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Metabolic liver disease children

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https://www.readbyqxmd.com/read/28220684/living-donor-liver-transplantation-during-the-first-three-months-of-life
#1
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
#2
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/28213828/screening-for-non-alcoholic-fatty-liver-disease-in-children-and-adolescents-with-type-1-diabetes-mellitus-a-cross-sectional-analysis
#3
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/28208962/the-effect-of-nutritional-status-on-outcome-of-hospitalization-in-paediatric-liver-disease-patients
#4
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/28197978/managing-cardiovascular-risk-in-lysosomal-acid-lipase-deficiency
#5
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
https://www.readbyqxmd.com/read/28123333/clinical-molecular-and-genetic-evaluation-of-galactosemia-in-turkish-children
#6
Sezen Ugan Atik, Semra Gürsoy, Tuba Koçkar, Hasan Önal, Servet Erdal Adal
AIM: Galactosemia is a carbohydrate metabolism disorder with autosomal recessive inheritance. The most frequent enzyme deficiency is galactose-1-phosphate-uridylytransferase, which causes classic galactosemia. When the enzyme is absent, an infant cannot metabolize galactose-1-phosphate and it cumulates in liver, kidney, brain, tongue, lens, and skin. This study aimed to evaluate the clinical and molecular characteristics of patients with galactosemia, which is observed more frequently in our country than anywhere else in the world...
December 2016: Türk Pediatri Arşivi
https://www.readbyqxmd.com/read/28111909/profile-and-outcome-of-first-109-cases-of-pediatric-acute-liver-failure-at-a-specialized-pediatric-liver-unit-in-india
#7
Seema Alam, Rajeev Khanna, Vikrant Sood, Bikrant Bihari Lal, Dinesh Rawat
BACKGROUND AND AIMS: The outcome of Pediatric acute liver failure largely depends on age and etiology. The aim of this work was to study the etiological spectrum and outcome of the pediatric acute liver failure cases. METHODS: This prospective observational study included all children (< 18 years age) fulfilling pediatric acute liver failure study group definition. Etiological evaluation was done and predictive factors for poor outcome (death or liver transplantation) were analyzed...
January 23, 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/28111263/an-update-on-the-assessment-and-management-of-metabolic-syndrome-a-growing-medical-emergency-in-paediatric-populations
#8
REVIEW
Chiara Mameli, Gian Vincenzo Zuccotti, Carla Carnovale, Erica Galli, Pilar Nannini, Davide Cervia, Cristiana Perrotta
In the last decades the increasing rate of obesity in children and adolescents worldwide has led to the onset in paediatric age of metabolic syndrome, a disease commonly associated to adulthood. Central obesity, dyslipidaemia, hyperglycaemia, and hypertension are typical features of metabolic syndrome that seem to hesitate often in type 2 diabetes, cardiovascular disease, non-alcoholic fatty liver disease, and many other clinical conditions. Thus preventing and curing metabolic syndrome in paediatric patients is becoming an urgent need for public health...
January 19, 2017: Pharmacological Research: the Official Journal of the Italian Pharmacological Society
https://www.readbyqxmd.com/read/28099338/liver-steatosis-in-children-with-chronic-hepatitis-b-and-c-prevalence-predictors-and-impact-on-disease-progression
#9
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/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
#10
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/28009756/regeneration-and-cell-recruitment-in-an-improved-heterotopic-auxiliary-partial-liver-transplantation-model-in-the-rat
#11
Yoshihiro Ono, Angelica Pérez-Gutiérrez, Mladen I Yovchev, Kentaro Matsubara, Shinichiro Yokota, Jorge Guzman-Lepe, Kan Handa, Alexandra Collin de l'Hortet, Angus W Thomson, David A Geller, Hiroshi Yagi, Michael Oertel, Alejandro Soto-Gutierrez
BACKGROUND: Auxiliary partial liver transplantation (APLT) in humans is a therapeutic modality used especially to treat liver failure in children or congenital metabolic disease. Animal models of APLT have helped to explore therapeutic options. Though many groups have suggested improvements, standardizing the surgical procedure has been challenging. Additionally, the question of whether graft livers are reconstituted by recipient-derived cells after transplantation has been controversial...
