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Zhu-Tokita-Takenouchi-Kim (ZTTK) syndrome, an intellectual disability syndrome first described in 2016, is caused by heterozygous loss-of-function variants in SON. Haploinsufficiency in SON may affect multiple genes, including those involved in the development and metabolism of multiple organs. Considering the broad spectrum of SON functions, it is to be expected that pathogenic variants in this gene can cause a wide spectrum of clinical symptoms. We present an additional ZTTK syndrome case due to a de novo heterozygous variant in the SON gene (c.5751_5754delAGTT). The clinical manifestations of our patient were similar to those present in previously reported cases; however, the diagnosis of ZTTK syndrome was delayed for a long time and was carried out during the diagnostic work-up of significant chronic liver disease (CLD). CLD has not yet been reported in any series; therefore, our report provides new information on this rare condition and suggests the expansion of the ZTTK syndrome phenotype, including possible liver involvement. Correspondingly, we recommend screening patients with SON variants specifically for liver involvement from the first years of life. Once the CLD has been diagnosed, an appropriate follow-up is mandatory, especially considering the role of SON as an emerging player in cancer development. Further studies are needed to investigate the role of SON haploinsufficiency as a downregulator of essential genes, thus potentially impairing the normal development and/or functions of multiple organs.
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Oftalmopatías , Discapacidad Intelectual , Humanos , Discapacidad Intelectual/patología , Fenotipo , Síndrome , Hígado/patologíaRESUMEN
Sarcopenia is a clinical condition characterized by a reduction in muscle mass, which typically affects adult patients; however, it has recently been recognized in pediatric literature. Few studies in children with chronic liver disease (CLD) undergoing liver transplantation (LT) have investigated the role of sarcopenia, with controversial results. The aim of our study was to assess the prevalence and impact of sarcopenia among children with CLD who are candidates for LT. We conducted a retrospective, single-center study at Bambino Gesù Children's Hospital (Rome, Italy) from July 2016 to July 2021, evaluating all children (0-16 years old) with CLD listed for LT with an abdomen computed tomography imaging available before LT. The total psoas muscle surface area (t-PMSA) was defined as the sum of left and right psoas muscle surface area measured at L4-L5 on axial images. The t-PMSA z-score was calculated according to reference data, and sarcopenia was defined as a t-PMSA z-score of ≤-2 (1-16 years) or a psoas muscle index [PMI; PMI = t-PMSA/(100 × BSA)] of <50th percentile of the population examined (<1 year). Clinical, laboratory, and LT outcome data were collected from all the patients with CLD. 27 out 48 (56%) of the patients aged 1-16 years were sarcopenic. No differences were noted in anthropometrics, nutritional support, liver function tests, model for ESLD (MELD), or pediatric ESLD (PELD) scores between patients with and without sarcopenia. The former showed a higher prevalence of respiratory complications (66.7% vs. 42.1%) and need for inotropes (40.7% vs. 10.8%) after LT. Among patients aged 0-1 years (n: 36), those with reduced muscle mass (50%) had a longer hospitalization time (44 vs. 24 days) and higher incidences of multi-organ failure syndrome (38.9% vs. 0%) and intensive care unit-related infections (61.1% vs. 27.8%) compared to those with greater muscle mass. t-PMSA and PMI were statistically significant predictors of LT outcomes. Sarcopenia is a reliable index of frailty in children with CLD, as its presence is associated with the risk of a more challenging LT. Future studies will have to investigate the functional aspects of sarcopenia and conceive preventive measures of muscle wasting in CLD patients.
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Neonatal and infantile cholestasis (NIC) can represent the onset of a surgically correctable disease and of a genetic or metabolic disorder worthy of medical treatment. Timely recognition of NIC and identification of the underlying etiology are paramount to improve outcomes. Upon invitation by the Italian National Institute of Health (ISS), an expert working grouped was formed to formulate evidence-based positions on current knowledge about the diagnosis of NIC. A systematic literature search was conducted to collect evidence about epidemiology, etiology, clinical aspects and accuracy of available diagnostic tests in NIC. Evidence was scored using the GRADE system. All recommendations were approved by a panel of experts upon agreement of at least 75% of the members. The final document was approved by all the panel components. This position document summarizes the collected statements and defines the best-evidence diagnostic approach to cholestasis in the first year of life.
