Mean Body Mass Index Z-score Research Articles

INFLUENCE OF PRO-INFLAMMATORY ADIPOKINES IN THE VASCULAR PHENOTYPE OF OVERWEIGHT AND OBESE ADOLESCENTS

Objective: Obesity is a complex disease that leads to an adipocyte dysfunction favoring a profile characterized by insulin and leptin resistance with low adiponectin values. The objective of the present study is to assess in overweight and obese subjects the impact of the unfavorable profile of adipokines in blood pressure and vascular function. Design and method: Overweight and obese adolescents referred for cardiovascular risk assessment were included in a cross-sectional study. Height, body weight, and body composition were measured by bioelectrical impedance analysis. Hemodynamics were assessed by office blood pressure. Pulse wave velocity (PWV) and central blood pressure (cBP) were measured with Sphigmocorâ device. The logarithm of Aortic pulse pressure (AoPP) was calculated. Metabolic profile including fasting glucose and insulin levels, lipids profile and uric acid was assessed. Leptin and adiponectin were measured and the leptin/adiponectin ratio (L/A r) was calculated. Results: A total of 60 subjects (mean age 12.5 years, 53% male, and 68% obese) were included. Participants with obesity (mean BMI Z-score 2,3 ± 0.2) had higher levels of daytime SBP (118 Vs 116 mmHg; p = 0.05), and no differences in PWV (5.5 vs 5 m/s; p = 0.06) as compared to overweight. No differences were found in the metabolic profile, in contrast, leptin, was higher in subjects with obesity (43 vs 30 ng/L p = 0.045). Overall, a positive and significant correlation was found between leptin and PWV. Focusing on cBP values, a significant and negative correlation among adiponectin and AoPP was observed. In contrast a positive correlation to L/A r was found (figure). The multiple regression analysis showed that PWV was partially explained by L/A r and AoPP by adiponectin, when age, sex, BMI Zscore, insulin, leptin, and L/A r were included in the model. Conclusions: Obesity leads to an adipokines profile that is related to unfavorable vascular phenotypes in youth.

Youth‐onset type 2 diabetes in Israel: A national cohort

Prevalence of youth-onset type 2 diabetes (T2D) has increased worldwide, paralleling the rise in pediatric obesity. Occurrence and clinical manifestations vary regionally and demographically. We assessed the incidence, and clinical and demographic manifestations of youth-onset T2D in Israel. In a national observational study, demographic, clinical, and laboratory data were collected from the medical records of children and adolescents, aged 10-18 years, diagnosed with T2D between the years 2008 and 2019. The incidence of youth-onset T2D in Israel increased significantly from 0.63/100,000 in 2008 to 3.41/100,000 in 2019. The study cohort comprised 379 individuals (228 girls [59.7%], 221 Jews [58.3%], mean age 14.7± 1.9 years); 73.1% had a positive family history of T2D. Mean body mass index (BMI) z-score was 1.96 ± 0.7, higher in Jews than Arabs. High systolic (≥ 130 mmHg) and diastolic blood pressure (≥ 85 mmHg) were observed in 33.7% and 7.8% of patients, respectively; mean glycosylated hemoglobin (A1c) level at diagnosis was 8.8± 2.5%. Dyslipidemia, with high triglyceride (>150 mg/dl) and low HDL-c (<40 mg/dl) levels, was found in 45.6% and 56.5%, respectively. Microalbuminuria and retinopathy were documented at diagnosis, 15.2% and 1.9%, respectively) and increased (36.7% and 4.6%, respectively) at follow-up of 2.9± 2.1 years. Criteria of metabolic syndrome were met by 224 (62.2%) patients, and fatty liver documented in 65%, mainly Jews. Psychosocial comorbidity was found in 31%. Treatment with metformin (45.6%), insulin (20.6%), and lifestyle modification (18%) improved glycemic control. Youth-onset T2D in Israel has increased significantly and presents a unique profile.

Prenatal Environmental Exposure to Persistent Organic Pollutants and Indices of Overweight and Cardiovascular Risk in Dutch Adolescents.

Persistent organic pollutants (POPs) may have obesogenic effects. Knowledge about the effects of prenatal exposure to POPs on anthropometric measurements and metabolic parameters into adolescence is limited. Therefore, the aim of the current study was to determine whether prenatal environmental exposure to several POPs is associated with indices of overweight and cardiovascular risk in 13–15-year-old children. In this Dutch observational cohort study, 194 mother–infant pairs were included (1998–2002). Maternal pregnancy serum levels of PCBs, OH-PCBs, PBDEs, and other POPs were measured. At follow-up (2014–2016), levels of cholesterol, HDL-C, LDL-C, triglycerides, fasting insulin, fasting glucose, leptin, and adiponectin were measured in their children. The children’s height, weight, waist circumference, hip circumference, and blood pressure were measured. In total, 101 adolescents (14.4 ± 0.8 years; 53.7% of invited) participated of which 55 were boys. Mean BMI was 19.1 ± 3.6 kg/m2 and mean BMI z-score 0.13 ± 1.14. Higher prenatal levels of PCBs were associated with lower levels of HDL-C and adiponectin in boys and higher levels of PBDEs with higher triglycerides in girls. We found significant differences by sex in the associations with OH-PCBs, with lower HDL-C and adiponectin, higher LDL-C/HDL-C ratio, fasting glucose, HOMA2-IR, height, and weight for boys. Our study indicates that higher prenatal exposure to PCBs, OH-PCBs, and PBDEs was associated with adolescent levels of some metabolic cardiovascular risk markers and hormones associated with the development of obesity and cardiovascular disease.

