scholarly journals Autistic, Aberrant, and Food-Related Behaviors in Adolescents and Young Adults with Prader-Willi Syndrome: The Effects of Age and Genotype

2017 ◽  
Vol 2017 ◽  
pp. 1-10 ◽  
Author(s):  
Atsushi Ishii ◽  
Hiroshi Ihara ◽  
Hiroyuki Ogata ◽  
Masayuki Sayama ◽  
Masao Gito ◽  
...  

The effects of age and genotype were examined, with regard to the severity of aberrant, autistic, and food-related behaviors in Prader-Willi syndrome (PWS), with an emphasis on the contrast between adolescents and young adults. The Aberrant Behavior Checklist Japanese version (ABC-J), the Food Related Problem Questionnaire (FRPQ), and the Pervasive Developmental Disorders Autism Society Japan Rating Scale (PARS) were administered to 65 PWS patients, including 20 adolescents (ages 12 to 17) and 45 young adults (ages 18 to 29). Significant differences (Mann–Whitney U tests) were found in ABC-J (p=0.004) and PARS (p=0.021), with lower scores in adolescents than in young adults. While DEL subgroups showed no significant differences between the two age groups in ABC-J (p=0.063) and PARS (p=0.134), mUPD subgroups showed a statistically significant difference in terms of ABC-J (p=0.007). No significant differences were found between adolescents and young adults, in terms of FRPQ (p=0.163). These results suggest that aberrant and autistic behaviors follow a marked worsening trend from around the age of 18. On the other hand, food-related behaviors give no sign of change at this transitory stage. Young adults with mUPD were found to be significantly more severe than adolescents with mUPD, in terms of aberrant behaviors.

2015 ◽  
Vol 2015 ◽  
pp. 1-8 ◽  
Author(s):  
Masao Gito ◽  
Hiroshi Ihara ◽  
Hiroyuki Ogata ◽  
Masayuki Sayama ◽  
Nobuyuki Murakami ◽  
...  

Objectives. This study measured gender differences in Prader-Willi syndrome (PWS) in regard to the severity of behavioral symptoms.Methods. The Food Related Problem Questionnaire (FRPQ), the Aberrant Behavior Checklist Japanese Version, the Childhood Routines Inventory, the Pervasive Developmental Disorders Autism Society Japan Rating Scale, and Japanese ADHD-RS were administered to PWS patients (45 males aged 6 to 58 and 37 females aged 6 to 45). To examine the effects that gender and genotype have on the severity of each symptom, two-way ANOVAs were conducted.Results. Significant interactions were found only in regard to FRPQ scores, such as FRPQ total score (F(1, 78)= 8.43,p<0.01). The FRPQ of male deletion (DEL) individuals was higher than that of female DEL and male mUPD. The FRPQ of male maternal uniparental disomy (mUPD) was lower than that of female mUPD.Conclusions. In terms of problem behaviors, routines, autistic behaviors, and hyperactivity, no significant differences were found. Food-related behaviors in DEL were more severe in males, although those in mUPD were less severe in males.


2020 ◽  
Vol 79 (OCE2) ◽  
Author(s):  
Lucille Desbouys ◽  
Manon Rouche ◽  
Karin De Ridder ◽  
Katia Castetbon

AbstractIntroductionThe transition from adolescence toward adulthood is a critical period regarding changes in dietary behaviours. Moreover at these ages, socio-economic and regional disparities in food group consumption are observed. The aim of our study was to determine how the nutritional quality of diet, measured by the modified Nutrient Profiling System of the British Food Standards Agency (FSAm-NPS), evolved between 2004 and 2014, according to socio-economic and regional characteristics of adolescents and young adults living in Belgium.Material and methodsTwo non-consecutive 24-hour dietary recalls were carried out in two nationally-representative samples of 15-to-39-year-old respondents included in the Belgian Food Consumption Surveys in 2004 (n = 1,186) and 2014 (n = 952). Weighting factor (according to age, gender, day of recall, season and province) and sample design were considered. The weighted mean individual FSAm-NPS was computed from all foods and beverages consumed and converted into a scale from 0 (less favourable diet) to 100 (more favourable diet). Slope (SII) and Relative (RII) Index of Inequality were compared between 2004 and 2014 in three age groups (15–18 y, 19–25 y and 26–39 y), adjusting for gender, energy intake, and other socio-economic and regional characteristics.ResultsIn the three age groups, the weighted mean FSAm-NPS significantly increased between 2004 and 2014 (2004: 55.2 (SEM: 0.2) vs. 2014: 57.3 (0.5), p < 0.001 in 15–18-year-olds; 54.9 (0.6) vs. 58.0 (0.4), p < 0.001 in 19–25-year-olds; 56.9 (0.3) vs 58.3 (0.3), p < 0.01 in 26–39-year-olds). While a significant FSAm-NPS gradient was observed according to household education among 15–18- and 26–39-year-olds in 2004 (adjusted SII: 2.56 (95% CI: 1.08–4.04) and 2.73 (0.34–5.12), respectively; RII: 1.05 (1.02–1.08) and 1.05 (1.01–1.09)), no significant difference was found in 2014. Conversely, no significant score disparity was observed according to household type in 2004, but disparities appeared in 2014: index of inequality were significant among subjects aged 19–25 y (SII: 3.89 (0.62–7.17); RII: 1.07 (1.01–1.13)) and 26–39 y (SII: 2.74 (0.31–5.17); RII: 1.05 (1.01–1.09)), the FSAm-NPS being more favourable for those living in two-parent families. The FSAm-NPS was generally higher for subjects living in Flanders than those in Wallonia (significant SII and RII only among 26–39-year-olds), the magnitude of regional disparities remaining stable over time.DiscussionOverall FSAm-NPS improved during this 10-year period, but differentially according to family structure and household education. However, statistical power was weakened by the limited sample size. Additional investigations of changes in food group consumption disparities will complement our interpretations.


