Children-Basel最新文献

{"title":"The Effectiveness of Leg- and Arm-Powered Trike Training Among Children with Impaired Walking Ability-A Pilot Study.","authors":"Loredana Tschenett, Heiner Baur","doi":"10.3390/children12030382","DOIUrl":"10.3390/children12030382","url":null,"abstract":"<p><p><b>Background/Objectives:</b> The GO-TRYKE^® Kid (GTK^®) is an arm- and leg-powered tricycle which, in addition to promoting strength, endurance, and coordination, aims to reactivate the central pattern generators of the spine for locomotion through cyclical movements. The present study investigated the effects of GTK^® training on walking ability, GTK^® riding performance, and health-related quality of life in children with walking disabilities. <b>Methods:</b> Nine children trained with the device twice a week for nine weeks. Short- and long-term effects on walking ability were measured using the timed up and go test (TUG) and the two-minute walk test (2MWT). GTK^® riding performance and health-related quality of life were compared before and after the intervention period. <b>Results:</b> While no long-term effect on walking was found, a significant short-term effect on functional walking ability was observed (<i>p</i> = 0.009). GTK^® riding performance improved significantly over the training period (<i>p</i> = 0.004). There were no significant changes in health-related quality of life. <b>Conclusions:</b> GTK^® enables children with walking disabilities to participate in cycling as part of play and sport. Further research is required to investigate its functional and participatory effects, as there is significant potential to improve physical activity and overall well-being in this population.</p>","PeriodicalId":48588,"journal":{"name":"Children-Basel","volume":"12 3","pages":""},"PeriodicalIF":2.0,"publicationDate":"2025-03-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11941570/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143732408","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Child Opportunity Index Predicts Outcomes in Pediatric Spine Trauma: A Novel Application of Social Determinants of Health.","authors":"Gabriel Urreola, Omar Ortuno, Michael Juma, Jose Castillo","doi":"10.3390/children12030380","DOIUrl":"10.3390/children12030380","url":null,"abstract":"<p><p><b>Objectives:</b> Social factors play a crucial role in health outcomes for pediatric patients, yet in the neurosurgery pediatric literature, these factors are rarely reported. To develop a deeper understanding of pediatric spine trauma outcomes, we investigate demographic and social factors measured by the Child Opportunity Index (COI) and Social Deprivation Index (SDI). We hypothesize that social factors predict clinical presentation, injury severity, and clinical outcomes. <b>Methods:</b> We conducted a retrospective cohort study of pediatric patients treated for spinal trauma at a Level 1 trauma center in Sacramento, California. We collected patient clinical data such as mechanisms of injury (MOIs), length of stay (LOS), treatment type, hospital disposition, polytrauma incidence, and follow-up attendance. Each patient's social environment was characterized using COI and SDI metrics. Statistical comparisons were performed to assess associations between social factors and clinical outcomes. <b>Results:</b> Patients with worse childhood opportunity (lower COI and higher SDI) were more likely to be insured through Medi-Cal, identify as Hispanic, and experience violent MOI. Female patients were more likely to sustain polytrauma and had a higher likelihood of requiring surgical intervention. Additionally, patients from underserved communities demonstrated longer hospital stays and poorer follow-up adherence, with COI and SDI scores correlating with these disparities. <b>Conclusion</b>: Social disparities are associated with worse outcomes in pediatric spine trauma. We found COI and SDI to be valuable clinical metrics, motivating further research to be carried out at the state and national levels. These findings highlight health disparities in pediatric spine trauma.</p>","PeriodicalId":48588,"journal":{"name":"Children-Basel","volume":"12 3","pages":""},"PeriodicalIF":2.0,"publicationDate":"2025-03-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11941705/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143732554","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Impact of Fortified Whole Grain Infant Cereal on the Nutrient Density of the Diet in Brazil, the UAE, and the USA: A Dietary Modeling Study.","authors":"Lynda O'Neill, Maria F Vasiloglou, Fanny Salesse, Regan Bailey, Carlos Alberto Nogueira-de-Almeida, Ayesha Al Dhaheri, Leila Cheikh Ismail, Nahla Hwalla, Tsz Ning Mak","doi":"10.3390/children12030384","DOIUrl":"10.3390/children12030384","url":null,"abstract":"<p><strong>Background/objectives: </strong>Complementary feeding (CF) influences future health outcomes. The aim of this study was to evaluate the impact of fortified whole grain infant cereal (WGIC), a complementary food, among 6- to 12-month-old infants on the nutrient density of the diet in three diverse settings: Brazil, the United Arab Emirates (UAE), and the US.