Trend in BMI z-score among Private Schools’ Students in Delhi using Multiple Imputation for Growth Curve Model

Objective: The aim of the study is to assess the trend in mean BMI z-score among private schools’ students from their anthropometric records when there were missing values in the outcome. Methodology: The anthropometric measurements of student from class 1 to 12 were taken from the records of two private schools in Delhi, India from 2005 to 2010. These records comprise of an unbalanced longitudinal data that is not all the students had measurements recorded at each year. The trend in mean BMI z-score was estimated through growth curve model. Prior to that, missing values of BMI z-score were imputed through multiple imputation using the same model. A complete case analysis was also performed after excluding missing values to compare the results with those obtained from analysis of multiply imputed data. Results: The mean BMI z-score among school student significantly decreased over time in imputed data (β= -0.2030, se=0.0889, p=0.0232) after adjusting age, gender, class and school. Complete case analysis also shows a decrease in mean BMI z-score though it was not statistically significant (β= -0.2861, se=0.0987, p=0.065). Conclusions: The estimates obtained from multiple imputation analysis were better than those of complete data after excluding missing values in terms of lower standard errors. We showed that anthropometric measurements from schools records can be used to monitor the weight status of children and adolescents and multiple imputation using growth curve model can be useful while analyzing such data

Abstract 008: Randomized Trial Of A Healthy Weight Intervention For Youth With Mental Illness: The Champion Trial

Introduction: Overweight and obesity in youth with serious emotional disturbance (SED) is exceedingly common. In 2015 the AHA called attention to mental illnesses in youth as important risk conditions for early CVD and the need for transformational change in management of overweight and obesity in this group. Our objective was to test a 12-month, innovative healthy weight intervention in youth with SED. Hypothesis: The active intervention is more effective than control in decreasing BMI Z-score compared at 12 m. Methods: We conducted a two-arm randomized trial in 2 outpatient pediatric mental health settings in 112 youth, ages 8-18 yrs. The active intervention group was offered 12m of in-person and virtual individual weight management sessions led by health coaches who provided guidance on improving diet and increasing physical activity, and engaged parents. Results: At baseline, mean (SD) age was 13.0 (2.7) yrs with 46% ages 8-12 and 54% 13-18; 55% were male, 46% Black, 39% had household income less than $50K/yr and 31% lived in a single-parent household. Primary diagnoses were ADHD (41%), major depression (23%), and anxiety (23%). Mean BMI Z-score (SD) was 2.0(0.4), BMI 30.4 (6.4) kg/m 2 .Mean(SD) psychotropic medications were 2.1(1.4). At 12m, 111 (99%) had a follow-up weight; 42 were collected after the onset of the COVID pandemic). The intervention group compared to the control group had 0.15 decrease in BMI Z-Score (95% CI 0.26 to 0.04), p&lt;0.007) between baseline and 12 m (Figure) and a 1.43 kg/m 2 decrease in BMI (95% CI 2.43, 0.42, p&lt;0.006). Estimated net effect on BMI Z-score for intervention vs. control was enhanced during the pandemic but not statistically different from net effects pre-pandemic (p=0.06). Conclusions: A weight control intervention designed for children with SED decreased BMI Z-score substantially over 12 months, including during the COVID-19 pandemic. These results provide empirical evidence in support of weight control programs in a population at high risk for early development of CVD risk factors.

Creatinine-based GFR-estimating equations in children with overweight and obesity

