Real life long-term efficacy and safety of rhGH therapy in children with SHOX deficiency

Objective: This Italian survey aims to evaluate real-life long-term efficacy and safety of rhGH therapy in children with short stature homeobox-containing gene deficiency disorders (SHOX-D) and to identify potential predictive factors influencing response to rhGH therapy. Design and methods: This is a national retrospective observational study collecting anamnestic, anthropometric, clinical, instrumental and therapeutic data in children and adolescents with a genetic confirmation of SHOX-D treated on rhGH. Data were collected at the beginning of rhGH therapy (T0), yearly during the first 4 years of rhGH therapy (T1, T2, T3, T4) and at near-final height (nFH) (T5), when available. Results: 117 SHOX-D children started rhGH therapy (initial dose 0.23 ± 0.04 mg/kg/week) at a mean age of 8.67 ± 3.33years (74% prepubertal), 99 completed the 1st year of treatment, and 46 reached nFH. During rhGH therapy, growth velocity (GV) SDS and height (H) SDS improved significantly. Mean H SDS gain from T0 was +1.14±0.58 at T4 and +0.80 ± 0.98 at T5. Both patients carrying mutations involving intragenic SHOX region (group A) and ones with regulatory region defects (group B) experienced a similar beneficial therapeutic effect. The multiple regression analysis identified the age at the start of rhGH treatment (β -0.31, p = 0.030) and the GV during the first year of rhGH treatment (β 0.45, p = 0.008) as main independent predictor factors of height gain. During rhGH therapy, no adverse event of concern was reported. Conclusions: Our data confirm the efficacy and safety of rhGH therapy in SHOX-D children, regardless the wide variety of genotype

Diagnosis, treatment and prevention of pediatric obesity: consensus position statement of the Italian Society for Pediatric Endocrinology and Diabetology and the Italian Society of Pediatrics

The Italian Consensus Position Statement on Diagnosis, Treatment and Prevention of Obesity in Children and Adolescents integrates and updates the previous guidelines to deliver an evidence based approach to the disease. The following areas were reviewed: (1) obesity definition and causes of secondary obesity; (2) physical and psychosocial comorbidities; (3) treatment and care settings; (4) prevention.The main novelties deriving from the Italian experience lie in the definition, screening of the cardiometabolic and hepatic risk factors and the endorsement of a staged approach to treatment. The evidence based efficacy of behavioral intervention versus pharmacological or surgical treatments is reported. Lastly, the prevention by promoting healthful diet, physical activity, sleep pattern, and environment is strongly recommended since the intrauterine phase

Computer Aided Detection System for Prediction of the Malaise during Hemodialysis

Monitoring of dialysis sessions is crucial as different stress factors can yield suffering or critical situations. Specialized personnel is usually required for the administration of this medical treatment; nevertheless, subjects whose clinical status can be considered stable require different monitoring strategies when compared with subjects with critical clinical conditions. In this case domiciliary treatment or monitoring can substantially improve the quality of life of patients undergoing dialysis. In this work, we present a Computer Aided Detection (CAD) system for the telemonitoring of patients' clinical parameters. The CAD was mainly designed to predict the insurgence of critical events; it consisted of two Random Forest (RF) classifiers: the first one (RF 1 ) predicting the onset of any malaise one hour after the treatment start and the second one (RF 2 ) again two hours later. The developed system shows an accurate classification performance in terms of both sensitivity and specificity. The specificity in the identification of nonsymptomatic sessions and the sensitivity in the identification of symptomatic sessions for RF 2 are equal to 86.60% and 71.40%, respectively, thus suggesting the CAD as an effective tool to support expert nephrologists in telemonitoring the patients

Computer Aided Detection System for Prediction of the Malaise during Hemodialysis

Monitoring of dialysis sessions is crucial as different stress factors can yield suffering or critical situations. Specialized personnel is usually required for the administration of this medical treatment; nevertheless, subjects whose clinical status can be considered stable require different monitoring strategies when compared with subjects with critical clinical conditions. In this case domiciliary treatment or monitoring can substantially improve the quality of life of patients undergoing dialysis. In this work, we present a Computer Aided Detection (CAD) system for the telemonitoring of patients’ clinical parameters. The CAD was mainly designed to predict the insurgence of critical events; it consisted of two Random Forest (RF) classifiers: the first one (RF1) predicting the onset of any malaise one hour after the treatment start and the second one (RF2) again two hours later. The developed system shows an accurate classification performance in terms of both sensitivity and specificity. The specificity in the identification of nonsymptomatic sessions and the sensitivity in the identification of symptomatic sessions for RF2 are equal to 86.60% and 71.40%, respectively, thus suggesting the CAD as an effective tool to support expert nephrologists in telemonitoring the patients