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Colestasis , Medicina Basada en la Evidencia , Gastroenterología/normas , Enfermedades del Recién Nacido , Guías de Práctica Clínica como Asunto , Femenino , Humanos , Lactante , Recién Nacido , MasculinoRESUMEN
Allograft fibrosis (AF) after pediatric liver transplantation (pLT) is frequent, but its dynamics are unclear. Our aim was to assess the evolution and risk factors of AF after pLT. A retrospective single-center analysis of pLT patients with a follow-up of ≥5 years who underwent protocol liver biopsies at 6 months, 1 year, 2 years, 5 years, and 10 years was performed. Fibrosis was assessed using the METAVIR and Ishak systems and the liver allograft fibrosis score (LAFs). Of 219 pLTs performed from 2008 to 2018, 80 (36.5%) pLTs were included, and 320 biopsies were reviewed. At 6 months after pLT, fibrosis was found in 54 (67.5%) patients by the METAVIR/Ishak systems and in 59 (73.8%) by the LAFs (P = 0.65). By 5 years, AF was detected in 67 (83.8%), 69 (86.3%), and 72 (90%) specimens using the METAVIR, Ishak, and LAFs systems, respectively (P = 0.54); mild (METAVIR, 51 [63.8%]; Ishak, 60 [75%]; LAFs, 65 [81.2%]) and moderate (METAVIR, 16 [20%]; Ishak, 9 [11.9%]; LAFs, 7 [8.8%]) stages were detected, but severe fibrosis was not found (P = 0.09). In the LAFs, fibrosis involved the portal (85%), sinusoidal (15%), and centrolobular (12%) areas. Of 18 patients with 10-year protocol biopsies, AF was present in 16 (90%), including 1 (5.5%) with severe fibrosis. In all systems, 36.3% of patients showed fibrosis progression from 2 years to 5 years after LT, but they remained stable at the 10-year biopsies without clinical implications. In multivariate analysis, only donor age >40 years was a risk factor for moderate AF at 5 years after LT (odds ratio, 8.3; 95% confidence interval, 1.6-42.1, P = 0.01). Cold ischemia time (CIT) >8 hours was associated with portal (P < 0.001)/sinusoidal fibrosis (P = 0.04), donor age >40 years was associated with sinusoidal (P = 0.01)/centrilobular (P = 0.04) fibrosis, and low tacrolimus trough level within 1 year after LT was associated with centrilobular fibrosis (P = 0.02). AF has a high incidence after pLT, occurring early after transplantation. In most cases, AF is mild or moderate and remains stable in the long run without clinical implications. Donor selection, short CIT, and immunosuppression adherence are crucial to reducing the risk of advanced AF.
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Trasplante de Hígado , Adulto , Aloinjertos/patología , Biopsia , Niño , Fibrosis , Humanos , Incidencia , Hígado/patología , Cirrosis Hepática/diagnóstico , Cirrosis Hepática/epidemiología , Cirrosis Hepática/etiología , Trasplante de Hígado/efectos adversos , Estudios Retrospectivos , Factores de RiesgoAsunto(s)
Fallo Hepático Agudo , Poliendocrinopatías Autoinmunes , Pruebas Genéticas , Humanos , MutaciónRESUMEN
BACKGROUND: Infantile cholestasis (IC) is defined as an impairment of bile production or flow occurring in the first months of life. The diagnostic approach in IC is challenging since the differential diagnosis is broad. METHODS: We retrospectively evaluated 91 cholestatic infants referred to our department from 2014 to 2019. Patients with cholestasis underwent a complete IC diagnostic work-up including quantification of plasma oxysterols 7-ketocholesterol (7-KC) and cholestan-3ß,5α,6ß-triol (C-Triol). RESULTS: Oxysterols concentrations were mildly elevated in IC compared to control population. 7-KC and C-Triol plasma levels presented a linear relationship between them and with Spleen-Z score. Patients with NP-C showed the highest concentrations of both oxysterols compared with other etiologies of IC. Excluding NP-C patients, oxysterols concentrations were similar among all other etiological groups with no correlations found between them and the levels of cholesterol and bilirubin. ROC analysis identified AUCs of 1.0 for both oxysterols in predicting NP-C. CONCLUSION: Infants with IC should undergo a stepwise evaluation in which detailed clinical and deep analytical assessments are the main crossroads. Plasma oxysterols, a simple, reliable, and convenient diagnostic test should be included in the first steps of the diagnostic process in IC.