January 2017: Transplantation
https://www.readbyqxmd.com/read/28007783/early-pqq-supplementation-has-persistent-long-term-protective-effects-on-developmental-programming-of-hepatic-lipotoxicity-and-inflammation-in-obese-mice
#12
Karen R Jonscher, Michael S Stewart, Alba Alfonso-Garcia, Brian C DeFelice, Xiaoxin X Wang, Yuhuan Luo, Moshe Levi, Margaret J R Heerwagen, Rachel C Janssen, Becky A de la Houssaye, Ellen Wiitala, Garrett Florey, Raleigh L Jonscher, Eric O Potma, Oliver Fiehn, Jacob E Friedman
Nonalcoholic fatty liver disease (NAFLD) is widespread in adults and children. Early exposure to maternal obesity or Western-style diet (WD) increases steatosis and oxidative stress in fetal liver and is associated with lifetime disease risk in the offspring. Pyrroloquinoline quinone (PQQ) is a natural antioxidant found in soil, enriched in human breast milk, and essential for development in mammals. We investigated whether a supplemental dose of PQQ, provided prenatally in a mouse model of diet-induced obesity during pregnancy, could protect obese offspring from progression of NAFLD...
December 22, 2016: FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology
https://www.readbyqxmd.com/read/27992361/risk-factors-that-affect-metabolic-health-status-in-obese-children
#13
Selin Elmaogullari, Fatma Demirel, Nihal Hatipoglu
BACKGROUND: While some obese children are metabolically healthy (MHO), some have additional health problems, such as hypertension, dyslipidemia, insulin resistance, and hepatosteatosis, which increase mortality and morbidity related to cardiovascular diseases (CVD) during adulthood. These children are metabolically unhealthy obese (MUO) children. In this study we assessed the factors that affect metabolic health in obesity and the clinical and laboratory findings that distinguish between MHO and MUO children...
January 1, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/27957309/hepatocyte-transplantation-in-children-with-liver-cell-failure
#14
REVIEW
Mohamed Hamooda
Patients with hepatic failure and liver-based metabolic disorders require management which is both costly and complex. Hepatocyte transplantation has been very encouraging as an alternative to organ transplantation for liver disease treatment, and studies in rodents, show that transplants involving isolated liver cells can reverse hepatic failure, and correct various metabolic deficiencies of the liver. This 2016 review is based on a literature search using PubMed including original articles, reviews, cases and clinical guidelines...
October 2016: Electronic Physician
https://www.readbyqxmd.com/read/27941191/diagnostic-and-therapeutic-management-of-children-with-lysosomal-acid-lipase-deficiency-lal-d-review-of-the-literature-and-own-experience
#15
Aldona Wierzbicka-Rucińska, Wojciech Jańczyk, Agnieszka Ługowska, Dariusz Lebensztejn, Piotr Socha
Lysosomal acid lipase deficiency may present at any age (in infants, children and adults). Its presenting features commonly include elevated serum transaminase activity levels, hypercholesterolemia, fatty liver, progressive liver fibrosis, and cirrhosis. Nonspecific clinical manifestations can lead to a delay in the diagnosis of both children and adults. The early development of fibrosis and cirrhosis suggests that the lysosomal accumulation of cholesterol esters and triglycerides in the liver is a potent inducer of fibrosis...