BackgroundWith the increasing prevalence of childhood obesity and related development of chronic kidney disease (CKD), there is a critical need to understand how best to assess kidney function in children with obesity. Since serum creatinine (SCr) is recommended as marker of first choice for GFR estimation, we evaluated and compared creatinine-based GFR equations in children with overweight and obesity.MethodsSix hundred children with overweight and obesity (53.5% female; mean age 12.20 ± 3.28 years; mean BMI z-score 3.31 ± 0.75) were included from the Centre for Overweight Adolescent and Children’s Healthcare (COACH).ResultsSerum creatinine (SCr), normalized using Q-age polynomials obtained from reference values, results in median and mean SCr/Q value close to “1” for all age groups, and 96.5% of the children have a SCr/Q within the reference band [0.67–1.33], corresponding to the 2.5th and 97.5th percentile. eGFR CKiD (bedside Schwartz equation) and Schwartz-Lyon decreased with age, whereas eGFR EKFC and modified CKD-EPI40 showed no age-dependency, but the distribution of eGFR values was not symmetrical. eGFR CKiD under 25 (CKiDU25) demonstrated no age-dependency but major sex differences were observed. eGFR FAS age, FAS height, and adjusted-creatinine revised Lund-Malmö (LMR18) showed a relatively symmetrical distribution and no age-dependency.ConclusionsSerum creatinine (SCr) values of children with overweight and obesity are mostly within the reference range for children. Normalization of SCr using reference Q-age polynomials works very well in this cohort. After evaluation of the different equations, we suggest that FAS age, FAS height, and LMR18 are the preferred creatinine-based GFR-estimating equations in children with overweight and obesity.Clinical trial registration.ClinicalTrial.gov; Registration Number: NCT02091544.Graphical abstractA higher resolution version of the Graphical abstract is available as Supplementary information

Management precautions for risk of obesity are necessary among infants with PKU carrying the rs113883650 variant of the LAT1 gene: A cross-sectional study.

Patients with phenylketonuria (PKU), an inborn error of phenylalanine metabolism, require consistent treatment to avoid the brain toxicity caused by hyperphenylalaninemia. The treatment consists of life-long use of a low-phenylalanine diet, which aims at decreasing hyperphenylalaninemia and maintaining blood phenylalanine concentration in a safe range. Problems with balancing diet can result in suboptimal treatment outcomes; however, recent findings suggest that genetic alteration of the transport of phenylalanine might result in an additional health burden. We assessed the effect of a common variant (rs113883650) of the LAT1(SLC7A5) gene, which encodes the main transmembrane phenylalanine transporter, on the development of overweight in 54 infants with PKU who received standard therapy and adhered well to therapeutic prescriptions, and in 55 infants with a milder disease form—the so-called mild hyperphenylalaninemia (MHP), which does not require treatment. We found that infants with PKU—carriers of the rs113883650 variant had significantly higher Body Mass Index (BMI) at 1 year compared to PKU infants without the variant (mean BMI Z-Score of +1.15 SD vs -0.15 SD, respectively; t(52) = 5.25, p = 0.00005). Conversely, no significant BMI differences were detected in the subgroups of infants with MHP (t(53) = 1.15, p = 0.25). Additionally, high BMI in infants with PKU—carriers of the rs113883650 variant positively correlated with high variability of their blood phenylalanine levels (r(52) = 0.42, p = 0.002). It should be noted that this is an observational study, which does not determine causation. Nevertheless, our findings show that the rs113883650 variant of the LAT1 gene may be a risk factor for overweight in properly treated infants with PKU. Management precautions should be taken to prevent the development of overweight and obesity.

ORAL CHANGES IN PEDIATRIC PATIENTS WITH EATING DISORDERS.

Numerous oral changes develop as a result of dysfunctional eating behavior in patients with eating disorders (ED). The aim of this study was to evaluate the correlation among oral manifestations, age, disease duration and nutritional status in pediatric patients with ED. The study included 50 female ED patients, median age 14 (range 10-18) years and median disease duration 9 (range 1-42) months. Nutritional status was expressed as z-score for body mass index (BMI). Mean BMI z-score was -2.10±1.64. The most commonly observed oral findings were dental plaque, marginal gingivitis, morsicatio, dental calculus, caries, pharyngeal erythema, exfoliative cheilitis and angular cheilitis. Dental plaque and pharyngeal erythema were correlated with shorter disease duration (p=0.048; p=0.040), while frictional keratosis of tongue was correlated with longer disease duration (0.011). Linea alba and pain in the temporomandibular joint were associated with younger age (p=0.012; p=0.024), and tooth impression on tongue with lower degree of nutrition (p=0.030). This study showed that there was a link among oral manifestations, age, disease duration and degree of nutritional disorder, although further investigations comparing the groups of ED patients with different age, disease duration and nutritional status would give better, concrete and precise conclusions.