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Colestasis/sangre , Colestasis/diagnóstico , Oxiesteroles/sangre , Femenino , Humanos , Lactante , Masculino , Estudios RetrospectivosRESUMEN
BACKGROUND: Children affected with chronic liver disease are at risk for fat-soluble vitamins (FSV) deficiency, in this scenario the role of liver transplant has been only partially explored. AIMS: This study aimed to evaluate the prevalence of FSV deficiency in a cohort of paediatric patients awaiting liver transplant, analyze relationships between plasma vitamin concentrations and risk of acute rejections and liver fibrosis and assess the impact of the transplant on vitamin status. METHODS: 166 children candidates for liver transplant were retrospectively evaluated. Vitamin concentrations were measured before and 12 months after transplantation. RESULTS: Before transplant vitamin A, vitamin E and vitamin D deficiency was found in 66.6%, 40.6% and 36.3% of patients, respectively. 12 months after surgery, the prevalence of deficiency decreased to 29,5% and 2,6% for vitamin A and E while remained the same for vitamin D (36.3%). No association was found between vitamin status and the risk of acute rejections or the severity of liver fibrosis. CONCLUSION: Liver transplant was effective to improve vitamin A and E, but it did not affect vitamin D. A consensus is needed to define optimal nutritional management of these patients in order to prevent deficiencies.
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Hepatopatías/terapia , Trasplante de Hígado , Deficiencia de Vitamina A/epidemiología , Deficiencia de Vitamina D/epidemiología , Deficiencia de Vitamina E/epidemiología , Enfermedad Crónica , Femenino , Humanos , Lactante , Italia , Hepatopatías/complicaciones , Masculino , Estudios Retrospectivos , Vitamina A/sangre , Deficiencia de Vitamina A/complicaciones , Vitamina D/sangre , Deficiencia de Vitamina D/complicaciones , Vitamina E/sangre , Deficiencia de Vitamina E/complicacionesRESUMEN
The Publisher regrets that this article is an accidental duplication of an article that has already been published, https://doi.org/10.1016/j.dld.2019.10.005. The duplicate article has therefore been withdrawn. The full Elsevier Policy on Article Withdrawal can be found at https://www.elsevier.com/about/our-business/policies/article-withdrawal.
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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.
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Suplementos Dietéticos , Enfermedad del Hígado Graso no Alcohólico/tratamiento farmacológico , Terapia Nutricional , Obesidad Infantil/complicaciones , Niño , Ácidos Docosahexaenoicos/uso terapéutico , Humanos , Enfermedad del Hígado Graso no Alcohólico/etiología , Estado Nutricional , Probióticos/uso terapéutico , Vitaminas/uso terapéuticoRESUMEN
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.
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Pancreatitis , Enfermedad Aguda , Niño , Humanos , Páncreas/anomalías , Páncreas/patología , Pancreatitis/diagnóstico , Pancreatitis/etiología , Pancreatitis/terapia , Pancreatitis Crónica/diagnóstico , Pancreatitis Crónica/etiología , Pancreatitis Crónica/terapia , Guías de Práctica Clínica como Asunto , RecurrenciaRESUMEN
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 and P < .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.