Author(s):  
Dhananjay Chaudhari ◽  
Vivek Agarwal ◽  
Prabhat Sitholey

Background: Pervasive Developmental Disorders (PDD) are group of developmental disorder with impairments in interaction, communication and behaviour. The study aims to explore the phenomenological aspects of subjects with PDD.Methods: Patients in Psychiatry outpatient department (OPD), presented with impairment in social- interaction, language, communication and mental retardation were assessed for features of PDD by applying Developmental Behaviour Check List (DBCL), ICD-10 Diagnostic Criteria for Research and Multi-Axial version of ICD-10. The subjects were assessed for severity of PDD on Childhood Autism Rating Scale (CARS).Results: Total number of screened positive cases were 20, in which males were over-represented (90%). Majority belonged to urban locality (65%) and nuclear family (75%). Cases of childhood autism were found in all age groups, while childhood disintegrative disorder, Rett’s disorder and atypical autism were found in younger subjects. No family history of PDD was found in 1st degree relatives of PDD subjects. Five subjects (25%) had birth and perinatal complication.Conclusions: The mean age at presentation of the children with PDD was 8.12 years. Eighty percent (80%) of the subjects had severe autism on CARS.  Hyperactivity, inattention and impulsivity were present in 90%, 80% and 45% of subjects respectively.


2021 ◽  
Vol 10 (20) ◽  
pp. 4746
Author(s):  
Andrea Karoline Mohr ◽  
Constanze Laemmer ◽  
Sandra Schulte ◽  
Bettina Gohlke

To reduce transmission of the coronavirus disease 2019 (COVID-19), many countries implemented lockdowns, causing the closure of childcare services. This study was designed to evaluate the impact of the COVID-19 lockdown in March–April 2020 on children, adolescents, and young adults with Prader–Willi syndrome (PWS) living in Germany. We recruited 180 participants with a genetically confirmed PWS. All families completed a questionnaire, and participants underwent a post-lockdown assessment; the last examination before the lockdown was determined as the pre-lockdown assessment. We used bivariate analyses to compare pre- and post-lockdown outcomes. Weight standard deviation scores (SDSPWS) and body mass index (BMI)-SDSPWS remained stable or even decreased in some age groups. A statistically significant gain in lean body mass (LBM) was found in all groups <18 years of age. We observed an increase in IGF-I and IGFBP-3 concentrations without a significant change in growth hormone (GH) dosage. Most families (95.4%) reported set mealtimes and implementation of structured activities (72.2%) during the lockdown period. We therefore suggest that the favorable development of weight/BMI and LBM was caused by an interplay of a suspected enhanced GH administration and continuous parental commitment. However, more intense behavioral problems were observed in 45.7%, which persisted post-lockdown in 33.7%.


2015 ◽  
Vol 26 (3) ◽  
pp. 415-425 ◽  
Author(s):  
Morten Schrøder ◽  
Kirsten A. Boisen ◽  
Jesper Reimers ◽  
Grete Teilmann ◽  
Jesper Brok

AbstractPurposeWe performed a systematic review and meta-analysis of observational studies assessing quality of life in adolescents and young adults born with CHD compared with age-matched controls.MethodsWe carried out a systematic search of the literature published in Medline, Embase, PsychINFO, and the Cochrane Library’s Database (1990–2013); two authors independently extracted data from the included studies. We used the Newcastle–Ottawa scale for quality assessment of studies. A random effects meta-analysis model was used. Heterogeneity was assessed using the I2-test.ResultsWe included 18 studies with 1786 patients. The studies were of acceptable-to-good quality. The meta-analysis of six studies on quality of life showed no significant difference – mean difference: −1.31; 95% confidence intervals: −6.51 to +3.89, I2=90.9% – between adolescents and young adults with CHD and controls. Similar results were found in 10 studies not eligible for the meta-analysis. In subdomains, it seems that patients had reduced physical quality of life; however, social functioning was comparable or better compared with controls.ConclusionFor the first time in a meta-analysis, we have shown that quality of life in adolescents and young adults with CHD is not reduced when compared with age-matched controls.


2022 ◽  
Vol 22 (1) ◽  
Author(s):  
Xuechan Lyu ◽  
Tianzhen Chen ◽  
Zhe Wang ◽  
Jing Lu ◽  
Chenyi Ma ◽  
...  