Growth Failure in Pediatric Inflammatory Bowel Disease: A Preliminary Analysis

Background: Inflammatory bowel disease (IBD) is a chronic relapsing disease, characterized by chronic inflammation of the gastrointestinal tract (GIT). The incidence of IBD has been increasing over the last two decades, for example, from 1999- 2008 incidence increased from 21.3 to 26.2 per 100,000. Inflammatory bowel disease is mainly classified into two types: Crohn’s disease (CD), and ulcerative colitis (UC). Both types can be identified and distinguished through laboratory tests such as ESR, CRP, and fecal calprotectin, radiographic imaging, and direct visualization via colonoscopy. The etiology of growth failure in IBD, BMI z-score of less than -2.5, is poorly understood; however, chronic inflammation, low-calorie intake, and consequential steroid treatment are the most important factors. This study aims to determine the incidence of growth failure and associated factors in pediatric patients with IBD.&#x0D; Methods: This was a retrospective cohort study of children aged 15 years and below, with the diagnosis of IBD. Data were obtained from the pediatric gastroenterology department pediatric inflammatory bowel disease database from 2007 to 2017. Our study identifies the prevalence of growth failure via a calculated BMI z-score of less than -2.5 among the pediatric inflammatory bowel disease (PIBD) population. The Chi-square test is used for categorical data and Wilcoxon rank-sum test for continuous data. A p-value &lt;0.05 was considered statistically significant. All analyses were carried out with Stata IC/15.1 (StataCorp LP, College Station, TX, USA).&#x0D; Results: A total of 36 patients were included. The mean age of children with growth failure at the time of IBD diagnosis was 8 (±4), and more than half of them (58.3%) were boys. The incidence of growth failure at the time of diagnosis was 22.2% and declined to 11.1% over 26 months. Furthermore, the mean BMI z-score gradually improved from -0.741 to -0.1 over the same period of follow-up after proper management and multidisciplinary involvement.&#x0D; Conclusion: Growth failure in IBD patients according to our data is uncommon, however, it was reduced by 50% over 26 months. This could be attributed to nutritional status improvement over the management period. We recommend following the stepwise approach according to the severity of the disease and managing IBD patients by a multidisciplinary team. However, due to the relatively small sample size and low growth failure incidence, a large-scale study is recommended to determine any causal relations.

Intuitive eating Scale-2: Factor structure and associations with disordered eating, impulsivity and quality of life in adolescents with overweight/obesity

ObjectiveThe Intuitive Eating Scale-2 (IES-2) has been shown to be a valid tool to assess the capability of eating in reaction to natural hunger/satiety cues. However, its factor structure seems to differ in function of cultural/socioeconomic backgrounds, and its psychometric properties among the adolescents with overweight/obesity (BMI-for-age percentile >85th) have not been examined yet. Thus, this study aims to 1) investigating the factorial structure/psychometric properties of IES-2 in adolescents with overweight/obesity; and 2) exploring the associations between impulsivity, quality of life disordered and intuitive eating. MethodsA total of 202 Portuguese adolescents (124 girls; 78 boys; 12–19 years) under weight-loss treatment with a mean BMI z-score of 2.41 (SD = 0.75) participated in this study. The IES-2 factor structure was explored by confirmatory factor analysis and bifactor models. Test-retest reliability analyses were performed over 6 months (n = 41) and associations between the variables under study were explored. ResultsConfirmatory factor analyses with posthoc modifications resulted in a bifactor model with acceptable fit supporting one general factor (intuitive eating) and three specific factors (IES-2 subscales). The “Unconditional Permission to Eat” subscale could not be replicated in this sample. Test-retest reliability analyses suggested good temporal stability. Intuitive eating scores were negatively associated with grazing eating behavior and impulsivity (negative urgency) and positively linked to quality of life. ConclusionsAn adjusted version of IES-2 can be an appropriate measure for assessing intuitive eating levels in adolescents with overweight/obesity. Research on intuitive eating has the potential to enhance pediatric weight-loss interventions.

Evaluating the use of laparoscopic-assisted gastrostomy tube feeding in children with epidermolysis bullosa: A single-center retrospective study

BackgroundNutritional management of children with epidermolysis bullosa (EB) presents multiple challenges including reduced oral intake compounded by mucosal fragility. Gastrostomy tube feeding is effective in improving nutritional status however there is limited data on the safety and tolerance of this technique in EB children. We aim to review the effectiveness and morbidity of our minimally invasive two-port laparoscopic-assisted gastrostomy (LAG) approach using Seldinger techniques with serial dilatations in children with EB. MethodsA retrospective, observational cohort study was conducted on all consecutive EB patients who underwent LAG tube insertion between 2009 and 2019. Patient demographics, admission details and 12-month clinical outcomes were reported. Results32 EB patients underwent LAG placement. Median age at insertion was 7.3 (IQR ± 6.3) years, with 8 (25.0%) and 3 (9.4%) of patients also undergoing oesophageal dilatation and fundoplication, respectively. Minor complications arose in 58.1% of patients including: peri-stomal overgranulation (25.8%), gastrostomy infection (22.6%), pain (22.6%), mild gastrostomy leakage (16.1%), blockage (9.7%) and device failure (3.2%). 2 patients (6.5%) developed major complications with extensive gastrostomy site leakage. Improvements in growth were reflected in mean height Z-scores (-1.99 to -1.71). Mean weight Z-scores improved in patients aged 0–10 years (-2.30 to -1.61) and mean BMI Z-scores increased in patients more than 10 years (-2.71 to -1.46). No cases of gastrostomy-related mortality were reported. ConclusionLAG is well-tolerated in EB patients with improvements in growth and minimal morbidity 12-months post-gastrostomy insertion. An extended follow-up period is required to ascertain the long-term implications of gastrostomy feeding.

