49 results on '"Nobili, Valerio"'
Search Results
2. Comparison of the Phenotype and Approach to Pediatric vs Adult Patients With Nonalcoholic Fatty Liver Disease
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Nobili, Valerio, Alisi, Anna, Newton, Kimberly P, and Schwimmer, Jeffrey B
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Biomedical and Clinical Sciences ,Clinical Sciences ,Rare Diseases ,Hepatitis ,Chronic Liver Disease and Cirrhosis ,Digestive Diseases ,Pediatric Research Initiative ,Liver Disease ,Pediatric ,2.1 Biological and endogenous factors ,Aetiology ,Good Health and Well Being ,Adult ,Cardiovascular Diseases ,Child ,Comorbidity ,Disease Progression ,Female ,Humans ,Liver ,Male ,Metabolic Syndrome ,Non-alcoholic Fatty Liver Disease ,Phenotype ,Prevalence ,Risk Factors ,Nonalcoholic Fatty Liver Disease ,Nonalcoholic Steatohepatitis ,Children ,Adults ,Neurosciences ,Paediatrics and Reproductive Medicine ,Gastroenterology & Hepatology ,Clinical sciences ,Nutrition and dietetics - Abstract
Nonalcoholic fatty liver disease (NAFLD) is one of the main chronic noncommunicable diseases in Westernized societies; its worldwide prevalence has doubled during the last 20 years. NAFLD has serious health implications not only for adults, but also for children. However, pediatric NAFLD is not only an important global problem in itself, but it is likely to be associated with increases in comorbidities, such as metabolic syndrome and cardiovascular diseases. There are several differences between NAFLD in children and adults, and it is not clear whether the disease observed in children is the initial phase of a process that progresses with age. The increasing prevalence of pediatric NAFLD has serious implications for the future adult population requiring appropriate action. Studies of NAFLD progression, pathogenesis, and management should evaluate disease phenotypes in children and follow these over the patient's lifetime. We review the similarities and differences of NAFLD between children and adults.
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- 2016
3. Epidemiology and natural history of NAFLD
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Della Corte Claudia, Ferrari Federica, Villani Alberto, and Nobili Valerio
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nonalcoholic fatty liver disease ,nonalcoholic steatohepatitis ,children ,obesity ,metabolic syndrome ,Biochemistry ,QD415-436 - Abstract
Paralleling the growing prevalence of obesity and metabolic syndrome, nonalcoholic fatty liver disease (NAFLD) is emerging as the most frequent hepatopathy in adults and children. The true prevalence of pediatric NAFLD is still unknown, because of the heterogeneity of diagnostic methods used for diagnosis in the available studies and the different characteristics of the populations evaluated. Pediatric NAFLD is typically of primary origin and it is strongly associated with several features of the metabolic syndrome. Age, gender and race/ethnicity are significant determinants of risk, and sex hormones, insulin sensitivity and adipocytokines are implicated in the pathogenesis of pediatric NAFLD. The natural history of NAFLD in children is still poorly understood, because of its complex nature and the scarcity of prospective studies, especially in pediatric populations. Both genetic and environmental factors seem to be implicated in the development and progression of the disease via multiple mechanisms that involve liver crosstalk with other organs and tissues, especially gut and adipose tissue. To evaluate and effectively treat pediatric NAFLD, the pathophysiology and natural history of the disease should be clarified and noninvasive methods for screening, diagnosis, and longitudinal assessment developed.
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- 2015
4. Circulating Soluble Fas and Fas Ligand Levels Are Elevated in Children with Nonalcoholic Steatohepatitis
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Alkhouri, Naim, Alisi, Anna, Okwu, Vera, Matloob, Ammar, Ferrari, Federica, Crudele, Annalisa, De Vito, Rita, Lopez, Rocio, Feldstein, Ariel E., and Nobili, Valerio
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- 2015
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5. Non-high-density lipoprotein cholesterol (non-HDL-C) levels in children with nonalcoholic fatty liver disease (NAFLD)
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Alkhouri, Naim, Eng, Katharien, Lopez, Rocio, and Nobili, Valerio
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- 2014
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6. Association between nocturnal blood pressure dipping and insulin resistance in children affected by NAFLD
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Giordano, Ugo, Della Corte, Claudia, Cafiero, Giulia, Liccardo, Daniela, Turchetta, Attilio, Hoshemand, Kazem Mohammad, Fintini, Danilo, Bedogni, Giorgio, Matteucci, Maria Chiara, and Nobili, Valerio
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- 2014
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7. Treatment of nonalcoholic fatty liver disease in adults and children: a closer look at the arsenal
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Nobili, Valerio and Sanyal, Arun J.
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- 2012
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8. Albuminuria and insulin resistance in children with biopsy proven non-alcoholic fatty liver disease
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Manco, Melania, Ciampalini, Paolo, DeVito, Rita, Vania, Andrea, Cappa, Marco, and Nobili, Valerio
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- 2009
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9. Is juvenile liver biopsy unsafe? Putting an end to a common misapprehension
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Pietrobattista, Andrea, Fruwirth, Rodolfo, Natali, Gianluigi, Monti, Lidia, Devito, Rita, and Nobili, Valerio
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- 2009
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10. Wilson's Disease in Children: A Position Paper by the Hepatology Committee of the European Society for Paediatric Gastroenterology, Hepatology and Nutrition
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Socha, Piotr, Janczyk, Wojciech, Dhawan, Anil, Baumann, Ulrich, D'Antiga, Lorenzo, Tanner, Stuart, Iorio, Raffaele, Vajro, Pietro, Houwen, Roderick, Fischler, Björn, Dezsofi, Antal, Hadzic, Nedim, Hierro, Loreto, Jahnel, Jörg, Mclin, Valérie Anne, Nobili, Valerio, Smets, Francoise, Verkade, Henkjan J, and Debray, Dominique
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ddc:618 ,Monitoring ,DNA Mutational Analysis ,Gastroenterology ,Chelating Agents/therapeutic use ,Liver Transplantation ,Physiologic/methods ,Hepatitis ,Treatment ,Copper/metabolism ,Liver ,Liver Function Tests/methods ,Medical ,Diagnosis ,Hepatolenticular Degeneration/diagnosis/therapy ,Humans ,Wilson's disease ,Ceruloplasmin/metabolism ,Liver/pathology ,Child ,Societies ,Children - Abstract
Clinical presentations of Wilson's disease (WD) in childhood ranges from asymptomatic liver disease to cirrhosis or acute liver failure, whereas neurological and psychiatric symptoms are rare. The basic diagnostic approach includes serum ceruloplasmin and 24-hour urinary copper excretion. Final diagnosis of WD can be established using a diagnostic scoring system based on symptoms, biochemical tests assessing copper metabolism, and molecular analysis of mutations in the ATP7B gene. Pharmacological treatment is life-long and aims at removal of copper excess by chelating agents as D-penicillamine, trientine, or inhibition of intestinal copper absorption with zinc salts. Acute liver failure often requires liver transplantation. This publication aims to provide recommendations for diagnosis, treatment, and follow-up of WD in children.
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- 2018
11. Plasma N‐terminal propeptide of type III procollagen accurately predicts liver fibrosis severity in children with non‐alcoholic fatty liver disease.
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Mosca, Antonella, Comparcola, Donatella, Romito, Ilaria, Mantovani, Alessandro, Nobili, Valerio, Byrne, Christopher D., Alisi, Anna, and Targher, Giovanni
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FATTY liver ,COLLAGEN ,RECEIVER operating characteristic curves ,BRAIN natriuretic factor ,FIBROSIS ,ENZYME-linked immunosorbent assay - Abstract
Background & Aims: We examined the diagnostic performance of plasma N‐terminal propeptide of type III procollagen (PIIINP) levels, aspartate aminotransferase to platelet ratio index (APRI) and Fibrosis‐4 (FIB‐4) score for predicting non‐alcoholic steatohepatitis (NASH) and liver fibrosis stage in children/adolescents with non‐alcoholic fatty liver disease (NAFLD). Methods: We enrolled 204 children/adolescents with biopsy‐proven NAFLD at the "Bambino Gesù" Children's Hospital. We measured plasma PIIINP levels using a commercially available enzyme‐linked immunosorbent assay kit and calculated APRI and FIB‐4 scores using standard methods. Results: Children with NASH had higher plasma PIIINP levels, APRI and FIB‐4 scores compared with those without NASH (all P < .001). However, PIIINP levels had much better diagnostic performance and accuracy than APRI and FIB‐4 scores for predicting liver fibrosis stage. PIIINP levels correlated with the total NAFLD activity score (NAS) and its constituent components (P < .0001). The risk of either NASH or F ≥ 2 fibrosis progressively increased with increasing PIIINP levels (P < .0001), independent of age, gender, adiposity measures, insulin resistance, NAS score and the patatin‐like phospholipase domain‐containing protein‐3 rs738409 polymorphism. For every 3.6 ng/mL increase in PIIINP levels, the likelihood of having F ≥ 2 fibrosis increased by ~14‐fold (adjusted‐odds ratio 14.1, 95% CI 5.50‐35.8, P < .0001) after adjustment for the aforementioned risk factors. The area under the receiver operating characteristics curve was 0.921 (95% CI 0.87‐0.97) for F ≥ 2 fibrosis, and 0.993 (95% CI 0.98‐1.0) for F3 fibrosis respectively. Conclusions: Unlike APRI and FIB‐4 scores, plasma PIIINP levels are a promising, non‐invasive biomarker for diagnosing liver fibrosis stage in children/adolescents with biopsy‐proven NAFLD. [ABSTRACT FROM AUTHOR]
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- 2019
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12. The Liver in Children With Metabolic Syndrome.