</p><p><strong>Methods: </strong>Data from the Feeding Infants and Toddler Study (FITS), a collection of dietary intake studies based on 24-h-dietary recalls, from said countries was utilized. Nutrient intakes were calculated for infant cereal (IC) consumers and non-consumers. Diet modeling was applied to IC consumers to substitute their regular fortified IC with WGIC with improved fortifications. The study estimated the average nutrient density, mean adequacy ratio (MAR), and percentage inadequacy of the diet in both IC consumers and non-consumers.</p><p><strong>Results: </strong>The analyses indicated that infants who consumed IC had higher intakes of calcium, zinc, magnesium, iron, and vitamin D in the three countries. Reduced micronutrient inadequacies were observed among IC consumers, particularly in Brazil and the U.S. Diet modeling with WGIC revealed a significantly higher density of choline, magnesium, zinc, iron, fiber, and protein, as well as reduced inadequacies. The MAR was significantly improved in the three countries.</p><p><strong>Conclusions: </strong>This study underscores the potential of fortified WGIC in increasing the nutrient density of the complementary diet. The intrinsic nutrients in whole grain infant cereals (WGICs) significantly enhance the nutrient density of the complementary diet. Given that whole grains play a role in preventing childhood obesity, their inclusion during CF may be crucial.</p>","PeriodicalId":48588,"journal":{"name":"Children-Basel","volume":"12 3","pages":""},"PeriodicalIF":2.0,"publicationDate":"2025-03-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11941295/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143732476","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Influence of Urban and Rural Areas, Type of School, and Parents' Education Level on Nutrition Habits and Their Relationship with Dental Caries in Schoolchildren in Mallorca.","authors":"Daniela Vallejos, Irene Coll, Nora López-Safont","doi":"10.3390/children12030383","DOIUrl":"10.3390/children12030383","url":null,"abstract":"<p><p><b>Background/Objectives</b>: Habits such as a diet high in sugars and poor dental biofilm control are linked to a higher prevalence of caries and low socioeconomic status. This study aimed to analyze the nutrition habits of schoolchildren in Mallorca and their relationship with the presence of dental caries, depending on the type of school, geographic location, and parents' education level. <b>Methods:</b> A cross-sectional study was conducted to examine the prevalence of dental caries based on World Health Organization (WHO) standards and nutritional practices following guidelines from the Food and Agriculture Organization of the United Nations (FAO) and the European Food Safety Authority (EFSA). This study included 718 students from three age groups: first-year elementary students (ages 5-6), sixth-year elementary students (age 12), and fourth-year secondary school students (age 15). Relevant sociodemographic factors were also considered in the analysis. <b>Results:</b> In schoolchildren aged 5-6 years, higher monthly consumption of processed and sugary foods, such as sweets (rural: 24.66 (CI 95%: 20.30-29.02); urban: 19.29 (CI 95%:16.27-22.304); <i>p</i> = 0.044), was noted in schoolchildren from rural sectors compared to those residing in urban areas. At 15 years of age, there was a higher consumption of potato chips in public schools than in subsidized/private schools (public: 26.95 (CI 95%: 24.42-29.49); subsidized/private: 18.29 (CI 95%: 13.92-22.65) <i>p</i> = 0.004). A high consumption of sweets is associated with an increased risk of caries (OR sweets: 1.76 CI: 1.04-2.98; <i>p</i> = 0.035). Fewer students with mothers with a lower education level eat dinner (elementary: 75%; secondary 91%; higher: 98%; <i>p</i> = 0.003). <b>Conclusions:</b> Higher consumption of sweets in rural areas and potato chips in public schools, along with the association between sweet consumption and caries risk, highlight how geographic location, school type, and parents' education level influence children's nutrition habits and caries.</p>","PeriodicalId":48588,"journal":{"name":"Children-Basel","volume":"12 3","pages":""},"PeriodicalIF":2.0,"publicationDate":"2025-03-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11941127/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143732597","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The Impact of COVID-19 on the Language Skills of Preschool Children: Data from a School Screening Project for Language Disorders in Greece.","authors":"Eleni Kyvrakidou, Giannis Kyvrakidis, Anastasia S Stefanaki, Asterios Asimenios, Athanasios Gazanis, Asterios Kampouras","doi":"10.3390/children12030376","DOIUrl":"10.3390/children12030376","url":null,"abstract":"<p><p><b>Background/Objectives:</b> The COVID-19 pandemic has significantly affected children's lives, particularly preschool-aged children who