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Factores de Crecimiento de Fibroblastos/sangre , Hígado/química , Enfermedad del Hígado Graso no Alcohólico/sangre , Receptores Citoplasmáticos y Nucleares/análisis , Adolescente , Factores de Edad , Ácidos y Sales Biliares/análisis , Biomarcadores/sangre , Biopsia , Estudios de Casos y Controles , Niño , Regulación hacia Abajo , Ensayo de Inmunoadsorción Enzimática , Femenino , Técnica del Anticuerpo Fluorescente , Humanos , Hígado/patología , Masculino , Enfermedad del Hígado Graso no Alcohólico/diagnóstico , Prueba de Estudio ConceptualAsunto(s)
Dieta Mediterránea , Enfermedad del Hígado Graso no Alcohólico , Niño , Hígado Graso , Humanos , HígadoRESUMEN
OBJECTIVE: In the last decade, it was demonstrated that the Mediterranean diet (MD) represents an ideal diet for all age groups and has an important role in the prevention of metabolic and cardiovascular diseases, as well as nonalcoholic fatty liver disease (NAFLD). The aim of the present study was to analyze the association between adherence to the MD and NAFLD, with laboratory and histologic evaluation, in a group of children and adolescents with obesity. METHODS: We enrolled 243 patients with obesity referred to our department from March 2014 to November 2015. In all patients, we performed abdominal ultrasound and laboratory assays. In selected cases (100 patients) liver biopsy was performed. Level of adherence to the MD was evaluated by a clinical questionnaire, the Mediterranean Diet Quality Index for children and adolescents (KIDMED). RESULTS: The prevalence of low KIDMED score was significantly higher in patients with nonalcoholic steatohepatitis compared with other groups; poor adherence to the MD correlated with liver damage, the NAFLD activity score >5, and grade 2 fibrosis. Moreover, in patients with poor adherence to the MD, higher values of C-reactive protein, fasting insulin, homeostatic model assessment of insulin resistance, and homeostatic model assessment of ß cell function were observed. CONCLUSION: The MD could be a safe and inexpensive therapeutic option for children with obesity and NAFLD.
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Diabetes Mellitus/prevención & control , Dieta Mediterránea/estadística & datos numéricos , Enfermedad del Hígado Graso no Alcohólico/prevención & control , Cooperación del Paciente/estadística & datos numéricos , Obesidad Infantil/epidemiología , Conducta de Reducción del Riesgo , Adolescente , Niño , Comorbilidad , Diabetes Mellitus/epidemiología , Femenino , Humanos , Italia/epidemiología , Masculino , Enfermedad del Hígado Graso no Alcohólico/epidemiología , RiesgoRESUMEN
Nonalcoholic steatohepatitis (NASH), a progressive form of nonalcoholic fatty liver disease, is one of the most common hepatic diseases in children. We conducted a randomized controlled clinical trial on children with biopsy-proven NASH based on a combinatorial nutritional approach compared with placebo. Participants were assigned to lifestyle modification plus placebo or lifestyle modification plus a mix containing docosahexaenoic acid, choline, and vitamin E (DHA-CHO-VE). Forty children and adolescents participated in the entire trial. The primary outcome was the improvement of liver hyperechogenicity. Secondary outcomes included alterations of alanine aminotransferase (ALT) and other metabolic parameters. Furthermore, changes of serum bile acids (BA) and plasma fibroblast growth factor 19 (FGF19) levels were evaluated as inverse biomarkers of disease severity. At the end of the study, we observed a significant decrease in severe steatosis in the treatment group (50% to 5%, p = 0.001). Furthermore, although the anthropometric and biochemical measurements in the placebo and DHA-CHO-VE groups were comparable at baseline, at the end of the study ALT and fasting glucose levels improved only in the treatment group. Finally, we found that BA levels were not influenced whereas FGF19 levels were significantly increased by DHA-CHO-VE. The results suggest that a combination of DHA, VE, and CHO could improve steatosis and reduce ALT and glucose levels in children with NASH. However, further studies are needed to assess the impact of a DHA and VE combination on repair of liver damage in paediatric NASH.