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D'Adamo, Ebe, Castorani, Valeria, and Nobili, Valerio
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FATTY liver ,METABOLIC syndrome ,INSULIN resistance ,CHILDREN ,ADOLESCENCE ,OBESITY - Abstract
Non-alcoholic fatty liver disease (NAFLD) is recognized as an emerging health risk in obese children and adolescents. NAFLD represents a wide spectrum of liver conditions, ranging from asymptomatic steatosis to steatohepatitis. The growing prevalence of fatty liver disease in children is associated with an increased risk of metabolic and cardiovascular complications. NAFLD is considered the hepatic manifestation of Metabolic Syndrome (MetS) and several lines of evidence have reported that children with NAFLD present one or more features of MetS. The pathogenetic mechanisms explaining the interrelationships between fatty liver disease and MetS are not clearly understood. Altough central obesity and insulin resistance seem to represent the core of the pathophysiology in both diseases, genetic susceptibility and enviromental triggers are emerging as crucial components promoting the development of NAFLD and MetS in children. In the present review we have identified and summarizied studies discussing current pathogenetic data of the association between NAFLD and MetS in children. [ABSTRACT FROM AUTHOR]
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- 2019
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13. The Antioxidant Effects of Hydroxytyrosol and Vitamin E on Pediatric Nonalcoholic Fatty Liver Disease, in a Clinical Trial: A New Treatment?
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Nobili, Valerio, Alisi, Anna, Mosca, Antonella, Crudele, Annalisa, Zaffina, Salvatore, Denaro, Marcella, Smeriglio, Antonella, and Trombetta, Domenico
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FATTY liver , *VITAMIN E , *THERAPEUTICS , *ADVANCED glycation end-products , *HYDROXYTYROSOL , *CLINICAL trials - Abstract
Nonalcoholic fatty liver disease (NAFLD) is the most common cause of chronic liver disease in children. Several studies suggest that the improvement of oxidative stress is suggested as a possible therapeutic strategy for pediatric nonalcoholic steatohepatitis. We performed a randomized, double-blind placebo-controlled trial to test the potential efficacy, assessed by improvement of oxidative stress parameters and liver ultrasound, and tolerability of a mixture of vitamin E and hydroxytyrosol (HXT) in adolescents with biopsy-proven NAFLD. Four hundred forty consecutive patients were screened, 80 of these with biopsy-proven NAFLD were enrolled. Forty patients received an oral dose of HXT and vitamin E and 40 children received the capsules of placebo for 4 months. Seventy patients completed the study. Patients in the treatment arm showed a decrease of insulin resistance (IR), triglyceride levels, oxidative stress parameters, and steatosis grade. Noteworthy, the steatosis improvement correlates with the levels of advanced glycation end products and carbonylated proteins. The HXT and vitamin E treatment improved the main oxidative stress parameters, IR, and steatosis in children with NAFLD. The use of two natural molecules that may have antioxidant effects seems a promising strategy that could be easily diet integrated to improve NAFLD-related liver damage in children. [ABSTRACT FROM AUTHOR]
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- 2019
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14. Eosinophilic esophagitis in children: current knowledge to open new horizons: Eosinophilic esophagitis in pediatrics.
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Alterio, Tommaso, Cardile, Sabrina, Trayers, Claire, Valenti, Simona, Loddo, Italia, Mardare, Roxana, Mosca, Antonella, and Nobili, Valerio
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GASTROESOPHAGEAL reflux ,EOSINOPHILIC esophagitis ,PEDIATRICS ,ESOPHAGUS diseases ,CHILDREN ,INFLAMMATION ,GASTROINTESTINAL diseases - Abstract
Eosinophilic Esophagitis (EoE) is a chronic immune/antigen-mediated condition which is also driven by genetic and environmental factors. It has been deeply investigated over the last years and its incidence is widely increasing in childhood. Although atopic diseases are closely linked with EoE, it does not recognize a classical IgE-mediate immune pathogenesis but it is rather a T helper type 2 inflammatory process. Familial clustering supports genetic predisposition in EoE and recent advances in understanding the genetic basis for EoE may eventually translate into targeted management of the disease. EoE diagnosis is based on clinical symptoms, micro, and macroscopic findings along with exclusion of gastroesophageal reflux disease (GERD) evidence. Management of the disease encompasses both dietary and pharmacological solutions that need to be specifically targeted on patients' history, clinical symptoms, and diagnostic evaluations. New therapies, currently not available in children, may represent the basis for future therapeutic options in the next years. [ABSTRACT FROM AUTHOR]
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- 2019
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15. Relationship between non‐alcoholic steatohepatitis, PNPLA3 I148M genotype and bone mineral density in adolescents.
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Mosca, Antonella, Nobili, Valerio, Fintini, Danilo, Cappa, Marco, Paone, Laura, Scorletti, Elenora, Byrne, Christopher D., and Zicari, Anna M.
- Abstract
Background & Aims: It is uncertain whether non‐alcoholic steatohepatitis (NASH) is a risk factor for low bone mineral density (BMD). Our aim was to investigate: (a) associations between NASH and BMD values and (b) associations between PNPLA3 I148M genotypes and BMD, in children with histologically proven non‐alcoholic fatty liver disease (NAFLD). Methods: BMD area (g/cm2) was measured using dual‐energy X‐ray absorptiometry (DEXA). NASH was diagnosed by a Steatosis, Activity and Fibrosis (SAF) score and FLIP algorithm. Genotyping for patatin‐like phospholipase domain containing‐3 (PNPLA3) I148M genotype (rs738409) (CC, CG and GG) was undertaken using the TaqMan SNP genotyping allelic discrimination method. Logistic regression was used to test associations [OR (95% CIs)] between low BMD, and both NASH and PNPLA3 I148M genotypes. Results: Thirty‐four adolescents (mean age 13.8 ± 1.1 years) with histologically confirmed NAFLD were studied. Subjects with NASH (n = 25) had a lower BMD (means (SDs) 0.87 ± 0.06 vs 0.97 ± 0.12, P = 0.005), compared to subjects without NASH. Subjects with PNPLA3 CG+GG genotypes had a lower BMD compared with subjects with PNPLA3‐CC genotype (means (SDs) 0.79 ± 0.20 vs 0.92 ± 0.10, P = 0.009). PNPLA3 CG+GG genotypes were independently associated with NASH [OR (95% CIs 1.78, 1.24, 2.99)], and low BMD was associated with both PNPLA3 CG+GG (OR 3.62 (95% CIs 1.21, 5.53), P = 0.028) and with SAF score (OR 2.76 (95% CIs 1.12, 5.41), P = 0.045). Conclusions: Taken together the independent associations between: (a) low BMD and PNPLA3 CG+GG genotype; (b) low BMD and NASH; and (c) PNPLA3 CG+GG genotype and NASH, provide support for a causal relationship between NASH and low BMD. [ABSTRACT FROM AUTHOR]
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- 2018
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16. Pancreatic disorders in children: New clues on the horizon.
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Della Corte, Claudia, Faraci, Simona, Majo, Fabio, Lucidi, Vincenzina, Fishman, Douglas S., and Nobili, Valerio
- Abstract
Abstract Pancreatic disorders in children represent a growing health problem in pediatric patients. In the past two decades, several advances have been made in the knowledge of pediatric pancreatic disorders, with better understanding of different etiologies and clinical manifestations of these disorders. Moreover, many efforts have been made in pancreatology, aiming to define guidelines in the management of pancreatitis in children, initially based on the available information in adults. A multidisciplinary and multicenter approach is necessary to better determine pancreatic disease pathways and treatment options in children. [ABSTRACT FROM AUTHOR]
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- 2018
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17. Hepatic farnesoid X receptor protein level and circulating fibroblast growth factor 19 concentration in children with NAFLD.