Initial response of young people with thyrotoxicosis to block and replace or dose titration thionamide.

ObjectivePatients with thyrotoxicosis are treated with anti-thyroid drug (ATD) using block and replace (BR) or a smaller, titrated dose of ATD (dose titration, DT).DesignA multi-centre, phase III, open-label trial of newly diagnosed paediatric thyrotoxicosis patients randomised to BR/DT. We compared the biochemical response to BR/DT in the first 6 months of therapy.MethodsPatients commenced 0.75 mg/kg carbimazole (CBZ) daily with randomisation to BR/DT. We examined baseline patient characteristics, CBZ dose, time to serum thyroid-stimulating hormone (TSH)/free thyroxine (FT4) normalisation and BMI Z-score change.ResultsThere were 80 patients (baseline) and 78 patients (61 female) at 6 months. Mean CBZ dose was 0.9 mg/kg/day (BR) and 0.5 mg/kg/day (DT). There was no difference in time to non-suppressed TSH concentration; 16 of 39 patients (BR) and 11 of 39 (DT) had suppressed TSH at 6 months. Patients with suppressed TSH had higher mean baseline FT4 levels (72.7 vs 51.7 pmol/L; 95% CI for difference 1.73, 31.7; P = 0.029). Time to normalise FT4 levels was reduced in DT (log-rank test, P = 0.049) with 50% attaining normal FT4 at 28 days (95% CI 25, 32) vs 35 days in BR (95% CI 28, 58). Mean BMI Z-score increased from 0.10 to 0.81 at 6 months (95% CI for difference 0.57, 0.86; P < 0.001) and was greatest in patients with higher baseline FT4 concentrations.ConclusionsDT-treated patients normalised FT4 concentrations more quickly than BR. Overall, 94% of patients have normal FT4 levels after 6 months, but 33% still have TSH suppression. Excessive weight gain occurs with both BR and DT therapy.

The impact of parental migration background on cardiometabolic health in European children

Abstract Background There is accumulating evidence that children from migrant families have higher risk of developing overweight and obesity than their peers in the host population. This study aims to investigate the association between parental migration and cardiometabolic health, including the metabolic score and its components, in children and adolescents in Europe. Methods Data was collected from 8,828 children (aged 2-17) in eight European countries included in the multicentre IDEFICS/I.Family cohort. Multilevel linear regression models were used (accounting for correlations between siblings) to investigate the association between parental migration background (one or two migrant parent(s) vs native parents) and the cardiometabolic health outcomes body mass index (BMI), metabolic score and its individual components. Outcome variables were parametrized as age and sex-specific z-scores. We controlled for socioeconomic indicators, maternal weight status, as well as lifestyle and dietary patterns. Results We found a higher mean BMI z-score (+0.24 SD, p &amp;lt; 0.001) and overall metabolic z-score (+0.28 SD, p &amp;lt; 0.001) among children with two migrant parents in comparison to children with two native parents. No differences could be seen between children with one versus no migrant parent. The effects were attenuated when controlling for maternal weight status and levels of sport club activity. Parental education modified the associations for some health outcomes (BMI and waist circumference) such that they were more pronounced in children with low parental education. Conclusions European children and adolescents with two-parent migration background seem to be at higher risk of developing adverse cardiometabolic health than their peers. Children with one migrant parent seem to have similar risk as children with native parents. Maternal BMI and levels of physical activity partly explained the higher risk, a risk which was more prevalent in families with lower education level. Key messages European children and adolescents with two-parent migration background have higher risks of developing adverse cardiometabolic health than their peers with one or two native parents. The higher risk of being overweight and having cardiometabolic risk factors among children with two-parent migration background was most pronounced in families with low level of education.

Nocturnal non-dipping on 24-h Ambulatory Blood Pressure Monitoring in children and adolescents with obesity is associated with higher total cholesterol levels

ABSTRACT Background Few studies indicate the occurrence of abnormal nocturnal dipping of blood pressure (BP) in 35–50% of children and adolescents with obesity. The relation between that phenomenon and metabolic complications of obesity remains unclear. To evaluate the association between disorders of glucose and lipid metabolism, and nocturnal non-dipping in pediatric patients with obesity. Methods In 207 children (53.14% girls, mean age 14 (range 2–17), mean BMI Z-SCORE 4.38, range 2.07–10.74) standard 24-h Ambulatory Blood Pressure Monitoring was performed. Normal dipping was defined as a ≥ 10% decline in BP during the night. Results There were 106 (51.21%) cases of non-dippers. The mean 24-h nocturnal systolic BP (SBP) reduction (%) was 9.9 ± 5.5. The mean 24-h nocturnal diastolic BP (DBP) reduction (%) was 15.8 ± 8.5. There was a significant correlation between BMI Z-SCORE and mean day-time SBP (r = 0.14 P = .042). There are positive correlations between 24-h heart rate (beats/min) and BMI Z-SCORE (r = 0.15, P = .027), between fasting glucose and systolic BP Z-SCORE (r = 0.17, P = .03) and between mean diastolic BP and LDL cholesterol (r = 0.23, P = .004). Total cholesterol level was significantly higher in non-dippers (4.34 vs. 3.99 mmol/L, P = .034). There were no significant differences between non-dippers and dippers regarding fasting glucose (4.6 vs. 4.8 mmol/L), 120ʹpost load glucose (5.7 vs. 5.9 mmol/L), insulin (19 vs. 20.2 µIU/mL), HOMA-IR (2.36 vs. 2.44), LDL cholesterol (2.64 vs. 2.51 mmol/L), HDL cholesterol (1.06 vs. 1.03 mmol/L) or triglycerides (1.36 vs. 1.34 mmol/L) levels. Conclusion Nocturnal non-dipping is frequent in pediatric patients with obesity. It is associated with higher total cholesterol levels.