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Fenómenos Fisiológicos Nutricionales Infantiles , Colina/uso terapéutico , Suplementos Dietéticos , Ácidos Docosahexaenoicos/uso terapéutico , Hígado/fisiopatología , Enfermedad del Hígado Graso no Alcohólico/dietoterapia , Vitamina E/uso terapéutico , Adolescente , Biomarcadores/sangre , Biopsia , Niño , Colina/efectos adversos , Terapia Combinada/efectos adversos , Suplementos Dietéticos/efectos adversos , Progresión de la Enfermedad , Ácidos Docosahexaenoicos/efectos adversos , Método Doble Ciego , Ejercicio Físico , Femenino , Estudios de Seguimiento , Estilo de Vida Saludable , Humanos , Hígado/patología , Masculino , Enfermedad del Hígado Graso no Alcohólico/sangre , Enfermedad del Hígado Graso no Alcohólico/patología , Enfermedad del Hígado Graso no Alcohólico/fisiopatología , Índice de Severidad de la Enfermedad , Vitamina E/efectos adversosRESUMEN
Background and study aims Incidence of morbid obesity has grown dramatically in the last half century and this phenomenon affects with particular severity the pediatric population. Dietary restrictions and careful programs to improve lifestyle are often ineffective to manage this particular group of patients, due to poor compliance typical of the adolescence. The aim of this study was to evaluate the effectiveness of a new intragastric balloon for treatment of morbidly obese children. Patients and methods A new swallowable intragastric balloon (Obalon) has been used for the first time in 17 obese children in order to assess its safety and effectiveness in terms of reduction in excess weight. In 9 of 17 children a second balloon was placed 30 to 40 days after the first insertion. All devices were endoscopically removed after a mean time of 18 weeks. Results In the group of 16 patients who completed the study (1 patient still under treatment) mean weight decreased from 95.8â±â18.4âKg to 83.6â±â27.1 (Pâ<â0.05). Mean body mass index (BMI) decreased from 35.27± 5.89 (range 30.4â-â48) to 32.25â±â7.1 (range 23.5â-â45.7) (Pâ>â0.05); mean excess weight, calculated according to Cole's curves for pediatric populations, decreased from 36.2â±â15.9 to 29.4â±â18.3âKg (Pâ=â0.14), with an %EWL of 20.1â±â9.8 (range 2.3â-â35.1). Waist circumference decreased from 109â±â12.3âcm to 99â±â10.5âcm (Pâ<â0.05). Conclusions Obalon can be administered easily without complications, inducing an appreciable weight loss with a statistically significant reduction in BMI and an improvement in associated comorbidities.
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BACKGROUND: During recent years, treatment with statins has been suggested to induce several effect on bone status and vitamin D metabolism, interpreted as statins pleiotropic effects. Actually, the available data are not conclusive and the characteristics of this association remains unclear. AIM: The purpose of this meta-analysis was therefore to investigate the possible effect of statins therapy on vitamin D serum levels based on the reported results from RCTs and observational studies. METHODS: PubMed-Medline, SCOPUS, Web of Science and Google Scholar databases were searched to identify studies evaluating the impact of statins on plasma vitamin D concentrations from inception to September 16, 2015. A systematic assessment of bias in the included randomized controlled trials was performed using the Cochrane criteria. A random-effects model (using DerSimonian-Laird method) and the generic inverse variance method were used for the heterogeneity of studies in terms of demographic characteristics of populations being studied and also differences in study design and type of statin being studied. Sensitivity analysis was conducted using the leave-one-out method. RESULTS: Following a multiple database search, 1422 published studies were identified; among these, 7 studies were found to be eligible and included in the systematic review and meta-analysis. Our meta-analysis of data from seven studies (including 5 RCTs) did not indicate any significant effect of statins treatment on plasma vitamin D levels. CONCLUSION: Further well-designed trials are necessary to confirm these results and to define better a possible relationship between statins and vitamin D levels.