undergo rapid biological and psychosocial development. This study aimed to investigate the effects of the COVID-19 pandemic on the language skills of preschool children in Greece. <b>Methods:</b> To that end, a widely used screening tool was applied in a screening project involving 213 preschoolers. Language skills were assessed in three groups of children aged 2-4 years old before, during and after the pandemic. <b>Results:</b> A significant increase in the number of children with atypical language skills profile was identified in relation to the preschoolers after the pandemic versus those before or during the pandemic period. A higher prevalence of atypical profiles was observed in girls than in boys. Interestingly, an increase in the number of successfully produced or repeated words and pseudowords, along with enhanced expressive abilities, was observed during the pandemic compared to the periods before and after. <b>Conclusions:</b>Our findings suggest that post-pandemic preschool children exhibit higher rates of atypical language skill profiles compared to those assessed before and during the pan-demic. Given the importance of language development as a critical aspect of children's overall personality and well-being, further research is needed to explore the impact of specific pandemic-related factors on language competency. These factors include mask-wearing, increased screen time, reduced social interaction and exposure to language-rich environments, as well as impaired mental health and parental distress. Additionally, personalized interventions should be developed to support healthier developmental outcomes.</p>","PeriodicalId":48588,"journal":{"name":"Children-Basel","volume":"12 3","pages":""},"PeriodicalIF":2.0,"publicationDate":"2025-03-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11941272/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143732630","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Conventional Cardiopulmonary Resuscitation Versus Extracorporeal Membrane Oxygenation-Assisted CPR in Children: A Retrospective Analysis of Outcomes and Factors Associated with Conversion from the Former to the Latter.","authors":"Adrian C Mattke, Eugene Slaughter, Kerry Johnson, Michelle Low, Kim Betts, Kristen S Gibbons, Renate Le Marsney, Supreet Marathe","doi":"10.3390/children12030378","DOIUrl":"10.3390/children12030378","url":null,"abstract":"<p><strong>Background/objectives: </strong>Conventional cardiopulmonary resuscitation (CCPR) has been the foundational resuscitation approach for decades. Where CCPR is unsuccessful, extracorporeal membrane oxygenation-assisted CPR (ECPR) may improve outcomes. Predicting failure of CCPR and immediate need for ECPR is difficult, and data are lacking. In this retrospective analysis, we analysed both factors that are associated with conversion from CCPR to ECPR and survival outcomes for each event.</p><p><strong>Methods: </strong>Patients having a CPR event that occurred in the PICU between 2016 and 2022 were included. Pre-CPR-event clinical and laboratory data were collected. We recorded whether CPR was converted to ECPR and documented patient outcomes.</p><p><strong>Results: </strong>201 CPR events occurred in 164 children, with 45 events converted from CCPR to ECPR. Time to ROSC or time to ECMO flow was (median [IQR]) 2 (1.5) min for CCPR events and 37 (21.60) min for ECPR events. The maximum pre-CPR-event lactate values were 1.8 mmol/L for CCPR and 4.5 mmol/L for ECPR events. Respiratory arrest preceded 35.3% of CCPR and 4.4% of ECPR events. PICU mortality was 27.8% for CCPR and 50% for ECPR events. Most deaths occurred because of withdrawal of life-sustaining treatments. In a multivariable analysis, cardiac surgical diagnosis, pre-CPR-event lactate, as well as duration of CPR were associated with conversion from CCPR to ECPR.</p><p><strong>Conclusions: </strong>Our study demonstrates that pre-CPR-event lactate concentrations and duration of arrest should alert clinicians to a high likelihood of needing ECPR, while a preceding respiratory arrest may indicate a low likelihood. Mortality post CCPR is significant, mainly due to overall illness severity rather than the consequences of the CPR event.</p>","PeriodicalId":48588,"journal":{"name":"Children-Basel","volume":"12 3","pages":""},"PeriodicalIF":2.0,"publicationDate":"2025-03-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11941317/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143731565","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Early Intervention with a Preformed Eruption Guidance Appliance After the Excision of Cemento-Ossifying Fibroma: A Case Report.","authors":"Yeonjin Ju, Soyoung Park, Jonghyun Shin, Taesung Jeong, Eungyung Lee","doi":"10.3390/children12030379","DOIUrl":"10.3390/children12030379","url":null,"abstract":"<p><strong>Background: </strong>Cemento-ossifying fibroma (COF) is a benign, non-aggressive fibro-osseous tumor in which normal bone is replaced by fibrous tissue containing collagen fibers, bone, or cementum-like material. Although COF is rare in children, its occurrence requires careful management due to its potential impact on tooth development and occlusion. Surgical excision is the widely used treatment; however, post-operative occlusal guidance is crucial, particularly in pediatric patients.