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Monitoring of somatic development through the assessment of anthropometric variables such as weight, height, and BMI is vital for evaluating the physical development and nutritional status of children. This approach aids in the early identification of somatic developmental disorders, enabling timely medical interventions. It traditionally relies on Z-scores, which compare anthropometric variables with reference standards. In addition to somatic development monitoring, the early detection and management of pediatric and adolescent hypertension are crucial due to potential long-term health risks. However, manual calculations of Z-scores are time-consuming and error-prone, impeding timely interventions for at-risk children. This article introduces an innovative open-code solution for real-time Z-score assessments directly within the electronic data capture platform, Research Electronic Data Capture, (REDCap™), aiming to streamline the monitoring of somatic development in children. Leveraging the World Health Organization (WHO) growth standards and National Health and Nutrition Examination Survey (NHANES) references, our approach integrates Z-score computations directly into REDCap, providing a secure and user-friendly environment for healthcare professionals and researchers. We employed Bland-Altman analyses to compare our method with established calculators (Knirps and Growth XP™) using synthetic data values for all variables. Bland-Altman plots demonstrated strong agreement between our REDCap calculations and the Knirps and Growth XP systems. Z-scores for height, BMI, and blood pressure consistently aligned, affirming the accuracy of our approach across the measurement range. The integration with REDCap streamlines data collection and analysis, eliminating the need for separate software and data exports. Moreover, our solution uses the World Health Organization (WHO) growth standards and National Health and Nutrition Examination Survey (NHANES) references. This not only ensures calculation accuracy but also enhances its suitability for diverse research contexts. The Bland-Altman analyses establish the reliability of our method, contributing to a more effective approach to child growth and blood pressure monitoring.

Initially designed to identify children's movement impairments in clinical settings, the Movement Assessment Battery for Children-2 (MABC-2) is also widely used to evaluate children's movement in research. Standardised scores on the test are calculated using parametric methods under the assumption of normally-distributed data. In a pilot study with thirty five 8-10 year old children (i.e., in Age Band 2 of the MABC-2), we found that maximal performance was often reached. These 'ceiling effects' created distributions of scores that may violate parametric assumptions. Tests of normality, skew, and goodness-of-fit revealed this violation, most clearly on three of the eight sub-tests. A strong deviation from normality was again observed in a sample of 161 children (8-10 years, Experiment 1), however ceiling effects were reduced by modifying the scoring methods, and administering items designed for older children when maximal performance was reached. Experiment 2 (n = 81, 7-10 years) further refined the administration and scoring methods, and again improved the distributions of scores. Despite reducing ceiling effects, scores remained non-parametrically distributed, justifying non-parametric analytic approaches. By randomly and repeatedly resampling from the raw data, we generated non-parametric reference distributions for assigning percentiles to each child's performance, and compared the results with the standardised scores. Distributions of scores obtained with both parametric and non-parametric methods were skewed, and the methods resulted in different rankings of the same data. Overall, we demonstrate that some MABC-2 item scores are not normally-distributed, and violate parametric assumptions. Changes in administering and scoring may partially address these issues. We propose that resampling or other non-parametric methods are required to create new reference distributions to which an individual child's performance can be referred. The modifications we propose are preliminary, but the implication is that a new standardisation is required to deal with the non-parametric data acquired with the MABC-2 performance test.

Activity-dependent neuroprotective protein (ADNP) is one of the lead genes in autism spectrum disorder/intellectual disability. Heterozygous, de novo ADNP mutations cause the ADNP syndrome. Here, to evaluate natural history of the syndrome, mothers of two ADNP syndrome boys aged 6 and a half and two adults aged 27 years (man and woman) were subjected to Vineland III questionnaire assessing adaptive behavior. The boys were assessed again about 2 years after the first measurements. The skill measures, presented as standard scores (SS) included domains of communication, daily living, socialization, motor skills and a sum of adaptive behavior composite. The age equivalent (AE) and growth scale values (GSV) encompassing 11 subdomains assess the age level at which the subject’s raw score is found at a norm sample median and the individual temporal progression, respectively. The norm referenced standard scores age-matched, mean 100 ± 15 of the two children showed the lowest outcome in communication (SS: 20–30). Daily living skills presented SS of 50–60, with a possible potential loss of some activities as the child ages, especially in interpersonal relationships with people outside of the immediate family (boy A). In contrast, in socialization, both children were at the SS of 38, with some positive increase to SS of ~ 45 (interpersonal relations with family members and coping skills, depending on the particular individual), 2 years later. Interestingly, there was an apparent large difference in motor skills (gross and fine) at the young age, with subject B showing a relatively higher level of skills (SS: 70), decreasing to subject A level (SS: 40) 2 years later. Together, the adaptive behavior composite suggested a level of SS: 39–48 with B showing a potential increase (SS: 41–44) and A, a substantial decrease (SS: 48–39), suggesting a strong impact of daily living skills. Adults were at SS: 20, which is the lowest possible score. AE showed minor improvements for subject A and B, with all AE values being below 3 years. GSVs for subject A showed some improvement with age, especially in interpersonal, play and leisure, and gross motor subdomains. GSV for subject B showed minor improvements in the various subdomains. Notably, all subjects showed a percentile rank < 1 compared with age-matched norms except for subject B as to motor domain (2nd percentile) at the age of 6 years. In summary, the results, especially comparing SS and AEs between childhood and adulthood, implied a continuous deterioration of activities compared to the general population, encompassing a slower developmental process coupled to possible neurodegeneration, strongly supporting a great need for disease modifying medicinal procedures.

