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Metabolic liver disease children

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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
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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-non-alcoholic-fatty-liver-disease-in-obese-children-and-adolescents
#7
Shuang Liang, Xiangdeng Cheng, Yanyan Hu, Ruxin Song, Guimei Li
AIM: Few studies have investigated the relationship between paediatric non-alcoholic 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...
November 27, 2016: Acta Paediatrica
https://www.readbyqxmd.com/read/27878737/sebelipase-alfa-a-review-in-lysosomal-acid-lipase-deficiency
#8
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
#9
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
#10
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
#11
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
https://www.readbyqxmd.com/read/27830542/hepatocyte-transplantation-in-special-populations-clinical-use-in-children
#12
Zahida Khan, Stephen C Strom
Orthotopic liver transplantation remains the only proven cure for end-stage liver failure. Despite significant advances in the field, the clinical demand for donor organs far outweighs the supply. Hepatocyte transplantation has been proposed as an alternative approach to whole liver transplant in select diseases. Several international centers have reported experimental trials of human hepatocyte transplantation in acute liver failure and liver-based metabolic disorders. This chapter provides an introduction to hepatocyte transplantation from both a technical and clinical perspective...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/27822476/pathogenesis-of-nonalcoholic-steatohepatitis-interactions-between-liver-parenchymal-and-nonparenchymal-cells
#13
REVIEW
Nancy Magee, An Zou, Yuxia Zhang
Nonalcoholic fatty liver disease (NAFLD) is the most common type of chronic liver disease in the Western countries, affecting up to 25% of the general population and becoming a major health concern in both adults and children. NAFLD encompasses the entire spectrum of fatty liver disease in individuals without significant alcohol consumption, ranging from nonalcoholic fatty liver (NAFL) to nonalcoholic steatohepatitis (NASH) and cirrhosis. NASH is a manifestation of the metabolic syndrome and hepatic disorders with the presence of steatosis, hepatocyte injury (ballooning), inflammation, and, in some patients, progressive fibrosis leading to cirrhosis...
2016: BioMed Research International
https://www.readbyqxmd.com/read/27799810/novel-treatment-options-for-lysosomal-acid-lipase-deficiency-critical-appraisal-of-sebelipase-alfa
#14
REVIEW
Kim Su, Emma Donaldson, Reena Sharma
Lysosomal acid lipase deficiency (LAL-D) is a rare disorder of cholesterol metabolism with an autosomal recessive mode of inheritance. The absence or deficiency of the LAL enzyme gives rise to pathological accumulation of cholesterol esters in various tissues. A severe LAL-D phenotype manifesting in infancy is associated with adrenal calcification and liver and gastrointestinal involvement with characteristic early mortality. LAL-D presenting in childhood and adulthood is associated with hepatomegaly, liver fibrosis, cirrhosis, and premature atherosclerosis...
2016: Application of Clinical Genetics
https://www.readbyqxmd.com/read/27780972/developmental-origins-of-nafld-a-womb-with-a-clue
#15
REVIEW
Stephanie R Wesolowski, Karim C El Kasmi, Karen R Jonscher, Jacob E Friedman
Changes in the maternal environment leading to an altered intrauterine milieu can result in subtle insults to the fetus, promoting increased lifetime disease risk and/or disease acceleration in childhood and later in life. Particularly worrisome is that the prevalence of NAFLD is rapidly increasing among children and adults, and is being diagnosed at increasingly younger ages, pointing towards an early-life origin. A wealth of evidence, in humans and non-human primates, suggests that maternal nutrition affects the placenta and fetal tissues, leading to persistent changes in hepatic metabolism, mitochondrial function, the intestinal microbiota, liver macrophage activation and susceptibility to NASH postnatally...