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Enfermedad de la Arteria Coronaria/sangre , Enfermedad de la Arteria Coronaria/tratamiento farmacológico , Inhibidores de Hidroximetilglutaril-CoA Reductasas/farmacología , Inhibidores de Hidroximetilglutaril-CoA Reductasas/uso terapéutico , Vitamina D/sangre , LDL-Colesterol/sangre , Humanos , Inhibidores de Hidroximetilglutaril-CoA Reductasas/química , Lipoproteínas LDL/química , Lipoproteínas LDL/farmacologíaRESUMEN
BACKGROUND: There are no licensed treatments for non alcoholic fatty liver disease (NAFLD) in adults or children. In NAFLD, several studies have shown a benefit of omega-3 fatty acid treatment on lipid profile, insulin-sensitivity and hepatic steatosis and it has also been suggested that Vitamin D treatment has potential antifibrotic properties in liver disease. TRIAL DESIGN: To date, however, there are no studies that have tested the combination of Docosahexanoic acid (DHA) and vitamin D treatment which may benefit the whole spectrum of disease in NAFLD. Our aim therefore, was to test the effect of daily DHA (500 mg) plus vitamin D (800 IU) treatment, in obese children with biopsy-proven NAFLD and vitamin D deficiency, in a randomized, double-blind placebo-controlled trial. METHODS: The 41/43 patients completed the study (18-treatment, 23-placebo). At 12 months: i) the main outcome was liver histology improvement, defined by NAS; ii) the secondary outcome was amelioration of metabolic parameters. RESULTS: DHA plus vitamin D treatment reduced the NAFLD Activity Score (NAS), in the treatment group (5.4 v1.92; p<0.001 for baseline versus end of study). There was no change in fibrosis score, but a reduction of the activation of hepatic stellate cells (HSC) and fibrillar collagen content was noted (3.51±1.66 v. 1.59±1.37; p = 0.003) in treatment group. Moreover, the triglycerides (174.5 vs. 102.15 mg/dl), ALT (40.25 vs. 24.5 UI/l) and HOMA-IR (4.59 vs. 3.42) were all decreased with treatment. CONCLUSION: DHA plus vitamin D treatment improved insulin-resistance, lipid profile, ALT and NAS. There was also decreased HSC activation and collagen content with treatment.
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Ácidos Docosahexaenoicos/uso terapéutico , Hígado/patología , Enfermedad del Hígado Graso no Alcohólico/tratamiento farmacológico , Deficiencia de Vitamina D/tratamiento farmacológico , Vitamina D/uso terapéutico , Adolescente , Niño , Preescolar , Método Doble Ciego , Quimioterapia Combinada , Femenino , Humanos , Masculino , Enfermedad del Hígado Graso no Alcohólico/complicaciones , Enfermedad del Hígado Graso no Alcohólico/patología , Resultado del Tratamiento , Deficiencia de Vitamina D/complicaciones , Deficiencia de Vitamina D/patologíaRESUMEN
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. It is difficult to define the role of the various components of soft drinks and energy drinks in the pathogenesis of NAFLD and its progression in NASH, but the major role is played by high calorie and high sugar consumption, mainly fructose. In addition, other components of these beverages (e.g. xanthine) seem to have an important role in the pathogenesis of metabolic disorders, crucial pathways involved in NAFLD/NASH. The drastic reduction in the consumption of energy drinks and soft drinks is an appropriate intervention for the prevention of obesity and NAFLD in young people.
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Bebidas Gaseosas/efectos adversos , Dieta , Bebidas Energéticas/efectos adversos , Enfermedad del Hígado Graso no Alcohólico/etiología , Obesidad/etiología , Adolescente , Niño , Humanos , Enfermedad del Hígado Graso no Alcohólico/metabolismo , Obesidad/metabolismoRESUMEN
Nonalcoholic fatty liver disease (NAFLD) is one of the most important causes of chronic liver disease in children and adults. Recently, therapeutic supplementation with docosahexaenoic acid (DHA) showed an anti-inflammatory and insulin-sensitizing effect in children with NAFLD. The anti-inflammatory effects of DHA depend on its ability to alter phospholipid composition of the cell membrane, to disrupt lipid rafts and to hamper the transcriptional activity of nuclear factor-x03BA;B that controls the expression of proinflammatory cytokines. These effects of DHA are due to the interaction with the G-protein-coupled receptor 120 (GRP120), a lipid-sensing receptor highly expressed in activated macrophages. In fact, DHA may activate GPR120 expression in macrophages causing anti-inflammatory effects, and insulin-sensitizing and antidiabetic effects in vivo. Recently, it has been shown that GPR120 is also expressed by the Kupffer cells of the liver. A diet low in n-3 polyunsaturated fatty acids, as well as the presence of genetic factors, may induce a reduction in the GRP120 signal and the activation of Kupffer cells and inflammation during NAFLD. Therefore, it is conceivable that DHA/GRP120 may play a key role in slowing the progression of liver damage in patients with NAFLD.