Optimal discretization of continuously valued attributes is an uncertainty problem. The uncertainty of discretization is propagated and accumulated in the process of data mining, which has a direct influence on the usability and operation of the output results for mining. To address the limitations of existing discretization evaluation indices in describing accuracy and operation efficiency, this work suggests a discretization uncertainty index based on individuals. This method takes the local standard score as the general similarity measure in and between the intervals and evaluates discretization reliability according to the relative position of individuals in each interval. The experiment shows the new evaluation index is consistent with commonly used metrics. Under the premise of guaranteeing the validity of discrete evaluation, the proposed method has greater description accuracy and operation efficiency than extant approaches; it also has more advantages for massive data processing and special distribution detection.

In this paper, we propose an efficient algorithm based on the anisotropic diffusion model to detect defect in a low-contrast surface image, especially aimed at anti-reflective (AR) glass. The proposed algorithm has two important procedures: (1) a modified anisotropic diffusion model and (2) morphological directivity filter. The modified diffusion model based on an adaptive edge threshold, the standard score ( Z -score), is proposed to quickly and efficiently enhance the low-contrast defects. It acts as the adaptive enhancement process. The pixels with both the low gradient and the high Z -score or the high gradient and the low Z -score will generate a high diffusion coefficient. It enhances the gray levels of suspected defective edges and also preserves the original gray levels of an internal area of suspected defects, as well as generates the slight smoothing process for the noisy background. A simple and efficient method can easily segment the defects, followed by the effective morphological directivity filter, which removes noise from the thresholding image. The proposed algorithm is evaluated by a set of 23 low-contrast surface images of AR glass in this study. The experimental results show that the proposed algorithm is superior to the four competitive approaches. The defect detection results demonstrate that the proposed algorithm can segment the complete defects. In addition, the computational advantage compared to the Perona and Malik model, C-T model 1, C-T model 2, and histogram statistics model are about 4.48, 2.26, 19.53, and 26.63 times, respectively. It can be concluded that the proposed algorithm not only provides reliable inspection results but also improves the inspection efficiency over 2 times.

BackgroundMaintenance of physical fitness and optimum bone health is crucial to meet the training demands, decreased risk for injury and to achieve optimum sports performance.ObjectiveTo investigate the status of bone Mineral Density (BMD), serum vitamin D, serum calcium and injury history and association of BMD with speed, agility and injury history in National level Kho-Kho players. DesignThe participants were recruited through a purposive sampling technique. SettingThe study was conducted on male Indian Kho- Kho players undergoing training in a national camp.ParticipantsThe study included fifty-two healthy male Indian Kho- Kho players aged 16–31 years. The inclusion criteria were general good health and a willingness to participate in the study. The exclusion criteria included recent history of acute or chronic debilitating illness, injury, smoking, alcoholism, medication and food allergies. AssessmentBMD, serum calcium and vitamin-D3, 30m sprint and Illinois tests and Injury history. Main Outcome MeasurementsBMD scores: T Scores and Z scores were considered as independent variables. ResultsT-Score showed lower values in 22.5% (MeanSD: 0.32  0.96; Range: -2 to +3) of players as compared to the standard cut-offs given by WHO (-1SD & above). Serum Vitamin-D3 was lower (<20ng/ml), in 13.7% and insufficient (>20≤30ng/ml) in 54.9% of players. Ankle (23.1%), knee (15.4%) and shoulder injuries (11.5%) were higher as compared to other injuries. No significant correlation between BMD markers (T-Score, Z-Score), serum vitamin-D3 and calcium was observed. T-Score (r=0.295, p<0.05) and Z-score (r=0.321, p<0.05) were significantly positively correlated with speed, but not with agility. An association was found between Z-Score (Std.:1.38, CI:0.34 – 2.44, p<0.01) and T-Score (Std-:1.49, CI: 0.37–2.62, p<0.01) and speed. No significant association was found between injury history and BMD.ConclusionsLow BMD and vitamin-D3 deficiency were prevalent among Kho-Kho players. Speed performance has an association with BMD.