</p><p><strong>Case report: </strong>This case report presents the early intervention using a preformed eruption guidance appliance (EGA) following the excision of COF in a 5-year-old boy. The patient exhibited premature loss of the primary canine and displacement of the devel-oping permanent tooth bud. After surgical excision, an EGA was applied to facilitate proper eruption of the successor teeth and optimize oral muscle function. Over a four-year follow-up period, the permanent teeth in the affected area erupted favorably, and intercuspal relationships improved during the transition from primary to mixed dentition.</p><p><strong>Conclusions: </strong>Given the limited research on the long-term effects of COF on developing dentition, long-term follow-up and additional studies are necessary to further evaluate its impact and the effectiveness of eruption guidance appliances in pediatric patients.</p>","PeriodicalId":48588,"journal":{"name":"Children-Basel","volume":"12 3","pages":""},"PeriodicalIF":2.0,"publicationDate":"2025-03-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11941025/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143732237","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Obesity and Overweight Conditions in Children and Adolescents (6-18 Years) and Their Impact on Craniofacial Morphology: A Systematic Review.","authors":"Alessio Verdecchia, Carlota Suárez-Fernández, Ivan Menéndez Diaz, Veronica García Sanz, Enrico Spinas, Teresa Cobo","doi":"10.3390/children12030377","DOIUrl":"10.3390/children12030377","url":null,"abstract":"<p><p><b>Background</b>: Childhood obesity and overweight conditions impact systemic health and craniofacial development. <b>Objectives</b>: This review assessed the influence of elevated body mass index (BMI) on craniofacial morphology, considering age, sex, and ethnicity. <b>Methods</b>: A comprehensive search of Scopus, Web of Science, Embase, Cochrane, PubMed, and OpenGrey was conducted following PRISMA guidelines. Ten cross-sectional studies involving 1383 individuals aged 6 to 18 years were included. The sample comprised 812 females and 571 males, with most studies focusing on adolescents aged 12-18 years of different ethnicities depending on the study. Craniofacial structures were compared between overweight/obese and normal weight groups through cephalometric analysis. Study quality was assessed using the Newcastle-Ottawa Scale (NOS). <b>Results</b>: Overweight and obese individuals showed significant craniofacial changes, including increased anterior cranial base length, maxillary and mandibular dimensions, bimaxillary prognathism, and greater soft tissue thickness. These alterations may be influenced by differences in tissue composition, hormonal fluctuations, fat-to-bone ratio, and metabolic disorders. Variations in skeletal divergence, dental alignment, and airway space were also observed. The methodological quality ranged from moderate to high. <b>Conclusions</b>: Excess weight during growth is linked to distinct craniofacial alterations. Orthodontic diagnostics should integrate metabolic and hormonal considerations to optimize treatment outcomes. These changes should be carefully considered by orthodontists and pediatric dentists. Longitudinal studies are needed to understand the long-term effects of obesity on craniofacial development.</p>","PeriodicalId":48588,"journal":{"name":"Children-Basel","volume":"12 3","pages":""},"PeriodicalIF":2.0,"publicationDate":"2025-03-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11940931/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143732525","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Study of Ovarian Damage in Piglets in an Experimental Model of Neonatal Asphyxia.","authors":"Efstathia-Danai Bikouli, Rozeta Sokou, Monica Piras, Abraham Pouliakis, Eleftheria Karampela, Styliani Paliatsiou, Paraskevi Volaki, Gavino Faa, Theodoros Xanthos, Christos Salakos, Nicoletta M Iacovidou","doi":"10.3390/children12030371","DOIUrl":"10.3390/children12030371","url":null,"abstract":"<p><p><b>Background/Objectives</b>: Perinatal asphyxia constitutes a major complication of the perinatal period with well-described effects on multiple organs and systems of the neonate; its impact, though, on the ovaries is hardly known. The objective of the present study was to investigate potential histological alterations of the ovaries in an animal model of perinatal asphyxia with or without resuscitation. <b>Methods</b>: This was a prospective, randomized animal study; 26 female Large White/Landrace piglets, aged 1-4 days, were the study subjects and were randomly allocated in 3 groups. In Group A (control), the animals had their ovaries surgically removed without any manipulation other than the basic preparation and mechanical ventilation. The