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Nobili, Valerio, Mosca, Antonella, Della Corte, Claudia, Veraldi, Silvio, Alisi, Anna, De Stefanis, Cristiano, De Vito, Rita, D'Oria, Valentina, Jahnel, Joerg, Zohrer, Evelyn, Scorletti, Eleonora, and Byrne, Christopher D.
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FATTY liver , *FARNESOID X receptor , *FIBROBLAST growth factors , *BILE acids , *JUVENILE diseases , *LIVER histology , *GENETICS - Abstract
Abstract: Background & Aims: Treatment with the farnesoid X receptor (FXR) agonist obeticholic acid is ineffective in some patients with non‐alcoholic steatohepatitis (NASH) but the explanation is uncertain. We investigated hepatic FXR expression, and measurements of fibroblast growth factor 19 (FGF19) and bile acids (BAs) in children with NAFLD to investigate relationships with NASH. Methods: 33 children with NAFLD who underwent diagnostic liver biopsy were studied. Hepatic FXR protein levels and circulating FGF19 concentrations were compared with those analysed in five control subjects with proven normal liver histology. NASH was defined by the Paediatric NAFLD Histological Score (PNHS). Binary logistic regression with adjustment for covariates and potential confounders was undertaken to test factors independently associated with: a) NASH and b) hepatic FXR protein levels. Results: Mean ± SD age was 13.7 ± 1.9 years. Nineteen patients had NASH (PNHS ≥ 85) and 14 did not have NASH (PNHS < 85). Hepatic FXR level and plasma FGF19 concentration varied ~10‐fold and 5‐fold, respectively, between groups, and was highest in control subjects, intermediate in NAFLD without NASH, and lowest in NASH (between group differences
P < .001 andP < .01 respectively). NASH was independently associated with both FXR protein levels (OR = 0.18, 95% CI 0.09, 0.38) and FGF19 concentration (OR = 0.55, 95% CI 0.20, 0.89). Conclusions: FXR protein levels vary markedly between normal liver, NAFLD without NASH, and NASH. Low levels of FXR are independently associated with NASH. [ABSTRACT FROM AUTHOR]- Published
- 2018
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18. Nonalcoholic Fatty Liver Disease in Children.
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Mann, Jake P., Valenti, Luca, Scorletti, Eleonora, Byrne, Christopher D., and Nobili, Valerio
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FATTY liver ,HEPATIC fibrosis ,LIFESTYLES & health ,METABOLISM ,CIRRHOSIS of the liver ,IMMUNOLOGY - Abstract
Nonalcoholic steatohepatitis, a progressive form of nonalcoholic fatty liver disease (NAFLD), is one of the most common hepatic diseases in children who present with particular risk factors including obesity, sedentary lifestyle, and/or a predisposing genetic background. The worldwide prevalence of NAFLD in children is a worrying phenomenon because this disease is closely associated with the development of both cirrhosis and cardiometabolic syndrome in adulthood. To date, the etiopathogenesis of primary NAFLD in children is unknown. Understanding the pathogenetic mechanisms provides the basis to characterize early predictors of the disease and noninvasive diagnostic tools and to design novel specific treatments and possible management strategies. Despite a few clinical trials on the use of antioxidants combined with lifestyle intervention for NAFLD, no treatment exists for children with NAFLD. In this review, the authors provide an overview of current concepts in epidemiology, histological features, etiopathogenesis, diagnosis and treatment of NAFLD in pediatric population. [ABSTRACT FROM AUTHOR]
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- 2018
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19. Efficacy of docosahexaenoic acid-choline-vitamin E in paediatric NASH: a randomized controlled clinical trial.
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Zöhrer, Evelyn, Alisi, Anna, Jahnel, Jörg, Mosca, Antonella, Della Corte, Claudia, Crudele, Annalisa, Fauler, Günter, and Nobili, Valerio
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BLOOD sugar analysis ,THERAPEUTICS ,FATTY liver ,TREATMENT effectiveness ,BEHAVIOR modification ,CHOLINE ,CLINICAL trials ,DIETARY supplements ,GROWTH factors ,HEALTH behavior ,PROBABILITY theory ,STATISTICAL sampling ,VITAMIN E ,DOCOSAHEXAENOIC acid ,STATISTICAL significance ,ALANINE aminotransferase ,RANDOMIZED controlled trials ,DESCRIPTIVE statistics ,CHILDREN - Abstract
Copyright of Applied Physiology, Nutrition & Metabolism is the property of Canadian Science Publishing and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
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- 2017
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20. Enteral nutrition in pediatric intestinal failure: does initial feeding impact on intestinal adaptation?
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Capriati, Teresa, Nobili, Valerio, Stronati, Laura, Cucchiara, Salvatore, Laureti, Francesca, Liguori, Alessandra, Tyndall, Elaine, and Diamanti, Antonella
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INTESTINAL diseases ,SHORT bowel syndrome ,EPITHELIAL cells ,ENTERAL feeding ,PROTEINS - Abstract
Introduction: Primary IF can be due to impaired gut length or impaired gut function; short bowel syndrome (SBS) is the leading cause of IF. In IF patients complete enteral starvation should be avoided whenever possible and enteral/oral nutrition (EN/ON) should be employed at the maximum tolerated amount in each phase of the clinical evolution of IF. Intraluminal nutrients have stimulatory effects on epithelial cells and on trophism that enhance intestinal adaptation. Areas covered: Evidence for nutritional interventions in pediatric IF is limited and of poor quality. Clinical practice in SBS feeding are more ‘experience-based’ rather than ‘evidence-based’ and this dearth of clinical evidence is partly due to the rarity of this condition. This review updates knowledge concerning the impact of the initial diet with EN/ON in neonatal onset SBS in the process of bowel adaption. Expert commentary: Human milk resulted the preferred starting diet and it is generally combined with amino-acids (AAs) in Northern America and with hydrolyzed proteins (HFs) in Europe; polymeric diet is rarely employed. HFs were not more effective than AAs in promoting intestinal adaptation. [ABSTRACT FROM AUTHOR]
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- 2017
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21. Pediatric liver diseases: current challenges and future perspectives.
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Della Corte, Claudia, Mosca, Antonella, Vania, Andrea, Alterio, Arianna, Alisi, Anna, and Nobili, Valerio
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LIVER diseases ,JUVENILE diseases ,THERAPEUTICS ,FATTY liver ,VIRAL hepatitis ,AUTOIMMUNE disease treatment - Abstract
Chronic liver diseases in children represent a rising problem with significant effects on public health. In fact, several pediatric liver diseases are precursors of adult chronic hepatopathies, cirrhosis and hepatocellular carcinoma. The prevalence of liver diseases in children is unknown. In the USA, every year, 15,000 children are hospitalized for liver diseases, but these disorders continue to be under-recognized or diagnosed late. The main reason is due to the frequent absence of symptoms in the vast majority of liver diseases, especially in the early stages. In the last few decades several advances have been made in understanding the pathogenesis of liver diseases, permitting the discovery of new therapeutic targets to treat liver diseases, thus improving the natural history of these disorders. In this article we discuss the most recent advances in the understanding of the pathogenesis, diagnosis and treatment of the most frequent pediatric liver diseases. [ABSTRACT FROM PUBLISHER]
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- 2016
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22. A non-invasive prediction model for non-alcoholic steatohepatitis in paediatric patients with non-alcoholic fatty liver disease.
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Eng, Katharine, Lopez, Rocio, Liccardo, Daniela, Nobili, Valerio, and Alkhouri, Naim
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Background Non-alcoholic fatty liver disease encompasses a spectrum of diseases that range from simple steatosis to the aggressive form of non-alcoholic steatohepatitis. Non-alcoholic steatohepatitis is currently diagnosed through liver biopsy. Aim To develop a non-invasive predictive model of non-alcoholic steatohepatitis in children with non-alcoholic fatty liver disease. Methods Anthropometric, laboratory, and histologic data were obtained in a cohort of children with biopsy-proven non-alcoholic fatty liver disease. Multivariable logistic regression analysis was employed to create a nomogram predicting the risk of non-alcoholic steatohepatitis. Internal validation was performed by bootstrapping. Results Three hundred and two children were included in this analysis with a mean age of 12.3 ± 3.1 years, a mean body mass index percentile of 94.3 ± 6.9, and non-alcoholic steatohepatitis was present in 67%. Following stepwise variable selection, total cholesterol, waist circumference percentile, and total bilirubin were included as variables in the model, with good discrimination with an area under the receiver operating characteristic curve of 0.737. Conclusions A nomogram was constructed with reasonable accuracy that can predict the risk of non-alcoholic steatohepatitis in children with non-alcoholic fatty liver disease. If validated externally, this tool could be utilized as a non-invasive method to diagnose non-alcoholic steatohepatitis in children with non-alcoholic fatty liver disease. [ABSTRACT FROM AUTHOR]
- Published
- 2014
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23. Epidemiology and Natural History of Nafld/Epidemiologija I Prirodna Istorija Nealkoholne Masne Jetre.