2016: Developmental Period Medicine
https://www.readbyqxmd.com/read/27889912/insulin-like-growth-factor-1-and-metabolic-parameters-are-associated-with-nonalcoholic-fatty-liver-disease-in-obese-children-and-adolescents
#16
Shuang Liang, Xiangdeng Cheng, Yanyan Hu, Ruxin Song, Guimei Li
AIM: Few studies have investigated the relationship between paediatric nonalcoholic fatty liver disease (NAFLD) and insulin-like growth factor 1 (IGF-1). This study, carried out from July 2013 to September 2015, aimed to fill the gap and added metabolic parameters to the analysis. METHODS: This was a cross-sectional study of 168 obese children and adolescents (84% male), divided into two groups based on the presence (n = 90) or absence (n = 78) of NAFLD. All participants underwent clinical examinations, anthropometric and laboratory examinations and liver ultrasonography...
February 2017: Acta Paediatrica
https://www.readbyqxmd.com/read/27878737/sebelipase-alfa-a-review-in-lysosomal-acid-lipase-deficiency
#17
REVIEW
James E Frampton
Sebelipase alfa (Kanuma(®), Kanuma™), the first commercially available recombinant human lysosomal acid lipase (LAL), is approved in various countries worldwide, including those of the EU, the USA and Japan, as a long-term enzyme replacement therapy for patients diagnosed with LAL deficiency (LAL-D), an ultra-rare, autosomal recessive, progressive metabolic liver disease. In an ongoing study in nine infants presenting with early-onset LAL-D (Wolman disease), open-label treatment with sebelipase alfa significantly improved 1-year survival compared with historical controls...
December 2016: American Journal of Cardiovascular Drugs: Drugs, Devices, and Other Interventions
https://www.readbyqxmd.com/read/27875502/sniffing-out-paediatric-gastrointestinal-diseases-the-potential-of-volatile-organic-compounds-as-biomarkers-for-disease
#18
Martin Buijck, Daniel J C Berkhout, Evelien F J de Groot, Marc A Benninga, Marc P C van der Schee, Corneille Marie Frank Kneepkens, Nanne K H de Boer, Tim G J de Meij
The diagnostic work-up and follow-up of paediatric functional gastrointestinal disorders and organic conditions usually includes invasive tests, carrying a high burden on patients. There is a place, therefore, for novel, noninvasive disease-specific biomarkers. Volatile organic compounds (VOCs), originating from (patho)physiological metabolic processes in the human body, are excreted as waste products through all conceivable bodily excrements. The spectrum of VOCs harbours a magnificent source of information, with the potential to serve as noninvasive diagnostic biomarkers and to monitor disease activity...
December 2016: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/27832452/clinical-pharmacokinetics-and-pharmacodynamics-of-antihyperglycemic-medications-in-children-and-adolescents-with-type-2-diabetes-mellitus
#19
REVIEW
Fatemeh Akhlaghi, Kelly L Matson, Amir Hooshang Mohammadpour, Meghan Kelly, Asieh Karimani
The incidence of type 2 diabetes mellitus (T2DM) among children and adolescents has been rising. This condition is associated with obesity, and it's prevalence is higher among minority or female youth. Lifestyle modification including diet and exercise is only successful in a small proportion of patients; therefore, pharmacotherapy approaches are needed to treat T2DM among youth. Currently, in the USA, only metformin and insulin are approved for the treatment of T2DM in children. However, several antihyperglycemic agents including exenatide, glimepiride, glyburide, liraglutide, pioglitazone, and rosiglitazone are also used off-label in this population...
November 10, 2016: Clinical Pharmacokinetics
https://www.readbyqxmd.com/read/27830562/hepatocyte-transplantation-in-children
#20
Louise Coppin, Etienne Sokal, Xavier Stephenne
The liver has an important function in the human body and plays a crucial role in its metabolism. Orthotopic liver transplantation (OLT) is the gold standard treatment for patients presenting liver failure or end stage liver diseases, and is also applied for liver based intractable metabolic disorders. Due to organ shortage, invasive surgery and persistent mortality/morbidity, other treatments have to be explored. Amongst these, hepatocyte transplantation is an attractive alternative and has shown promising results in the treatment of miscellaneous metabolic disorders...
2017: Methods in Molecular Biology
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