other 2 groups, B (asphyxia) and C (asphyxia/resuscitation), underwent asphyxia until bradycardia and/or severe hypotension occurred. At the hemodynamic compromise, animals in group B had their ovaries surgically removed, while animals in group C were resuscitated. Following return of spontaneous circulation (ROSC), the latter were left for 30 min to stabilize and subsequently had their ovaries surgically removed. The ovarian tissues were assessed by the pathologists for the presence of apoptosis, balloon cells, vacuolated oocytes, and hyperplasia of the stroma. The histological parameters were graded from 0 (absence) to 3 (abundant presence). <b>Results</b>: The presence of balloon cells and apoptosis was found to be more prominent in the ovaries of animals in groups B and C, compared to that of the control group at a statistically significant degree (<i>p</i> = 0.0487 and <i>p</i> = 0.036, respectively). A significant differentiation in balloon cell presence was observed in cases with higher grading (2-3) in the asphyxia group (with or without resuscitation) (<i>p</i> value: 0.0214, OR: 9, 95% CI: 1.39-58.4). Although no statistically significant difference was noted regarding the other 2 histological parameters that were studied, there was a marked negative correlation between the duration of asphyxia and grade of vacuoles in oocytes when the potential effect of the duration of asphyxia or resuscitation on the histological findings was investigated (r = -0.54, <i>p</i> = 0.039). <b>Conclusions</b>: We aimed at investigating the potential effect on the neonatal ovaries in our animal model of perinatal asphyxia. Given that the presence of apoptosis and balloon cells was more prominent in cases of asphyxia, it can be speculated that perinatal asphyxia might have an impact on the neonatal ovaries in addition to the other, better-studied systemic effects. More research is needed in order to clarify the potential effect of perinatal asphyxia on the ovaries.</p>","PeriodicalId":48588,"journal":{"name":"Children-Basel","volume":"12 3","pages":""},"PeriodicalIF":2.0,"publicationDate":"2025-03-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11941492/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143732260","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A Focus on the Role of Dietary Treatment in the Prevention of Retinal Dysfunction in Patients with Long-Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency: A Systematic Review.","authors":"Evelina Maines, Giorgia Gugelmo, Nicola Vitturi, Alice Dianin, Laura Rubert, Giovanni Piccoli, Massimo Soffiati, Vittoria Cauvin, Roberto Franceschi","doi":"10.3390/children12030374","DOIUrl":"10.3390/children12030374","url":null,"abstract":"<p><p><b>Background</b>: Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHADD) is an inborn error affecting fatty acid β-oxidation (FAO). Differently than other FAO deficiencies, LCHADD patients may develop progressive retinopathy and peripheral neuropathy. The pathogenesis of retinopathy is not completely understood, and the role of dietary interventions in preventing the development of retinopathy remains uncertain. We examined the literature to assess the impact of the dietary management of LCHADD patients on retinopathy prevention. <b>Methods</b>: Our systematic search included studies published in the last 20 years according to PRISMA guidelines. The aims of the review were to analyze the correlation between retinopathy and the following: (1) age at first metabolic decompensation and/or at the start of the dietary treatment, (2) chronic dietary treatment, (3) emergency regimens, (4) other nutritional supplements. The protocol was registered in PROSPERO, and evidence was assessed using the GRADE system. <b>Results</b>: Seven full papers were identified according to search criteria, with only four including meaningful data. Early presentation of the disease, acute neonatal symptoms, and a suboptimal chronic treatment control were associated with more aggressive retinopathy and a poorer sight outcome. The number of metabolic decompensations and/or hospitalizations were also positively correlated with vision loss. Chronic fat modulation in the diet had less impact than emergency treatments. The role of other nutritional supplements was not well defined. <b>Conclusions</b>: Newborn screening may improve retinal outcomes. Nevertheless, early treatment adopting the current LCHADD therapeutic regimen can often only delay the onset of retinopathy. Clearly, our current treatment strategies are not adequate and retina-specific treatments are needed. The optimal composition of the diet, the role of fasting limitation, and the benefits of some nutritional supplements deserve further investigations.</p>","PeriodicalId":48588,"journal":{"name":"Children-Basel","volume":"12 3","pages":""},"PeriodicalIF":2.0,"publicationDate":"2025-03-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11941211/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143732133","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}