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Corte, Claudia Della, Ferrari, Federica, Villani, Alberto, and Nobili, Valerio
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OBESITY ,FATTY liver ,EPIDEMIOLOGY ,LIVER diseases ,FATTY degeneration ,DISEASE prevalence ,METABOLIC syndrome - Abstract
Copyright of Journal of Medical Biochemistry is the property of Society of Medical Biochemists of Serbia and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
- Published
- 2014
- Full Text
- View/download PDF
24. Non-Alcoholic Fatty Liver and Metabolic Syndrome in Children: A Vicious Circle.
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Alterio, Arianna, Alisi, Anna, Liccardo, Daniela, and Nobili, Valerio
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METABOLIC syndrome ,METABOLIC disorders in children ,INSULIN resistance ,FATTY liver ,PEDIATRICS ,CIRRHOSIS of the liver ,FATTY degeneration - Abstract
During the last decade, paediatricians have observed a dramatic increase of non-alcoholic fatty liver disease (NAFLD) and metabolic syndrome (MS) in children. Furthermore, several lines of evidence have reported that a large part of children with NAFLD presents one or more traits of MS making plausible that, in the coming years, these subjects may present a rapid course of disease towards more severe cirrhosis and cardiovascular disease. Genetic susceptibility and the pressure of intrauterine environment and lifestyle are all crucial to activate molecular machinery that leads to development of NAFLD and MS in childhood. In this scenario, central obesity and consequent adipose tissue inflammation are critical to promote both MS-associated metabolic dysfunctions and NAFLD-related hepatic damage. An excessive dietary intake may in fact cause a specific lipid partitioning and induce metabolic stressors, which in turn promote insulin resistance and the release of several circulating factors. These molecules, on the one hand, trigger steatosis and the inflammatory response that characterize liver damage in NAFLD, and on the other hand contribute to the onset of other features of MS. This review provides an overview of current genetic, pathogenetic and clinical evidence of the vicious circle created by NAFLD and MS in children. © 2014 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]
- Published
- 2014
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25. Management of chronic hepatitis B in children: An unresolved issue.
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Della Corte, Claudia, Nobili, Valerio, Comparcola, Donatella, Cainelli, Francesca, and Vento, Sandro
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CHRONIC hepatitis B , *LIVER cancer , *CIRRHOSIS of the liver , *DISEASE risk factors , *LYMPHOKINES - Abstract
Although a rather benign course of chronic hepatitis B virus ( HBV) infection during childhood has been described, 3-5% and 0.01-0.03% of chronic carriers develop cirrhosis or hepatocellular carcinoma before adulthood. Considering the whole lifetime, the risk of hepatocellular carcinoma rises to 9-24% and the incidence of cirrhosis to 2-3% per year. The aim of this article is to review the current knowledge regarding the natural history and treatment of chronic hepatitis B in children and to focus on critical aspects and unresolved questions in the management of childhood HBV infection. A literature search was carried out on MEDLINE, EMBASE, and Web of Science for articles published in English in peer-reviewed journals from January 1980 to February 2013. The search terms used included 'Hepatitis B virus infection,' 'children,' ' HBV,' 'interferon,' 'lamivudine,' 'adefovir,' 'entecavir,' and 'tenofovir.' Articles resulting from these searches and relevant references cited in the articles retrieved were reviewed. The current goals of therapy are to suppress viral replication, reduce liver inflammation, and reverse liver fibrosis. Therapeutic options for children are currently limited, and the risk for viral resistance to current and future therapies is a particular concern. Based on the data available at this time, it is the consensus of the panel that it is not appropriate to treat children in the immune-tolerant phase or in the inactive carrier state. For children in the immune-active or reactivation phases, liver histology can help guide treatment decisions. Outside of clinical trials, interferon is the agent of choice in most cases; currently, available nucleoside analogs are secondary therapies. [ABSTRACT FROM AUTHOR]
- Published
- 2014
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26. Does Vitamin E Improve the Outcomes of Pediatric Nonalcoholic Fatty Liver Disease? A Systematic Review and Meta‑analysis.
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Sarkhy, Ahmed A., Al‑Hussaini, Abdulrahman A., and Nobili, Valerio
- Subjects
THERAPEUTIC use of antioxidants ,THERAPEUTIC use of vitamin E ,CONFIDENCE intervals ,FATTY liver ,MEDICAL databases ,INFORMATION storage & retrieval systems ,MEDICAL information storage & retrieval systems ,MEDLINE ,META-analysis ,ONLINE information services ,RESEARCH funding ,SYSTEMATIC reviews ,ALANINE aminotransferase ,RANDOMIZED controlled trials ,RELATIVE medical risk ,CHILDREN - Abstract
Background and Aims: To systemically evaluate the efficacy of adjuvant vitamin E on the outcomes of nonalcoholic fatty liver disease (NAFLD) and/or nonalcoholic steatohepatitis (NASH) in children. Materials and Methods: We searched MEDLINE, PUBMED, EMBASE, the Cochrane Central Register Controlled Trials, and the Cochrane Database of Systematic Reviews over the period between January 1980 and September 2012 for the studies that examined the role of adjuvant vitamin E given at any dose or duration, alone or in combination with other interventions, on the outcome of pediatric NAFLD. The outcomes are alanine aminotransferase (ALT) normalization and histological improvement. Results: Five randomized trials were eligible to be included in our analysis, with a total of 270 participants. There was no statistically significant difference in the effect of adjuvant vitamin E on normalizing serum ALT [risk ratio (RR) =1.18, confidence interval (CI) =0.92-1.53, P = 0.77 for heterogeneity, I2 = 0%]. Sensitivity analysis showed that using higher doses of vitamin E, a longer duration of therapy or adding vitamin C did not change the effect on the measured outcome. Only two studies looked at histological changes as an outcome. We observed substantial heterogeneity between the two studies. Conclusions: Our meta‑analysis did not find a significant effect of adjuvant vitamin E over placebo in normalizing serum ALT. Data on the long‑term effect of adjuvant vitamin E on histological improvements in NAFLD patients are still lacking. Larger, well‑designed randomized controlled trials (RCTs) in children with histological endpoints are still needed to answer this question. [ABSTRACT FROM AUTHOR]
- Published
- 2014
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27. Non-alcoholic fatty liver disease.
- Author
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Della Corte, Claudia, Liccardo, Daniela, Mosca, Antonella, Vania, Andrea, and Nobili, Valerio
- Subjects
THERAPEUTICS ,OBESITY complications ,FATTY liver ,METABOLIC syndrome ,SEDENTARY lifestyles ,DISEASE complications ,CHILDREN - Abstract
Abstract: Non-alcoholic fatty liver disease is rapidly becoming one of most common liver diseases in the paediatric population in industrialized countries due to the growing prevalence of obesity and overweight. Paediatric NAFLD is typically of primary origin and it is strongly associated with several features of the metabolic syndrome. The mainstay of NAFLD therapy is represented by lifestyle interventions on obesogenic environment and sedentary lifestyle, which aim to improve obesity, hepatic changes and quality of life as well. Unfortunately, this goal is very difficult to be achieved and pharmacological approaches have become necessary. Although in fairly recent years, many pharmacological agents, on the basis of pathogenetic mechanism of the disease, have been attempted, to date guidelines for the management of fatty liver are still lacking. Establishing effective therapeutic strategies to treat the disease represents the challenge for paediatric hepatologists in the near future. In this article, we briefly review the current knowledge and ideas concerning paediatric non-alcoholic fatty liver disease. [Copyright &y& Elsevier]
- Published
- 2013
- Full Text
- View/download PDF
28. The I148M Variant of PNPLA3 Reduces the Response to Docosahexaenoic Acid in Children with Non-Alcoholic Fatty Liver Disease.