Demographic characteristics, clinical and laboratory features, and the distribution of pathogenic variants in the CFTR gene in the Cypriot cystic fibrosis (CF) population demonstrate the utility of a national CF patient registry

BackgroundSpecialized clinical care for cystic fibrosis (CF) in Cyprus, a small island country, has been implemented since the 1990s. However, only recently, a national CF patient registry has been established for the systematic recording of patients’ data. In this study, we aim to present data on the epidemiological, genotypic and phenotypic features of CF patients in the country from the most recent data collection in 2019, with particular emphasis on notable rare or unique cases.ResultsOverall, data from 52 patients are presented, 5 of whom have deceased and 13 have been lost to follow-up in previous years. The mean age at diagnosis was 7.2 ± 12.3 years, and the mean age of 34 alive patients by the end of 2019 was 22.6 ± 13.2 years. Patients most commonly presented at diagnosis with acute or persistent respiratory symptoms (46.2%), failure to thrive or malnutrition (40.4%), and dehydration or electrolyte imbalance (32.7%). Sweat chloride levels were diagnostic (above 60 mmol/L) in 81.8% of examined patients. The most common identified mutation was p.Phe508del (F508del) (45.2%), followed by p.Leu346Pro (L346P) (6.7%), a mutation detected solely in individuals of Cypriot descent. The mean BMI and FEV1 z-scores were 0.2 ± 1.3 and − 2.1 ± 1.7 across all age groups, respectively, whereas chronic Pseudomonas aeruginosa colonization was noted in 26.9% of patients. The majority of patients (74.5%) were eligible to receive at least one of the available CFTR modulator therapies. In 25% of patients we recovered rare or unique genotypic profiles, including the endemic p.Leu346Pro (L346P), the rare CFTR-dup2, the co-segregated c.4200_4201delTG/c.489 + 3A > G, and the polymorphism p.Ser877Ala.ConclusionsCF patient registries are particularly important in small or isolated populations, such as in Cyprus, with rare or unique disease cases. Their operation is necessary for the optimization of clinical care provided to CF patients, enabling their majority to benefit from evolving advances in precision medicine.

Use of a Mobile Lifestyle Intervention App as an Early Intervention for Adolescents With Obesity: Single-Cohort Study.

BackgroundEffective, resource-efficient treatment is urgently needed to address the high rates of pediatric and adolescent obesity. This need has been accelerated by the COVID-19 pandemic. The use of a mobile health tool as an early intervention before a clinic-based multidisciplinary weight management program could be an effective treatment strategy that is appropriate during a pandemic.ObjectiveThis study aims to assess the effectiveness of and adolescent engagement with a mobile app–based lifestyle intervention program as an early intervention before enrollment in a clinic-based multidisciplinary weight management program.MethodsThis prospective single-cohort study involved adolescents, aged 10-16 years, who were overweight and obese (defined as BMI percentile above the 85th percentile). Participants used the mobile Kurbo app as an early intervention before enrolling in a clinic-based multidisciplinary weight management program. Kurbo’s health coaches provided weekly individual coaching informed by a model of supportive accountability via video chat, and participants self-monitored their health behavior. The implementation of Kurbo as an early intervention was evaluated using the reach, effectiveness, adoption, implementation, and maintenance framework by reach (number who consented to participate out of all patients approached), implementation (Kurbo engagement and evaluation), and effectiveness as measured by the primary outcome of the BMI z-score at 3 months. Secondary outcome measures included changes in body fat percentage, nutrition and physical activity levels, and quality of life at 3 months. Maintenance was defined as the outcome measures at 6-month follow-up.ResultsOf the 73 adolescents who were approached for enrollment, 40 (55%) of adolescents were recruited. The mean age was 13.8 (SD 1.7) years, and the mean BMI z-score was 2.07 (SD 0.30). In the multiethnic Asian sample, 83% (33/40) of the participants had household incomes below the national median. Kurbo engagement was high, with 83% (33/40) of participants completing at least 7 coaching sessions. In total, 78% (18/23) of participants rated the app as good to excellent and 70% (16/23) stated that they would recommend it to others. There were no statistically significant changes in BMI z-scores at 3 months (P=.19) or 6 months (P=.27). Participants showed statistically significant improvements in measured body fat percentage, self-reported quality of life, and self-reported caloric intake from the 3-day food diaries at 3 and 6 months.ConclusionsThe use of Kurbo before enrollment in an outpatient multidisciplinary clinical care intervention is a feasible strategy to expand the reach of adolescent obesity management services to a low-income and racially diverse population. Although there was no significant change in BMI z-scores, the use of Kurbo as an early intervention could help to improve quality of life and reduce body fat percentage and total caloric intake.