October 26, 2016: Nature Reviews. Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/27761562/diverse-etiology-of-hyperlipidemia-among-hospitalized-children-in-western-region-of-saudi-arabia
#16
Abdulmoein E Al-Agha, Abrar M Alnawab, Tala M Hejazi
To determine the various etiologies of primary and secondary hyperlipidemia among children visiting the pediatric endocrine clinic. Methods: This is a retrospective, cross-sectional, cohort study conducted at King Abdulaziz University Hospital (KAUH), Jeddah, Kingdom of Saudi Arabia from January 2010 to 2015 that included 253 children aged from birth to 12 years old. Data were obtained by reviewing medical reports of patients who presented with hyperlipidemia to the clinic, and their laboratory investigation results using KAUH electronic "Phoenix" system...
November 2016: Saudi Medical Journal
https://www.readbyqxmd.com/read/27754444/the-potential-mechanisms-of-berberine-in-the-treatment-of-nonalcoholic-fatty-liver-disease
#17
REVIEW
Xiaopeng Zhu, Hua Bian, Xin Gao
Nonalcoholic fatty liver disease (NAFLD) is a globally observed metabolic disease with high prevalence both in adults and children. However, there is no efficient medication available yet. Increased evidence indicates that berberine (BBR), a natural plant product, has beneficial effects on NAFLD, though the mechanisms are not completely known. In this review, we briefly summarize the pathogenesis of NAFLD and factors that influence the progression of NAFLD, and focus on the potential mechanisms of BBR in the treatment of NAFLD...
October 14, 2016: Molecules: a Journal of Synthetic Chemistry and Natural Product Chemistry
https://www.readbyqxmd.com/read/27738602/associations-among-the-degree-of-nonalcoholic-fatty-liver-disease-metabolic-syndrome-degree-of-obesity-in-children-and-parental-obesity
#18
Min-Su Oh, Sorina Kim, Joon-Hyuck Jang, Jong Yoon Park, Hyun-Sik Kang, Mu Sook Lee, Ki Soo Kang
PURPOSE: To analyze the associations among the degrees of nonalcoholic fatty liver disease (NAFLD) by ultrasonography and metabolic syndrome, degrees of obesity in children, and degrees of parental obesity. METHODS: A total of 198 children with obesity who visited a pediatric obesity clinic were prospectively enrolled in this study. The severity of NAFLD based on ultrasonography was classified into no, mild, moderate, or severe NAFLD group. The degree of obesity based on the percentage over standard weight for height per sex was classified into mild, moderate, or severe...
September 2016: Pediatric Gastroenterology, Hepatology & Nutrition
https://www.readbyqxmd.com/read/27737396/transient-hyperphosphatemia-a-benign-laboratory-disorder-in-a-boy-with-gitelman-syndrome
#19
Sylva Skalova, Stepan Kutilek
Transient hyperphosphatasemia of infancy and early childhood (THI) is characterized by transiently increased activity of serum alkaline phosphatase (S-ALP), predominantly its bone or liver isoform, in children under five years of age. There are no signs of metabolic bone disease or hepatopathy corresponding with the increased S-ALP. THI is benign disorder, rather laboratory than clinical disorder, which is usually accidentally detected in both healthy and sick children. When encountered in a child with either chronic bone, liver or kidney disease, it might concern the physician...
July 2016: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
https://www.readbyqxmd.com/read/27709857/distribution-of-diseases-causing-liver-function-test-abnormality-in-children-and-natural-recovery-time-of-the-abnormal-liver-function
#20
Mirinae Jang, Min Su Oh, Se Cheol Oh, Ki Soo Kang
Although liver function test abnormality is frequently noted in children, there is no report about the distribution of the etiology and natural recovery time of the abnormal liver function. From March 2005 to February 2014, clinical information was retrospectively collected from 559 children who had abnormal liver function and were hospitalized or visited the outpatient clinic at the Jeju National University Hospital. The etiology of abnormal liver function was classified into groups and the natural recovery time of abnormal liver function was analyzed...
November 2016: Journal of Korean Medical Science
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