- Author
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Nobili, Valerio, Bedogni, Giorgio, Donati, Benedetta, Alisi, Anna, and Valenti, Luca
- Subjects
- *
THERAPEUTICS , *FATTY liver , *ALLELES , *CHILDREN'S health , *CHILD nutrition , *CLINICAL trials , *CONFIDENCE intervals , *DIETARY supplements , *GENES , *LONGITUDINAL method , *HEALTH outcome assessment , *STATISTICAL sampling , *DOCOSAHEXAENOIC acid , *SECONDARY analysis , *RANDOMIZED controlled trials , *TREATMENT effectiveness , *DESCRIPTIVE statistics , *CHILDREN - Abstract
The aim of this secondary analysis of a randomized controlled trial was to test whether the I148M variant of Patatin-like phospholipase domain-containing protein-3 (PNPLA3) is associated with the response to docosahexaenoic acid (DHA) in children with non-alcoholic fatty liver disease (NAFLD). Sixty children with NAFLD were randomized in equal numbers to DHA 250 mg/day, DHA 500 mg/day or placebo. Coherently with the primary analysis, the probability of more severe steatosis after 24 months of DHA supplementation was 50% lower [95% confidence interval (CI) −59% to −42%)] in the combined DHA 250 and 500 mg/day groups versus placebo. The present secondary analysis revealed an independent effect of PNPLA3 status on the response to DHA. In fact, the probability of more severe steatosis was higher (37%, 95% CI 26-48%) for the PNPLA3 M/M versus I/M genotype and lower (−12%, 95% CI −21% to −3%) for the I/I versus I/M genotype (Somers' D for repeated measures). We conclude that the 148M allele of PNPLA3 is associated with lower response, and the 148I allele with greater response, to DHA supplementation in children with NAFLD. [ABSTRACT FROM AUTHOR]
- Published
- 2013
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- View/download PDF
29. A 360-degree overview of paediatric NAFLD: Recent insights
- Author
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Nobili, Valerio, Svegliati-Baroni, Gianluca, Alisi, Anna, Miele, Luca, Valenti, Luca, and Vajro, Pietro
- Subjects
- *
FATTY degeneration , *PEDIATRICS , *FIBROSIS , *JUVENILE diseases , *FATTY liver , *OBESITY , *DIAGNOSIS - Abstract
Summary: Non-alcoholic fatty liver disease (NAFLD) is a multi-faceted disorder, which ranges from simple steatosis to non-alcoholic steatohepatitis (NASH) with/without fibrosis. The effects of specific risk factors, such as obesity and sedentary lifestyle, on predisposing genetic settings eventually lead to the development of NAFLD in children. The complex interplay between genes and environment in NAFLD pathogenesis is sustained by multiple mechanisms that involve liver crosstalk with other organs and tissues, especially gut and adipose tissue. Unfortunately, natural history of paediatric NAFLD is lacking, and the etiopathogenesis is still in the process of being defined. Potential early predictors and suitable non-invasive diagnostic tools can be discovered based on the pathogenetic mechanisms and histological patterns. This will also help design novel treatments and a comprehensive and successful management strategy for patients. In this review, we discuss the recent advances made in genetics, etiopathogenesis, diagnosis, and therapeutic management of NAFLD, focusing especially on the obesity-related steatotic liver condition. [ABSTRACT FROM AUTHOR]
- Published
- 2013
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30. Combined paediatric NAFLD fibrosis index and transient elastography to predict clinically significant fibrosis in children with fatty liver disease.
- Author
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Alkhouri, Naim, Sedki, Emad, Alisi, Anna, Lopez, Rocio, Pinzani, Massimo, Feldstein, Ariel E., and Nobili, Valerio
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FATTY liver ,FIBROSIS ,GENETIC markers ,PEDIATRIC diagnosis ,RECEIVER operating characteristic curves - Abstract
Background Nonalcoholic fatty liver disease ( NAFLD) encompasses a spectrum of disease from simple steatosis to steatohepatitis, to fibrosis and cirrhosis. The paediatric NAFLD fibrosis index ( PNFI) and transient elastography ( TE) are potential noninvasive markers for fibrosis. To prospectively evaluate the performance of PNFI and TE in assessing clinically significant fibrosis in children with biopsy-proven NAFLD. Methods Our cohort consisted of 67 consecutive children with biopsy-proven NAFLD. The stage of fibrosis was scored according to the Nonalcoholic Steatohepatitis Clinical Research Network. Fibrosis ≥ 2 was considered clinically significant. PNFI was calculated using age, waist circumference and triglycerides. TE was performed using the Fibroscan apparatus. Results Ten patients had fibrosis stage 2-3 and 57 patients had stage 0-1. Both PNFI and TE values were significantly higher in patients with significant fibrosis ( P < 0.05). The area under the receiver operating characteristic ( ROC) curve for predicting significant fibrosis of PNFI and TE were 0.747 and 1.00 respectively ( P = 0.005). The combined use of PNFI and TE could predict the presence or absence of clinically significant fibrosis in 98% of children with NAFLD. Conclusions In children with NAFLD, the combination of PNFI and TE can be used to accurately assess the presence of clinically significant liver fibrosis. This will help to identify patients who should undergo liver biopsy because the confirmation of advanced fibrosis would lead to closer follow-up and screening for cirrhosis-related complications. [ABSTRACT FROM AUTHOR]
- Published
- 2013
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- View/download PDF
31. Nonalcoholic Fatty Liver in Children and Adolescents: An Overview.
- Author
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Della Corte, Claudia, Alisi, Anna, Saccari, Alessia, De Vito, Rita, Vania, Andrea, and Nobili, Valerio
- Abstract
Abstract: Nonalcoholic fatty liver disease is rapidly becoming one of the most common liver diseases in the pediatric population in industrialized countries because of the growing prevalence of obesity and overweight. For this reason, there is a keen and broad interest among researchers to identify new diagnostic noninvasive tools and novel treatment modalities for this condition. Unfortunately, to date, liver biopsy remains the imperfect gold standard for diagnosis. In addition, available noninvasive markers are not fully satisfactory for the diagnosis of fatty liver. Although in recent years many pharmacological agents, on the basis of pathogenetic mechanism of the disease, have been attempted, to date, the guidelines for the management of fatty liver are still lacking. Establishing effective therapeutic strategies to treat the disease represents the challenge for pediatric hepatologists in the near future. In this article, we briefly review the current knowledge and ideas concerning pediatric nonalcoholic fatty liver disease, and discuss the new perspective therapies. [Copyright &y& Elsevier]
- Published
- 2012
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- View/download PDF
32. Thyroid Function Tests in Obese Prepubertal Children: Correlations with Insulin Sensitivity and Body Fat Distribution.
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Brufani, Claudia, Manco, Melania, Nobili, Valerio, Fintini, Danilo, Barbetti, Fabrizio, and Cappa, Marco
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THYROID gland function tests ,CHILDHOOD obesity ,DUAL-energy X-ray absorptiometry ,THYROID hormones ,THYROID gland ,BODY composition ,OVERWEIGHT children ,PHYSIOLOGY - Abstract
Background/Aims: Elevated thyroid-stimulating hormone (TSH) concentrations in association with normal/slightly elevated free triiodothyronine (fT3) and/or free thyroxine (fT
4 ) have been consistently found in obese children. To examine relationships between adiposity, insulin sensitivity, and TSH, fT3 and fT4 . Methods: 240 overweight/obese prepubertal children were studied. Fasting TSH, fT3 , fT4 , glucose, insulin, C-peptide, lipids, leptin and adiponectin were evaluated. Insulin sensitivity and resistance were estimated [quantitative insulin check index (QUICKI), insulin sensitivity index (ISI), and hepatic insulin resistance index]. Body fat was measured by dual-energy X-ray absorptiometry. The central obesity index was calculated as the ratio of fat tissue in the trunk region to fat tissue in the leg region. Results: The multiple regression analysis with age, gender and measures of fatness as covariates showed that QUICKI was the only significant negative predictor of TSH and central obesity index the strongest positive predictor of fT3 , in association with either age or hepatic insulin resistance index, and that the only positive determinant of fT4 was hepatic insulin resistance index. Conclusions: Reduced insulin sensitivity is associated with augmented TSH and fT4 , while progressive central fat accumulation is strictly related to a parallel increase in fT3 levels, independently from total body fat. Further studies are needed to understand mechanisms linking thyroid function to insulin sensitivity and body composition in obese children. [ABSTRACT FROM AUTHOR]- Published
- 2012
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33. Non-alcoholic fatty liver disease in children now: Lifestyle changes and pharmacologic treatments
- Author
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Alisi, Anna and Nobili, Valerio
- Subjects
- *
INSULIN resistance , *THERAPEUTIC use of vitamin E , *DOCOSAHEXAENOIC acid , *THIAZOLIDINEDIONES , *BEHAVIOR modification , *DIABETES , *DIETARY supplements , *FATTY liver , *HEALTH behavior , *METABOLIC regulation , *WEIGHT loss , *OXIDATIVE stress , *METABOLIC syndrome , *PHYSICAL activity , *CHILDREN , *PREVENTION , *THERAPEUTICS , *DISEASE risk factors - Abstract
Abstract: Over the past decade, non-alcoholic fatty liver disease (NAFLD) has become one of most common chronic liver diseases in children. A greater understanding about the risk factors and molecular pathogenesis of NAFLD suggests that lifestyle interventions aiming to decrease obesity/body mass index and metabolic derangement are the first line of treatments adopted in children affected by this disease. However, because these therapeutic options are often at the beginning misjudged by the patients and their parents, the use of pharmacologic agents may help to protect the liver and other organs from further irreversible tissue damage. Pharmacologic therapies against one or more specific factors and/or molecules involved in the development of NAFLD (i.e., insulin resistance, free fatty acid lipid toxicity, and oxidative stress) also might slow the progression of this increasingly prevalent pediatric disorder. On this basis, insulin sensitizers, antioxidants, cytoprotective agents, and dietary supplementations have been evaluated in pediatric clinical trials. In this review, we discuss the efficacy of the dietary approaches, possibly coupled with regular exercise, on decreasing the metabolic and histologic damage in pediatric NAFLD. We also emphasize several advantages of the pharmacologic treatments adopted or adoptable in combination with lifestyle interventions in children with NAFLD. [Copyright &y& Elsevier]
- Published
- 2012
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34. Epigenetic mechanisms elicited by nutrition in early life.
