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Metabolic liver disease children

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
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
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
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
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
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
Yue-E Wu, Chong-Lin Zhang, Qing Zhen
Metabolic syndrome (MetS) is a cluster of cardiometabolic risk factors, including central obesity, insulin resistance, glucose intolerance, dyslipidemia and increased blood pressure. The prevalence of MetS is on the increase worldwide owing to the epidemic of overweight and obesity. The risk of prevalence of MetS greatly increases during adulthood for those children exposed to cardiometabolic risk factors in their early lives. MetS has also been associated with liver fat accumulation in children. Elevated levels of plasma alanine aminotransferase and γ-glutamyl transferase have been associated with liver fat accumulation...
October 2016: Experimental and Therapeutic Medicine
Maria Grazia Clemente, Claudia Mandato, Marco Poeta, Pietro Vajro
Non-alcoholic fatty liver disease (NAFLD) in children is becoming a major health concern. A "multiple-hit" pathogenetic model has been suggested to explain the progressive liver damage that occurs among children with NAFLD. In addition to the accumulation of fat in the liver, insulin resistance (IR) and oxidative stress due to genetic/epigenetic background, unfavorable lifestyles, gut microbiota and gut-liver axis dysfunction, and perturbations of trace element homeostasis have been shown to be critical for disease progression and the development of more severe inflammatory and fibrotic stages [non-alcoholic steatohepatitis (NASH)]...
September 28, 2016: World Journal of Gastroenterology: WJG
Ryan W Himes, Sarah E Barlow, Kevin Bove, Norma M Quintanilla, Rachel Sheridan, Rohit Kohli
Lysosomal acid lipase deficiency (LAL-D) is a classic lysosomal storage disorder characterized by accumulation of cholesteryl ester and triglyceride. Although it is associated with progressive liver injury, fibrosis, and end-stage liver disease in children and adolescents, LAL-D frequently presents with nonspecific signs that overlap substantially with other, more common, chronic conditions like nonalcoholic fatty liver disease (NAFLD), metabolic syndrome, and certain inherited dyslipidemias. We present 2 children with NAFLD who achieved clinically significant weight reduction through healthy eating and exercise, but who failed to have the anticipated improvements in aminotransferases and γ-glutamyl transferase...
September 13, 2016: Pediatrics
M W Mazumder, A B Karim, M Rukunuzzaman, M A Rahman
Acute liver failure (ALF) is a rapidly progressive, potentially fatal syndrome resulting from rapid death or injury to a large proportion of hepatocytes, caused by a variety of insult, leaving insufficient hepatic paranchymal mass to sustain liver function. The aetiology of ALF varies according to the age of patient and development of the country. The outcome of ALF also varies according to aetiology: survival is better in paracetamol poisoning whereas it is poor in metabolic diseases. The present study was undertaken to observe the underlying aetiology and outcome of ALF in children under 18 years of age admitted at the department of Paediatric Gastroenterology & Nutrition, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh...
July 2016: Mymensingh Medical Journal: MMJ
Mehmet Boyraz, Ediz Yeşilkaya, Fatih Ezgü, Aysun Bideci, Haldun Doğan, Korkut Ulucan, Peyami Cinaz
OBJECTIVE: Although polymorphisms in suppressor of cytokine signaling 3 (SOCS3) was reported to be related to obesity, metabolic syndrome and type 2 diabetes in various adult studies, there is a lack of data in children. In this study, we examined eight reported polymorphisms of SOCS3 in obese Turkish children with and without metabolic syndrome and compared the results with that of controls. METHODS: 148 obese and 63 age and sex matched control children were enrolled in the study...
September 9, 2016: Journal of Clinical Research in Pediatric Endocrinology
Sue Creviston Kaste, Scott E Snyder, Monika L Metzger, John T Sandlund, Scott Howard, Matthew Krasin, Barry L Shulkin
BACKGROUND: Methionine transport across plasma membranes occurs via large amino acid transporter ( LAT1), which is overexpressed in malignant cells leading to tracer accumulation within tumors. We investigated the uptake of (11)C-methionine in children and young adults newly diagnosed with Hodgkin lymphoma (HL) or non-Hodgkin lymphoma (NHL) and compared the biodistribution of (11)C-methionine PET-CTs ((11)C- METPET) to that of (18)F-FDG PET-CT ((18)F-FDGPET). METHODS & MATERIALS: Conducted under an IND, we prospectively enrolled patients with newly diagnosed HL (N = 19) and NHL (N = 2) onto the IRB-approved investigation of (11)C-METPET...
September 8, 2016: Journal of Nuclear Medicine: Official Publication, Society of Nuclear Medicine
Katherine Shedlock, Apryl Susi, Gregory H Gorman, Elizabeth Hisle-Gorman, Christine R Erdie-Lalena, Cade M Nylund
OBJECTIVES: To assess for an increased risk of obesity, type 2 diabetes mellitus, hypertension, hyperlipidemia, and nonalcoholic fatty liver disease/nonalcoholic steatohepatitis in children with autism spectrum disorders (ASD). Additionally, to determine the rates of prescribed treatment for obesity-related metabolic disorders and to determine whether treatment with psychotropic medications is associated with the development of obesity for children with ASD. STUDY DESIGN: A retrospective 1:5 case-control study was performed by use of the Military Health System database from October 2000 to September 2013...