Maternal Emotional States in Relation to Offspring Weight and Health-Related Quality of Life: Tehran Lipid and Glucose Study.

BackgroundMaternal characteristics have been known to be associated with parenting practices, which could eventually influence their child’s weight and health-related quality of life (HRQoL).ObjectivesThis study aimed to assess the direct and indirect associations of maternal emotional states (depression, anxiety, and stress) with body mass index (BMI) and HRQoL in their children.MethodsThis study was conducted within the framework of the Tehran lipid and glucose study (TLGS). Participants were the children (n = 231) enrolled in TLGS during 2014 - 2016, who had complete data on maternal emotional states. The body weight and height of children were measured using the standard protocol, and BMI Z-score was determined using Anthroplus. Also, HRQoL in children and emotional states in mothers were assessed using the Iranian version of the pediatric quality of life inventory (PedsQLTM4.0) and the depression, anxiety, and stress scale (DASS-21), respectively. Structural equations modeling (SEM) was used to assess the direct and indirect relations of maternal emotional states with children’s BMI Z-score and HRQoL.ResultsMean age, BMI Z-score, and HRQoL total score in children were 13.8 ± 3.1 years, 0.74 ± 1.5, and 84.7 ± 11.3, respectively. In the mothers, median DASS-21 scores (interquartile ranges) in the three scales of depression, anxiety, and stress were 4 (0 - 10), 6 (2 - 12), and 14 (8 - 20), respectively. Maternal level of education was significantly associated with the DASS-21 score (β = -0.23, 95% CI: -0.37,-0.07). Maternal DASS-21 score was significantly associated with BMI Z-score only in girls (β = 0.25, 95% CI: 0.06, 0.53). Significant determinants of HRQoL in boys were the child’s age (β = -0.21, 95% CI: -0.40, -0.01) and maternal education (β = -0.24, 95%CI: -0.44, -0.02) and emotional state (β = -0.24, 95% CI: -0.44, -0.03). The child’s age (β = -0.33, 95% CI: -0.53, -0.10) and maternal emotional state (β = -0.31, 95% CI: -0.54, -0.08) were significantly associated with HRQoL in girls.ConclusionsThe maternal emotional state is an important determinant of HRQoL in children, regardless of their weight status. Further research is recommended to examine the current hypothesized model in rural and suburban populations, taking into consideration more influential factors.

Long-term outcome of pancreatic function following oncological surgery in children: Institutional experience and review of the literature.

BACKGROUNDPancreatic neoplasms are uncommon in children and in most cases they are benign or have low malignant potential. Pancreatoblastoma and solid pseudopapillary tumor are the most frequent types in early and late childhood, respectively. Complete resection, although burdened by severe complications, is the only curative treatment for these diseases. Pancreatic surgery may result in impaired exocrine and endocrine pancreatic function. However, limited data are available on the long-term pediatric pancreatic function following surgical resection.AIMTo investigate endocrine and exocrine pancreatic function and growth after oncological pancreatic surgery in a pediatric series.METHODSA retrospective analysis of all pediatric patients who underwent surgery for pancreatic neoplasm in our Institution from January 31, 2002 to the present was performed. Endocrine and exocrine insufficiency, auxological and fat-soluble vitamin status (A, D, E and clotting tests) were assessed at diagnosis and at every follow-up visit. Exocrine insufficiency was defined as steatorrhea with fecal elastase-1 < 200 µg/g stool, while endocrine insufficiency was identified as onset of Diabetes or Impaired Glucose Tolerance. Growth was evaluated based on body mass index (BMI) z-score trend.RESULTSSixteen patients (12 girls and 4 boys, mean age 10.7 ± 5.3 years), were included. Nine patients (56%) had a neoplasm in the pancreatic head, 4 in the body/tail, 2 in the tail and 1 in the body. Histological findings were as follows: Solid pseudopapillary tumor in 10 patients (62.5%), insulinoma in 2 patients, neuroendocrine tumor in 2 patients and acinar cell carcinoma in 2 patients. The most frequent surgery was pancreaticoduodenectomy (50%). Exocrine failure occurred in 4 patients (25%) and endocrine failure in 2 patients (12.5%). Exocrine insufficiency occurred early (within 6 mo after surgery) and endocrine insufficiency later (8 and 10 years after surgery). Mean BMI z-score was 0.36 ± 1.1 at diagnosis and 0.27 ± 0.95 at the last assessment. Vitamin D was insufficient (< 30 ng/mL) in 8 of the 16 patients during the follow-up period. Vitamins A, E and clotting test were into the normal ranges in all patients.CONCLUSIONCareful and long-term monitoring should follow any pancreatic surgery, to recognize and promptly treat exocrine and endocrine pancreatic insufficiency, which can occur after surgery.