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Canani, Roberto Berni, Di Costanzo, Margherita, Leone, Ludovica, Bedogni, Giorgio, Brambilla, Paolo, Cianfarani, Stefano, Nobili, Valerio, Pietrobelli, Angelo, and Agostoni, Carlo
- Subjects
BIOMARKERS ,CHILDREN ,DATE of conception ,DIET ,DISEASE susceptibility ,DNA ,GENETICS ,NUTRITION ,NUTRITIONAL requirements ,PREGNANT women ,PRENATAL influences ,PHENOTYPES ,GENOMICS ,METABOLIC syndrome ,PROBIOTICS - Published
- 2011
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35. Use of metformin in pediatric age.
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Brufani, Claudia, Fintini, Danilo, Nobili, Valerio, Patera, Patrizia Ippolita, Cappa, Marco, and Brufani, Mario
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TYPE 2 diabetes treatment ,ANALYSIS of variance ,DIABETES ,DOSAGE forms of drugs ,FATTY acids ,FATTY liver ,GENETIC techniques ,GLUCOSE ,INSULIN resistance ,EVALUATION of medical care ,OBESITY ,PEDIATRICS ,POLYCYSTIC ovary syndrome ,ALANINE aminotransferase ,BODY mass index ,METFORMIN ,PHARMACODYNAMICS - Abstract
Brufani C, Fintini D, Nobili V, Patera PI, Cappa M, Brufani M. Use of metformin in pediatric age. [ABSTRACT FROM AUTHOR]
- Published
- 2011
- Full Text
- View/download PDF
36. Extrahepatic portal vein thrombosis in children and adolescents: Influence of genetic thrombophilic disorders.
- Author
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Pietrobattista, Andrea, Luciani, Matteo, Abraldes, Juan G., Candusso, Manila, Pancotti, Simona, Soldati, Massimo, Monti, Lidia, Torre, Giuliano, and Nobili, Valerio
- Subjects
PORTAL vein ,THROMBOSIS in children ,DISEASE prevalence ,GENETIC disorders ,HOMOCYSTEINE in the body ,BLOOD coagulation disorders - Abstract
AIM: To explore the prevalence of local and genetic thrombophilic disorders as risk factors for portal vein thrombosis (PVT) in our series, the largest ever published in pediatric literature. METHODS: We conducted a case-control study enrolling 31 children with PVT and 26 age-matched controls. All were screened for thrombophilia, including genetic disorders, protein C, protein S and homocysteine deficiencies. All coagulation parameters were studied at least 3 mo after the diagnosis of portal vein obstruction. RESULTS: In our study we showed that most pediatric patients with PVT have local prothrombotic risk factors, which are probably the most important factors leading to PVT. However, there is a clear association between the presence of prothrombotic disorders and PVT, suggesting that these increase the risk of thrombosis in patients with local factors such as perinatal umbilical vein catheterization or sepsis. CONCLUSION: Patients with PVT should be screened for inherited prothrombotic disorders regardless of a history of an obvious local risk factor. [ABSTRACT FROM AUTHOR]
- Published
- 2011
- Full Text
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37. Liver fibrosis in paediatric liver diseases.
- Author
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Alisi, Anna, de Vito, Rita, Monti, Lidia, and Nobili, Valerio
- Subjects
FIBROSIS ,PEDIATRICS ,LIVER biopsy ,CIRRHOSIS of the liver ,DISEASE progression ,HISTOPATHOLOGY - Abstract
Numerous paediatric liver diseases from different origins may be complicated by development of liver fibrosis and progression to cirrhosis. Although fibrogenesis, which represents a major driving force for the development of liver fibrosis, has common tracts whatever the aetiology, liver fibrosis has different histopathological patterns in paediatric liver disease. In these diseases management choices may depend upon the stage of liver fibrosis. Thus, the accurate estimation of histological pattern of liver fibrosis is important for the prevention of the subsequent complications. Liver biopsy has long been considered as a gold standard diagnostic method for assessing liver fibrosis. However, due to its several disadvantages, in the last decades alternative and accurate non-invasive means to estimate fibrosis are developed. In this review, we characterised the most frequent histological patterns of liver fibrosis in paediatric liver diseases. Furthermore, we describe use of liver biopsy in diagnosis and staging of liver fibrosis, list the alternative non-invasive techniques that have an emerging role in the assessment of liver fibrosis, and propose a management algorithm. [ABSTRACT FROM AUTHOR]
- Published
- 2011
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38. Relationship between portal chronic inflammation and disease severity in paediatric non-alcoholic fatty liver disease.
- Author
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Alisi, Anna, Bedogni, Giorgio, De Vito, Rita, Comparcola, Donatella, Manco, Melania, and Nobili, Valerio
- Subjects
FATTY liver ,INFLAMMATION ,CHRONIC diseases ,JUVENILE diseases ,FIBROSIS ,BIOPSY ,DISEASES in teenagers - Abstract
Abstract: Background: The non-alcoholic steato-hepatitis Clinical Research Network has recently shown that portal chronic inflammation is associated with liver fibrosis in American children with non-alcoholic fatty liver disease. Aim: We tested whether the portal chronic inflammation-fibrosis association was present in a series of Italian children with non-alcoholic fatty liver disease. Methods: We re-assessed the liver biopsies of 144 consecutive Italian children with non-alcoholic fatty liver disease aged 3–18 years and followed at the “Bambino Gesù” Paediatric Hospital. Non-alcoholic fatty liver disease and portal chronic inflammation were diagnosed using the non-alcoholic steato-hepatitis Clinical Research Network criteria. Anthropometry, body composition, liver enzymes, metabolic parameters and blood pressure were measured in all children. Results: Two children had no portal chronic inflammation, 84 had mild and 58 more than mild portal chronic inflammation according to the non-alcoholic steato-hepatitis Clinical Research Network criteria. Children with no or mild portal chronic inflammation had the same clinical features of those with more than mild portal chronic inflammation except for insulin resistance, which was greater. There was no association between steatosis, lobular inflammation, ballooning, fibrosis and portal chronic inflammation. Conclusion: We were not able to confirm the existence of a clinico-pathological association between portal chronic inflammation and disease severity in a series of Italian children with non-alcoholic fatty liver disease. Some clinico-pathological correlates of paediatric non-alcoholic fatty liver disease may be population-specific. [ABSTRACT FROM AUTHOR]
- Published
- 2011
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39. Insulin-like growth factors (IGF-I and -II): new actors in the development of non-alcoholic fatty liver disease.
- Author
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Inzaghi, Elena, Cianfarani, Stefano, and Nobili, Valerio
- Subjects
SOMATOMEDIN ,LIVER diseases ,FATTY liver ,INSULIN resistance ,OXIDATIVE stress ,SOMATOTROPIN - Abstract
Non-alcoholic fatty liver disease (NAFLD) is one of the most common causes of chronic liver disease worldwide, affecting 20-30% of adults and 3-10% of children in Western countries. The pathogenesis of NAFLD is considered to be multifactorial and factors such as insulin resistance, intrahepatic fat accumulation, oxidative stress, mitochondrial alterations, and stellate cell activation appear to substantially contribute to the development and progression of the disease. In this Editorial, we highlight some evidence suggesting a close link between NAFLD and growth hormone (GH)-IGF (insulin-like growth factor) axis. [ABSTRACT FROM AUTHOR]
- Published
- 2014
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40. Intrauterine Growth Retardation, Insulin Resistance, and Nonalcoholic Fatty Liver Disease in Children.