Background Vosoritide increases annualized growth velocity (AGV) in children with achondroplasia aged 5 to 18 years. This global, phase 2, randomized, double-blind, placebo-controlled study evaluated the safety and efficacy of vosoritide on growth in children with achondroplasia aged 3 months to <5 years. Methods This study compared once-daily subcutaneous administration of vosoritide, at doses of 15 or 30 μg/kg of body weight, with placebo. Eligible patients had participated, for up to 6 months, in an observational growth study to calculate their baseline AGV. The primary objective was to evaluate the safety and tolerability of vosoritide in children with achondroplasia. The primary efficacy evaluation was the change from baseline in height Z-score versus placebo at week 52 using an ANCOVA model. Secondary efficacy analyses included change from baseline in AGV and upper-to-lower body segment ratio versus placebo at Week 52 using an ANCOVA model. Results A total of 75 patients were enrolled, with 11 sentinel subjects who received vosoritide to establish PK and safety. A further 32 were randomized to receive vosoritide and 32 to receive placebo. A total of 73 patients completed the 52-week trial. All patients reported at least one adverse event. Four serious adverse events occurred with vosoritide and 8 with placebo, none were treatment-related. Two participants discontinued, one on vosoritide with pre-existing respiratory morbidity who had a fatal respiratory arrest and one on placebo who withdrew consent. In the full analysis population, vosoritide (n=43) compared to placebo (n=32), increased height Z-score by 0.30 SD (95% CI 0. 07, 0.54); increased AGV by 0.92cm/year (95% CI 0.24, 1.59); and did not worsen upper-to-lower body segment ratio which changed by -0. 06 (95% CI -0.15, 0. 03). Conclusions Daily, subcutaneous administration of vosoritide to young children with achondroplasia was safe and resulted in increases in height Z-score and AGV. (Funded by BioMarin; ClinicalTrials.gov NCT03583697) Presentation: Monday, June 13, 2022 12:30 p.m. - 2:30 p.m.

Abstract Disclosure: F. Giusti: None. F. Marini: None. B. Weiss: Employee; Self; Amolyt. M. Ovize: Employee; Self; Amolyt. S. Benvenga: None. F. Cetani: None. A. Colao: None. S. Corbetta: Consulting Fee; Self; Italfarmaco, Abiogen, Gedeon Richter. E.C. Degli Uberti: None. M. Iacobone: None. A. Lenzi: None. G. Mantovani: None. R. Ruggeri: None. M. Brandi: Consulting Fee; Self; Alexion Pharmaceuticals, Inc., Personal Genomics, Echolight, Amolyt, Aboca, Bruno Farmaceutici, Kyowa Kirin, UCB. Speaker; Self; Abiogen, Alexion Pharmaceuticals, Inc., Monte Rosa, Amorphical, Amgen Inc, Italfarmaco, Bruno Farmaceutici, Eli Lilly & Company, Kyowa Kirin, EchoLight, Amolyt, Takeda, UCB, Menarini, Enterabio, Gedeon Richter, Theramex. The HypoparaNet database includes 509 patients with chronic hypoparathyroidism (cHP) who have been followed in 20 centers in Italy from 2014 onwards. This population is composed of 110 male and 399 female patients. cHP etiology included post-surgical n=363 (71.3%), idiopathic n=78 (15.3%) and genetic n=64 (12.6%) cases.Bone health is an important consideration in this population of patients whose skeleton is exposed both to the cHP-induced alteration of bone microarchitecture and to the progression of osteopenia/osteoporosis related to ageing, specifically in post-menopausal (or over 50 years) women.In the present cohort, dual x-ray osteodensitometry (DXA) was performed in n=173 cHP patients. We specifically assessed the prevalence of osteopenia/osteoporosis in relation to menopause (or age below or above 50 years) in female patients. Osteoporosis was defined as the presence of at least one of the measured bone sites presenting a T-score (or a Z-score for individuals < 50 years) < -2.5. Osteopenia was defined as the presence of at least one of the measured bone sites presenting a T-score (or a Z-score for individuals < 50 years) < -1.0, and all the measured bone site > -2.5. Female patients n=45 at or below 50 years (F≤50) and female patients n=97 above 50 years (F>50) had comparable mean values of albumin-adjusted serum calcium, phosphate, magnesium, PTH, 25(OH) vitamin D and 1,25(OH)2 vitamin D. The prevalence of osteopenia was high and comparable in the F≤50 and F>50 subgroups, averaging 38% and 34%, respectively. The prevalence of osteoporosis was clearly higher in the F>50 group as compared to the F≤50 group, averaging 4% and 24% respectively.These real-world data indicate that nearly half of the female patients, who represent the majority of the hypoparathyroid population, exhibit a high prevalence of osteopenia or osteoporosis that increases in relation to age. In this context, new treatment modalities (including PTH replacement therapy) for this patient population should aim at restoring a balanced bone turnover without further bone loss in order to ensure bone safety. Presentation: 6/2/2024