Brain-Derived Neurotropic Factor, Vascular Endothelial Growth Factor and Matrix Metalloproteinases as Markers of Metabolic Status in Non-Growth Hormone-Treated Girls With Turner Syndrome.

BackgroundTurner syndrome (TS) presents a high risk of congenital heart defects and may predispose to both obesity and related metabolic complications. Hence the search for new markers as potential early predictors of the metabolic syndrome (MetS) and cardiovascular diseases appears warranted.ObjectiveTo assess MMP-1 (matrix metalloproteinase-1), MMP-2 (matrix metalloproteinase-2), MMP-9 (matrix metallopeptidase-9), BDNF (brain-derived neurotrophic factor), GDNF (glial cell line-derived neurotrophic factor), and VEGF (vascular endothelial growth factor) in non-MetS TS girls not treated with growth hormone (GH) vs. healthy short stature girls, and to assess the connection with basic metabolic parameters.MethodThe concentrations of circulating MMP-1, MMP-2, MMP-9, BDNF, GDNF and VEGF were measured in 12 patients with TS not treated with growth hormone. The control group was composed of 17 girls with non-pathologic short stature. The patients’ clinical and biochemical phenotypes were determined by weight, height, total cholesterol, HDL cholesterol, triglycerides, glucose, aminotransferases, IGF1, TSH and fT4.ResultsThere were no differences in mean age, weight, BMI Z-Score, or hSDS between the studied group and the controls; however, they differed in baseline values of ALT (18.2 ± 4.2 vs. 14.2 ± 4.1, p= 0.02), BDNF [29951.5 (26176.9 – 41271.9) vs. 23131.7 (18392.4 – 28313.3), p=0.01] and MMP-2 [91.8 (71.7 – 111.0) vs. 143.6 (123.7 - 244.5), p< 0.001]. BDNF correlated with ALT activity (r = 0.56 p = 0.002) and BMI Z-score (r = 0.38 p = 0.042), while MMP-2 correlated with HDL concentration (r = 0.48 p = 0.029) in all the patients. The analysis of the study group alone revealed significant positive correlations between MMP-9 and TSH (r = 0.74 p = 0.036), BDNF and both ALT (r = 0.73 p = 0.038) and TSH (r = 0.85 p = 0.008), and a negative correlation between MMP-1 and fT4 (r = -0.75 p = 0.032). The control group did not present any significant correlations.ConclusionThe higher concentrations of BDNF and lower of MMP-2 found in girls with TS without MetS compared to healthy girls with short stature, could have a major impact on the future “natural” development of the metabolic status. Our findings need further studies.

Bioelectrical spectroscopy impedance phase angle is not associated with nutritional status in a stable cohort of paediatric inflammatory bowel disease patients.

Nutritional assessment in paediatric inflammatory bowel disease (IBD) is key to supporting growth whilst minimising adiposity. Bedside assessment using bioelectrical impedance spectroscopy (BIS) has previous identified patients with declining cellular and nutritional health. We aimed to assess BIS measures in stable paediatric IBD patient. Stable IBD patients were recruited at routine hospital visits. All patients underwent BIS, anthropometry and disease activity assessment. Multivariable regression and receiver operator curve (ROC) analyses were undertaken to assess the utility of BIS phase angle 50KHz (PA-50) and 200/5KHz impedance ratio (IR) in nutritional assessment. There were 140 study visits from 97 patients, mean age 14.49 years, 62.9% Crohn's disease. Mean BMI Z-score (BMIZ) was 0.31 (range-2.97 to 3.99), 33% of patients were overweight (BMIZ>1) and 13.8% of patients were underweight (BMIZ<-1). Crohn's disease patients had a lower mean BMIZ score 0.14, compared to ulcerative colitis, 0.68, p=0.007. There was no relationship between PA-50 and BMIZ or disease activity. IR was not related to disease activity but was negatively related to BMIZ in a multivariable regression, accounting for age, sex and disease subtype (beta-0.331, p=0.001). ROC analyses did not identify a clinically useful cut off for either PA-50 or IR to identify patients with active disease, biologic use or BMIZ>1 or<-1. BIS appears to have limited added value in nutritional assessment of stable paediatric IBD patients. Nearly 1/3 patients were overweight and personalised approach to supplementation is vital to avoid overnutrition.