- Author
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Nobili, Valerio, Marcellini, Matilde, Marchesini, Giulio, Vanni, Ester, Manco, Melania, Villani, Alberto, and Bugianesi, Elisabetta
- Subjects
- *
FATTY liver , *CHILDREN , *FETAL growth retardation , *INSULIN resistance , *METABOLISM , *HEPATITIS , *INSULIN - Abstract
The article offers information on the results of a study on the association of nonalcoholic fatty liver disease (NAFLD) in children with intrauterine growth retardation independent of and in addition to insulin resistance. It says that small for gestational age (SGA) with NAFLD represent a subset with a higher prevalence of both metabolic abnormalities and Non-Alcoholic Steatohepatitis (NASH). It states that factors known to affect insulin sensitivity may also independently contribute to liver disease progression.
- Published
- 2007
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41. Reply to: “Energy drinks and adolescents – A hepatic health hazard?”.
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Nobili, Valerio, Mosca, A., Scorletti, E., and Byrne, Christopher D.
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- *
ENERGY drinks , *HEPATITIS - Published
- 2018
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42. Post‐transplant metabolic syndrome in children: Know better to cure better.
- Author
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Nobili, Valerio, Pietrobattista, Andrea, Valenti, Luca, and Spada, Marco
- Subjects
- *
METABOLIC syndrome , *FATTY liver , *ADIPOSE tissue diseases , *LIVER transplantation , *GENETICS , *CHILDREN - Abstract
The article focuses on Pediatric Transplantation. Topics discussed include pediatric liver transplant that provide practices in screening and managing Post‐transplant metabolic syndrome (PTMS); mentions the role of potential genetic in the post‐transplant setting; and information on the impact of genetic variants like PNPLA3 on adipose tissue and adiponectin secretion.
- Published
- 2019
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43. Nonalcoholic Fatty Liver Disease in Italian Children with Down Syndrome: Prevalence and Correlation with Obesity-Related Features.
- Author
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Valentini, Diletta, Alisi, Anna, di Camillo, Chiara, Sartorelli, Maria Rita, Crudele, Annalisa, Bartuli, Andrea, Nobili, Valerio, and Villani, Alberto
- Abstract
Objective: To assess the prevalence of overweight/obesity in a cohort of Italian children with Down syndrome (DS) and to investigate the correlation of both obesity and DS with nonalcoholic fatty liver disease (NAFLD).Study Design: We enrolled 280 children with DS (age range 5-18 years), who were referred to the DS outpatient clinic of the Bambino Gesù Children's Hospital in Rome. For all children, we collected the clinical history and measured anthropometric variables. Eighty-four of 280 children with DS were selected to undergo liver ultrasound scanning to evaluate the presence of NAFLD.Results: Italian children with DS exhibited a prevalence of 19.64% for overweight and 12.14% for obesity. The prevalence of NAFLD in nonobese (45%) and overweight/obese (82%) children with DS is greater than in the European pediatric nonobese (5.7%) or obese population (33%). Moreover, the severity of liver brightness on ultrasound scan correlated positively with body mass index, triglycerides, low-density lipoprotein-cholesterol, and leptin levels and negatively with adiponectin.Conclusions: We demonstrated that, independently from the obese phenotype, children with DS display a greater risk to develop NAFLD than the general pediatric population. [ABSTRACT FROM AUTHOR]- Published
- 2017
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44. A review of the pathogenic and therapeutic role of nutrition in pediatric nonalcoholic fatty liver disease.
- Author
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Panera, Nadia, Barbaro, Barbara, Della Corte, Claudia, Mosca, Antonella, Nobili, Valerio, and Alisi, Anna
- Subjects
- *
VITAMIN therapy , *DOCOSAHEXAENOIC acid , *THERAPEUTIC use of probiotics , *ANTIGENS , *DIETARY supplements , *FATTY liver , *MEDLINE , *NUTRITION , *ONLINE information services , *SYSTEMATIC reviews , *CHILDREN , *THERAPEUTICS - Abstract
Abstract Nonalcoholic fatty liver disease (NAFLD) is a multifaceted disorder that ranges from simple fatty liver to nonalcoholic steatohepatitis (NASH) with or without fibrosis, which may evolve toward cirrhosis and hepatocellular carcinoma. It is currently considered a "global" and "epidemic" disease, whose prevalence is progressively increasing even in pediatric age. The incidence of NAFLD is very high in overweight/obese children, and a greater risk of disease progression is associated with severe obesity, highlighting the role of nutrition. To date, for NAFLD, there are few guidelines for diagnostic and follow-up methods, and scarce validated protocols for treatment. The initial indications consist of gradual weight loss and regular exercise, but in children, the difficulty of adhering to long-term behavioral changes makes this approach limited. The purpose of this narrative review is to examine the mechanism underlying the pathogenic mechanisms that lead to NAFLD in children, with a major focus on the role of nutrition. Because this is particularly relevant in light of the absence of pharmacological treatments suitable for children, we also overview clinical studies on the potential effects of nutritional supplementations, including vitamins, docosahexaenoic acid, and probiotics.in children. To this aim, updated search was conducted on PubMed and clinicaltrials.gov databases. Future research should consider additional clinical studies in pediatric NAFLD patients to validate the benefits of dietary supplements and to define the appropriate dosage and duration for intervention. Furthermore, experimental studies with -omics approaches could be helpful to deepen the related mechanisms and to search for a possible optimal supplement combination against NAFLD in children. [ABSTRACT FROM AUTHOR]
- Published
- 2018
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45. Paediatric Non-Alcoholic Fatty Liver Disease: Impact on Patients and Mothers' Quality of Life.
- Author
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Mazzone, Luigi, Postorino, Valentina, De Peppo, Lavinia, Della Corte, Claudia, Lofino, Giuseppe, Vassena, Lia, Fatta, Laura, Armando, Marco, Bedogni, Giorgio, Vicari, Stefano, and Nobili, Valerio
- Subjects
- *
FATTY liver , *ANXIETY , *CHI-squared test , *CHILD Behavior Checklist , *MENTAL depression , *ENZYME-linked immunosorbent assay , *MOTHERS , *NONPARAMETRIC statistics , *PEDIATRICS , *QUALITY of life , *QUESTIONNAIRES , *SCALE analysis (Psychology) , *T-test (Statistics) , *DATA analysis software , *DIAGNOSIS - Abstract
Background: Non-alcoholic fatty liver disease (NAFLD) is one of the causes of fatty liver in adults and is currently the primary form of chronic liver disease in children and adolescents. However, the psychological outcome (i.e. the behavioural problems that can in turn be related to psychiatric conditions, like anxiety and mood disorders, or lower quality of life) in children and adolescents suffering of NAFLD has not been extensively explored in the literature. Objectives: The present study aims at evaluating the emotional and behavioural profile in children suffering from NAFLD and the quality of life in their mothers. Patients and Methods: A total of 57 children (18 females/39 males) with NAFLD were compared to 39 age-matched control children (25 females/14 males). All participants were submitted to the following psychological tools to assess behavior, mood, and anxiety: the Multidimensional Anxiety Scale for Children (MASC), the Child Behavior Checklist (CBCL), and the Children's Depression Inventory (CDI). Moreover, the mothers of 40 NAFLD and 39 control children completed the World Health Organization Quality of Life-BREF (WHOQOL-BREF) questionnaire. Results: NAFLD children scored significantly higher as compared to control children in MASC (P = 0.001) and CDI total (P < 0.001) scales. The CBCL also revealed significantly higher scores for NAFLD children in total problems (P = 0.046), internalizing symptoms (P = 0.000) and somatic complaints (P < 0.001). The WHOQOL-BREF revealed significantly lower scores for the mothers of NAFLD children in the overall perception of the quality of life (P < 0.001), and in the "relationships" domain (P = 0.023). Conclusions: Increased emotional and behavioural problems were detected in children with NAFLD as compared to healthy control children, together with an overall decrease in their mothers' quality of life. These results support the idea that these patients may benefit from a psychological intervention, ideally involving both children and parents, whose quality of life is likely negatively affected by this disease. [ABSTRACT FROM AUTHOR]
- Published
- 2013
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46. Development and validation of a new histological score for pediatric non-alcoholic fatty liver disease
- Author
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Alkhouri, Naim, De Vito, Rita, Alisi, Anna, Yerian, Lisa, Lopez, Rocio, Feldstein, Ariel E., and Nobili, Valerio