Abstract Background In recent years, there have been frequent reports of gaming disorder in China, with more focus on young people. We developed and psychometrically tested a Gaming Disorder screening scale (i.e., Gaming Disorder Screening Scale - GDSS) for Chinese adolescents and young adults, based on the existing scales and diagnostic criteria, but also considering the development status of China. Methods For testing content and criterion validity, 1747 participants competed the GDSS and the Internet Addiction Test (IAT). After 15 days, 400 participants were retested with the scales for to assess test-retest reliability. Besides, 200 game players were interviewed for a diagnosis of gaming disorder. Results The Cronbach’s alpha coefficient on the GDSS was 0.93. The test-retest coefficient of 0.79. Principal components analysis identified three factors accounting for 62.4% of the variance; behavior, functioning, cognition and emotion. Confirmatory factor analysis showed a good model fit to the data (χ2 /df = 5.581; RMSEA =0.074; TLI = 0.916, CFI = 0.928). The overall model fit was significantly good in the measurement invariance tested across genders and different age groups. Based on the clinical interview, the screening cut-off point was determined to be ≥47 (sensitivity 41.4%, specificity 82.3%). Conclusions The GDSS demonstrated good reliability and validity aspects for screening online gaming disorder among Chinese adolescents and young adults.


2021 ◽  
Vol 11 ◽  
Author(s):  
Paolo Del Fiore ◽  
Irene Russo ◽  
Beatrice Ferrazzi ◽  
Alessandro Dal Monico ◽  
Francesco Cavallin ◽  
...  

The “Veneto Cancer Registry” records melanoma as the most common cancer diagnosed in males and the third common cancer in females under 50 years of age in the Veneto Region (Italy). While melanoma is rare in children, it has greater incidence in adolescents and young adults (AYA), but literature offers only few studies specifically focused on AYA melanoma. The aim of this study was to describe the characteristics, surgical treatment, and prognosis of a cohort of AYA melanoma in order to contribute to the investigation of this malignancy and provide better patient care. This retrospective cohort study included 2,752 Caucasian patients (702 AYA and 2,050 non-AYA patients) from the Veneto Region who were over 15 years of age at diagnosis, and who received diagnosis and/or treatment from our institutions between 1998 and 2014. Patients were divided in adolescents and youth (15-25 years), young adults (26-39 years) and adults (more than 39 years) for the analysis. We found statistically significant differences in gender, primary site, Breslow thickness, ulceration, pathologic TNM classification (pTNM) stage and tumor subtype among the age groups. Disease-specific survival and disease-free survival were also different among the age groups. Our findings suggest that the biological behavior of melanoma in young people is different to that in adults, but not such as to represent a distinct pathological entity. Additional and larger prospective studies should be performed to better evaluate potential biological and cancer-specific differences between AYAs and the adult melanoma population.


2019 ◽  
Vol 15 (8) ◽  
pp. 433-441 ◽  
Author(s):  
Adriana Fonseca ◽  
A. Lindsay Frazier ◽  
Furqan Shaikh

Germ cell tumors (GCTs) are rare in childhood, representing only 3.5% of childhood cancers, but a common malignancy in adolescents and young adults (AYAs), accounting for 13.9% of neoplasms in adolescents between age 15 and 19 years. The overall outcomes of patients treated for GCTs are excellent. However, as seen in other cancers, outcomes for AYA patients are significantly worse. Understanding the reasons for this observation has led to different approaches to diagnosis, staging, and treatment. The Malignant Germ Cell International Consortium was created to bring together pediatric, gynecologic, and testicular cancer specialists to promote research initiatives and provide evidence-based approaches in the management of GCTs across different age groups. Collaboration between multiple subspecialties is essential to further understand the disease continuum, the underlying biologic characteristics, and the development of appropriate therapeutic approaches. This review focuses on the unique characteristics of patients with extracranial GCTs in the AYA group.


2016 ◽  
Vol 144 (12) ◽  
pp. 2605-2612 ◽  
Author(s):  
E. BERTI ◽  
S. SOLLAI ◽  
E. ORLANDINI ◽  
L. GALLI ◽  
M. DE MARTINO ◽  
...  

SUMMARYTo evaluate measles incidence and its relevant changes over a 14-year period (2000–2014), we analysed data from the regional hospital discharge database on children and adults hospitalized in Tuscany, Italy. A total of 181 paediatric and 413 adult cases were identified. Despite all the efforts towards regional measles elimination, we observed that the overall measles hospitalization rates for children and adults living in Tuscany globally increased from 0·45 to 0·85/100 000 during the study period (P = 0·001) showing fluctuations due to periodic measles outbreaks. Data stratified by age group showed that the hospitalization rate significantly increased in young adults over the study period, confirming an increase in susceptibility to measles in this subpopulation. Conversely, no statistically significant difference was observed in the hospitalization rate in the other age groups. However, children aged <1 year still exhibit the highest hospitalization rate. Pneumonia represented the most common complication in both the adult and children subsets. No death was reported. Measles still represents a public health problem, and national strategies should be implemented, focusing on emergent susceptible subsets, such as infants and young adults.


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