzhan Zengi, Mehmet Salih Akkurt, Mehmet Tasdemir
BACKGROUND: Osteoprotegerin (OPG) is a member of the tumor necrosis factor superfamily. Reduced OPG levels are related to obesity, insulin resistance, and non-alcoholic fatty liver disease (NAFLD). OBJECTIVES: The aim of this study was to evaluate the relationship between OPG levels, obesity, insulin resistance, and NAFLD in pediatric patients. METHODS: This was a prospective, cross-sectional, controlled study that was conducted in the department of pediatrics at Bagcilar training and research hospital in Istanbul, Turkey, between April and August 2015...
November 2016: Iranian Red Crescent Medical Journal
https://www.readbyqxmd.com/read/28199778/a-national-retrospective-study-of-peld-as-a-predictor-of-change-to-second-line-therapy-in-children-with-autoimmune-hepatitis
#19
Andréanne N Zizzo, Carolina Jimenez-Rivera, Joseph Kim, Richard A Schreiber, Simon C Ling, Jason Yap, Jeff Critch, Najma Ahmed, Fernando Alvarez, Binita M Kamath
BACKGROUND & AIMS: Adult studies of autoimmune hepatitis (AIH) have shown that the model of end-stage liver disease (MELD) is associated with resistance to first-line treatment. Using a multi-centre retrospective database, we sought to determine if the pediatric end-stage liver disease (PELD) score would similarly predict treatment resistance in pediatric AIH. METHODS: One hundred and seventy-one children from 13 Canadian centres who fulfilled the International Autoimmune Hepatitis Group (IAIHG) criteria were included and assessed for change to second-line therapy within 24 months of primary treatment onset...
February 15, 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/28197978/managing-cardiovascular-risk-in-lysosomal-acid-lipase-deficiency
#20
REVIEW
James J Maciejko
Lysosomal acid lipase deficiency (LAL-D) is a rare, life-threatening, autosomal recessive, lysosomal storage disease caused by mutations in the LIPA gene, which encodes for lysosomal acid lipase (LAL). This enzyme is necessary for the hydrolysis of cholesteryl ester and triglyceride in lysosomes. Deficient LAL activity causes accumulation of these lipids in lysosomes and a marked decrease in the cytoplasmic free cholesterol concentration, leading to dysfunctional cholesterol homeostasis. The accumulation of neutral lipid occurs predominantly in liver, spleen, and macrophages throughout the body, and the aberrant cholesterol homeostasis causes a marked dyslipidemia...
February 14, 2017: American Journal of Cardiovascular Drugs: Drugs, Devices, and Other Interventions
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