- Subjects
- *
FATTY liver , *CLINICAL trials , *JUVENILE diseases , *INFLAMMATION , *HISTOPATHOLOGY , *FATTY degeneration , *FIBROSIS , *TRIGLYCERIDES , *DIAGNOSIS - Abstract
Background & Aims: Pediatric non-alcoholic fatty liver disease (NAFLD) may present with a distinct histopathological pattern characterized by the presence of predominant portal-based injury and portal inflammation (PI). We aimed at developing a new grading score for pediatric NAFLD to be used in clinical trials that takes into account the presence of PI and the weight of histological features. Methods: Our training set consisted of 203 children with biopsy-proven NAFLD. The diagnosis of non-alcoholic steatohepatitis (NASH) was based on Brunt’s criteria. Histological features were scored: steatosis (0–3), lobular inflammation (0–3), ballooning (0–2), and PI (0–2). Logistic regression analysis was performed to apply weight to each feature. The new score was called the Pediatric NAFLD Histological Score or PNHS. The validation set consisted of 100 children with NAFLD. Results: The mean age of the initial cohort was 12.4±3.4years and significant fibrosis (fibrosis stage ⩾2) was present in 26 patients (12.8%). NASH was diagnosed in 135 patients with a mean NAS of 4.5±1.4. The mean PNHS in the NASH group was 89±20.5 compared to 21.9±24.5 in the “not NASH” group, p <0.001. PNHS correlated with the presence of NASH according to the pathologist’s diagnosis, better than the NAFLD activity score (NAS), p =0.011. The area under the ROC curve (AUC) for the diagnosis of NASH was 0.96 for PNHS. Similar findings were observed in the validation set with an AUC of 0.94. Conclusions: PNHS may be used for histological grading of pediatric NAFLD with excellent correlation with the presence of NASH. [ABSTRACT FROM AUTHOR]
- Published
- 2012
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- View/download PDF
47. Intima-media thickness and liver histology in obese children and adolescents with non-alcoholic fatty liver disease
- Author
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Manco, Melania, Bedogni, Giorgio, Monti, Lidia, Morino, Giuseppe, Natali, Gianluigi, and Nobili, Valerio
- Subjects
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CHILDHOOD obesity , *ADOLESCENT obesity , *FATTY liver , *ARTERIAL diseases , *LIVER biopsy , *ULTRASONIC imaging , *GLUCOSE tolerance tests , *INSULIN resistance - Abstract
Abstract: Objective: To evaluate the relationship between biopsy-proven non-alcoholic fatty liver disease (NAFLD) and carotid artery intima-media thickness (CIMT) in children and adolescents. Methods: A case–control study was performed. Cases were 31 mostly obese children and adolescents, with NAFLD detected at ultrasonography, and confirmed by liver biopsy. Controls were 49 mostly obese children matched for gender, age and BMI without NAFLD at ultrasonography and with normal levels of aminotransferases. Besides standard laboratory measurements, subjects underwent an oral glucose tolerance test to evaluate glucose tolerance and to estimate whole body insulin sensitivity (ISI). Results: CIMT was similar in cases and controls on the right side but higher in cases on the left side. Although statistically significant, this difference is unlikely to be clinically relevant because of substantial overlap of CIMT values between cases and controls. Moreover, there was no association between CIMT and the severity of steatosis, ballooning, fibrosis, and the non-alcoholic steato-hepatitis score in cases. At multivariable analysis in the pooled sample (n =80), age and the z-score of BMI but not NAFLD, gender, blood pressure and triglycerides, were associated with CIMT. Conclusions: We found no association between CIMT and NAFLD in children and adolescents. More importantly, there was no association between histological severity and CIMT in children with NAFLD. [Copyright &y& Elsevier]
- Published
- 2010
- Full Text
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48. Wilson's Disease in Children: A Position Paper by the Hepatology Committee of the European Society for Paediatric Gastroenterology, Hepatology and Nutrition
- Author
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Roderick H. J. Houwen, Dominique Debray, Jörg Jahnel, Nedim Hadzic, Françoise Smets, Wojciech Jańczyk, Loreto Hierro, Lorenzo D'Antiga, Stuart Tanner, Valerio Nobili, Raffaele Iorio, Ulrich Baumann, Anil Dhawan, Björn Fischler, Antal Dezsofi, Piotr Socha, Henkjan J. Verkade, Pietro Vajro, Valérie A. McLin, Center for Liver, Digestive and Metabolic Diseases (CLDM), Lifestyle Medicine (LM), Socha, Piotr, Janczyk, Wojciech, Dhawan, Anil, Baumann, Ulrich, D'Antiga, Lorenzo, Tanner, Stuart, Iorio, Raffaele, Vajro, Pietro, Houwen, Roderick, Fischler, Björn, Dezsofi, Antal, Hadzic, Nedim, Hierro, Loreto, Jahnel, Jörg, Mclin, Valérie, Nobili, Valerio, Smets, Francoise, Verkade, Henkjan J., and Debray, Dominique
- Subjects
Pediatrics ,Cirrhosis ,diagnosis ,medicine.medical_treatment ,Wilson’s disease ,DNA Mutational Analysis ,MUTATION ANALYSIS ,Liver transplantation ,hepatiti ,Liver disease ,0302 clinical medicine ,Hepatolenticular Degeneration ,Liver Function Tests ,hepatitis ,Child ,Societies, Medical ,Chelating Agents ,treatment ,ACQUIRED SIDEROBLASTIC ANEMIA ,Gastroenterology ,Ceruloplasmin ,LIVER-TRANSPLANTATION ,Wilson's disease ,diagnosi ,children, diagnosis, hepatitis, liver, treatment, Wilson’s disease ,INITIAL TREATMENT ,030211 gastroenterology & hepatology ,medicine.symptom ,medicine.medical_specialty ,BILIARY ATRESIA ,liver ,Asymptomatic ,03 medical and health sciences ,ZINC THERAPY ,children ,Biliary atresia ,Internal medicine ,medicine ,Humans ,DIAGNOSTIC-ACCURACY ,Monitoring, Physiologic ,Hepatitis ,business.industry ,CLINICAL PRESENTATION ,TRIETHYLENE TETRAMINE DIHYDROCHLORIDE ,Hepatology ,RELATIVE EXCHANGEABLE COPPER ,medicine.disease ,Liver Transplantation ,Pediatrics, Perinatology and Child Health ,business ,030217 neurology & neurosurgery ,Copper - Abstract
Background:Clinical presentations of Wilson's disease (WD) in childhood ranges from asymptomatic liver disease to cirrhosis or acute liver failure, whereas neurological and psychiatric symptoms are rare. The basic diagnostic approach includes serum ceruloplasmin and 24-hour urinary copper excretion. Final diagnosis of WD can be established using a diagnostic scoring system based on symptoms, biochemical tests assessing copper metabolism, and molecular analysis of mutations in the ATP7B gene. Pharmacological treatment is life-long and aims at removal of copper excess by chelating agents as D-penicillamine, trientine, or inhibition of intestinal copper absorption with zinc salts. Acute liver failure often requires liver transplantation. This publication aims to provide recommendations for diagnosis, treatment, and follow-up of WD in children.Methods:Questions addressing the diagnosis, treatment, and follow-up of WD in children were formulated by a core group of ESPGHAN members. A systematic literature search on WD using MEDLINE, EMBASE, Cochrane Database from 1990 to 2016 was performed focusing on prospective and retrospective studies in children. Quality of evidence was assessed according to the GRADE system. Expert opinion supported recommendations where the evidence was regarded as weak. The ESPGHAN core group and ESPGHAN Hepatology Committee members voted on each recommendation, using the nominal voting technique.
- Published
- 2018
49. Autoimmune hepatitis type 2 following anti-papillomavirus vaccination in a 11-year-old girl
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Corte, Claudia Della, Carlucci, Antonio, Francalanci, Paola, Alisi, Anna, and Nobili, Valerio
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CHRONIC active hepatitis , *HUMAN papillomavirus vaccines , *DISEASE susceptibility , *IMMUNE system , *IMMUNOLOGICAL adjuvants , *LIVER diseases , *SYMPTOMS - Abstract
Abstract: In the last years numerous reports describing a possible association between administration of vaccines and development of autoimmune phenomena and overt autoimmune disease were published. Possible mechanisms of induction of autoimmune phenomena by vaccines and their excipients are probably similar to those implicated in induction by infectious agents. Here we report the case of an 11-year-old girl who developed autoimmune hepatitis type II after four weeks from vaccination against human papillomavirus. The possible relationships between the use of adjuvated vaccine against papillomavirus and autoimmune hepatitis are discussed. Although we do not provide evidence for a causal link, we suggest that the occurrence of the autoimmune hepatitis may be related to the stimulation of immune system by adjuvated-vaccine, that could have triggered the disease in a genetically predisposed individual. Therefore a monitoring of liver function test following administration of vaccine against papillomavirus may be useful in adolescent girl with signs of hepatopathy, as jaundice, dark urine or hepatomegaly, to early identify and to promptly treat autoimmune liver disorders. [Copyright &y& Elsevier]
- Published
- 2011
- Full Text
- View/download PDF
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