DETERMINANTI DELLA HEALTH RELATED QUALITY OF LIFE (HRQOL) IN ADOLESCENTI OBESI ITALIANI

OBIETTIVI Valutare i determinanti socio-economici, legati allo stile di vita e alla gravità dell’eccesso ponderale della qualità di vita collegata alla salute (HRQoL) in un campione ambulatoriale di adolescenti obesi italiani reclutati in uno studio osservazionale multicentrico. METODI 210 adolescenti (109 M; età 14,2±1,1 anni; range 13-16) con obesità primaria (BMI > 95° percentile curve di Cacciari et al.), BMI medio 32,4±5,3; BMI SDS 2,3±0,6; scolarità padre 10,6±3,7 anni; scolarità madre 10,8±3,8 anni, sono stati arruolati consecutivamente alla prima visita in 12 centri italiani per la cura dell’obesità pediatrica afferenti alla SIEDP. Lo stile di vita è stato indagato con questionario su attività fi sica (quanti giorni alla settimana pratichi almeno 60 min in attività fi sica moderata/intensa?), sedentarietà (somma ore giornaliere di TV, videogiochi, computer) e qualità della dieta (indice Kidmed). La HRQoL dell’adolescente era valutata con il PedsQL™ versione 4, modulo 13-18 anni, composto da 4 scale: funzionamento fisico, emozionale, sociale, scolastico, da cui si ottengono un punteggio del funzionamento fisico, un punteggio del funzionamento psicosociale (emozionale, sociale e scolastico), un punteggio generale. RISULTATI Il PedsQL generale era 72,6±14,6, il funzionamento fisico 73,7±17,8 e il funzionamento psicosociale 72,0±15,3, senza differenze di genere. Il punteggio generale correlava negativamente con età e BMI-SDS e positivamente con il livello culturale di entrambi i genitori, ore di attività fi sica e indice Kidmed. L’analisi della regressione multipla indicava che gli unici determinanti indipendenti associati al PedsQL generale erano livelli di AF (beta 3,041, p<0.01), indice Kidmed (beta 2,488, p<0,02) e BMI-SDS (beta -1,94, p=0,05) controllando per età, livello culturale dei genitori, area geografi ca e ore di sedentarietà. Associazioni indipendenti signifi cative sono state riscontrate tra livelli di AF e i domini del funzionamento fisico (beta 3,628, p<0.01) e psicosociale (beta 2,156, p< 0,04). CONCLUSIONI La significativa associazione tra stile di vita più salutare e qualità della vita negli adolescenti obesi suggerisce l’importanza di promuovere l’attività fisica e migliorare la qualità nella dieta non solo per ottenere un calo ponderale, ma anche per migliorare lo stato di benessere generale

Poor Health Related Quality of Life and Unhealthy Lifestyle Habits in Weight-Loss Treatment-Seeking Youth

Obesity is associated with unhealthy lifestyle behaviors and poor Health Related Quality of Life (HRQOL). The cumulative effect of lifestyle behaviors on HRQOL has been demonstrated in chronically ill adolescents, but not in adolescents with obesity. The present study aimed to assess the association between HRQOL and adherence to the Mediterranean Diet (MD) and/or low levels of physical activity (PA) in a large sample of outpatient adolescents with overweight or obesity seeking weight loss treatment. Four-hundred-twenty participants were enrolled from 10 Italian outpatient clinics. The demographics and anthropometric features, KIDMED scores, and exercise levels of the participants were collected, together with parental features. The HRQOL was assessed by the Pediatric Quality of Life Inventory (PedsQLTM), Adolescents Version 4.0. PedsQL total score and functioning subscales were lower in adolescents who reported one or two unhealthy habits. Compared with the high/intermediate groups, the risk of low HRQOL was twice as high for each unit increase in BMI SDS, while the percentage was reduced by 12.2% for every unit increase in the KIDMED score and by 32.3% for each hour increase of exercise. The clustering of these two unhealthy behaviors conferred a 120% higher risk of low HRQOL. Similarly, adolescents displaying better diet quality and/or a physically more active lifestyle have better physical and psychological functioning. Further studies are needed to disclose whether these characteristics may be predictive of better adherence to weight loss treatment

The Interplay among BMI z-Score, Peer Victmization, and Self-Concept in Outpatient Children and Adolescents with Overweight or Obesity

Research has provided evidence that obesity is associated with peer victimization and low levels of self-concept. No study has examined the relationship between BMI z-score, self-concept in multiple domains, and peer victimization

Interaction gender by weight status for bullying.

<p>The graph show the different increase of bullying in males and females in function of the weight status.</p

Bullying roles by weight status.

<p>Note: Cross tabulation bullying roles by weight status. Observed frequencies (f), percentages (%; by column) and standardized residuals by chi square test (SR)</p><p>* p < .05 (standardized residuals ± 2)</p><p>Bullying roles by weight status.</p

Bullying and weight status.

<p>Note: Comparison between normal weight, overweight, moderate obese and severe obese. Adjusted means, standard errors, percentages, ANOVA’s F values, and statistical significance. Percentages indicate the numbers of subjects who declare to bully others 2 or 3 times a month or more. ANOVA was performed on log transformed values, but untransformed data are shown. Different letters indicate statistical differences (p < .05) between groups based on adjusted means and Sidak post-hoc test</p><p>* p < .05</p><p>** p < .01</p><p>*** p < .001</p><p>Bullying and weight status.</p