August 31, 2016: Journal of Pediatrics
Donghee Kim, W Ray Kim
Nonalcoholic fatty liver disease (NAFLD) refers to a group of conditions characterized by hepatic steatosis in the absence of significant alcohol consumption. NAFLD is commonly seen in patients with metabolic abnormalities associated with obesity, such as type II diabetes, dyslipidemia, and metabolic syndrome. Evidently, however, not all obese subjects develop NAFLD and, more importantly, NAFLD can be found in non-obese individuals. While NAFLD occurring in non-obese subjects has been reported in children and adults of all ethnicities, it appears to be recognized more frequently in Asians, even when strict ethnicity-specific body mass index criteria are used to define obesity...
August 28, 2016: Clinical Gastroenterology and Hepatology
Antonella Mosca, Claudia Della Corte, Maria Rita Sartorelli, Francesca Ferretti, Francesco Nicita, Andrea Vania, Valerio Nobili
Non-alcoholic fatty liver disease (NAFLD) is the most common chronic liver disease in children and adolescents, due to the increased worldwide incidence of obesity among children. It is now clear enough that of diet high in carbohydrates and simple sugars are associated with hepatic steatosis and non-alcoholic steatohepatitis (NASH). Several studies have shown that an increased consumption of simple sugars is also positively associated with overweight and obesity, and related co-morbidities, such as type 2 diabetes, metabolic syndrome and NAFLD...
August 26, 2016: Eating and Weight Disorders: EWD
Cheng-Yen Chen, Chinsu Liu, Niang-Cheng Lin, Hsin-Lin Tsai, Che-Chuan Loong, Cheng-Yuan Hsia
Hepatic-based metabolic disorders are characterized by an enzyme deficiency expressed solely or mainly in the liver. They are divided into cirrhotic or non-cirrhotic metabolic liver diseases (NCMLDs), and most of them can be treated by liver transplantation. Because the livers with NCMLDs are usually structurally and functionally normal, the primary aim of the liver graft is to support the deficient enzymes rather than maintaining liver functions. Hence, we hypothesize that the exchange of partial liver grafts by the technique of auxiliary partial orthotopic liver transplantation (APOLT) between patients with 2 different NCMLDs may be feasible to replace the deficient enzymes in each patient...
2016: Annals of Transplantation: Quarterly of the Polish Transplantation Society
Aileen Raizner, Nick Shillingford, Paul D Mitchell, Sarah Harney, Roshan Raza, Jessica Serino, Maureen M Jonas, Christine K Lee
OBJECTIVES: Transient elastography (TE) measures liver stiffness to assess fibrosis. Studies in adults have shown that inflammation increases stiffness, leading to an overestimation of fibrosis. We investigated the contribution of inflammation to liver stiffness measurements (LSM) in children/ young adults. METHODS: This was a cohort analysis of children/ young adults who underwent TE within 1 year of liver biopsy. ALT was obtained within 30d of the biopsy and LSM...
August 18, 2016: Journal of Pediatric Gastroenterology and Nutrition
Usha Shrivastava, Anoop Misra, V Mohan, Ranjit Unnikrishnan, D Bachani
Non-communicable diseases (NCDs; including coronary heart disease and type 2 diabetes) are rapidly increasing in India causing nearly 5.8 million deaths per year annually. Primary reasons for rise in NCDs in India are nutrition and lifestyle transitions. Further, presence of higher body fat, abdominal fat, liver and pancreatic fat and lower lean mass than whites, contribute to heightened metabolic and cardiovascular risk in Asian Indians. Importantly, conversion from pre-diabetes to diabetes occurs more rapidly, and reversion to normal glucose regulation with appropriate lifestyle measures is more difficult in Asian Indians than white population...
August 5, 2016: Current Diabetes Reviews
Aneta Gawlik, Michael Shmoish, Michaela F Hartmann, Ewa Malecka-Tendera, Stefan A Wudy, Ze'ev Hochberg
CONTEXT: The profile of urinary steroids as measured by GS-MS defines a subject's 'steroidal fingerprint'. OBJECTIVE: Here, we clustered steroidal fingerprints to characterize patients with non-syndromic childhood obesity by 'steroid metabolomic signatures'. HYPOTHESIS: Non-syndromic obesity is a symptom of different diseases and conditions, some of them will have their own signature. DESIGN: 31 steroid metabolites were quantified by GC-MS and their excretion rates were Z-transformed...
August 9, 2016: Journal of Clinical Endocrinology and Metabolism
Pooja Khandelwal, Tsuyoshi Fukuda, Kana Mizuno, Ashley Teusink-Cross, Parinda A Mehta, Rebecca A Marsh, Angela D M Kashuba, Alexander A Vinks, Stella M Davies
Maraviroc is an allosteric small molecule antagonist of chemokine receptor type 5 (CCR5) and has been used in adult allogeneic hematopoietic stem cell transplant (HSCT) recipients to prevent acute graft-versus-host disease (GVHD) of the gastrointestinal (GI) tract and liver. The goal of this study was to establish feasibility and pharmacokinetic and pharmacodynamic profiles of maraviroc in pediatric HSCT recipients. Children ages 2 to 12 years were enrolled and maraviroc was added to standard GVHD prophylaxis, which included a calcineurin inhibitor and either steroids or mycophenolate mofetil...
October 2016: Biology of Blood and Marrow Transplantation
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