Registry Of Children Research Articles

Pediatric Nephrology Workforce in the United States and Access to Waitlist Registration in Children with ESKD

Pediatric Nephrology Workforce in the United States and Access to Waitlist Registration in Children with ESKD

Threats Targeting Children on Online Social Networks

Today, practically everyone has access to the internet and owns some type of digital gadgets, such as a smartphone, laptop, tablet, etc. We live in a digital world where internet use is widespread. Nowadays, a growing number of children have access to the internet via portable electronic devices such as tablets, laptops, and gaming consoles. Online social networks (OSN) have become increasingly popular and are now widely used along with the rise of the internet. A large percentage of children worldwide have profiles on at least one online social networking site. Children register on these platforms so they can communicate with their peers, make new friends, share their interests and hobbies in the hopes of meeting someone who has similar ones, play games, and more. But in addition to the benefits and enjoyment that kids derive from social networks, there are also risks and dangers that specifically target children on OSN platforms. Online harassment, online predators, sexual solicitation, cyberbullying, and cyber grooming are all threats that target children on OSNs. This paper aims to bring awareness to parents and children about the potential dangers present on online social networking platforms and offers guidelines on how to better protect children in the social networking environment.

191 Clinical characteristics associated with functional abnormalities in pediatric and adult morphea: A cross-sectional study

Morphea is an inflammatory skin condition leading to fibrosis of the skin and subcutaneous tissue which produces cosmetic and functional impairment (FI). Few studies have systematically examined the demographic and clinical features of morphea associated with FI, making it difficult to stratify patients for screening for FI based on risk. To address this gap, we performed a multicenter cross-sectional analysis of adults and children with morphea (n=1104) enrolled in the Morphea in Adults and Children registry at UT Southwestern (n=756) or the National Registry for Childhood-Onset Scleroderma at the University of Pittsburgh (n=348).

571 Morphea in patients of color: A cross sectional study of the morphea in adults and children (MAC) cohort

Morphea is an autoimmune sclerosing skin condition. While clinical manifestations of morphea are well described in Caucasians, characteristics of morphea in patients of color have been poorly studied. Thus, the aim of this study was to determine the demographic and clinical characteristics of morphea in patients of color. This was an analysis of patients in the Morphea in Adults and Children Registry from 2007 to 2020. Kruskal-Wallis, Chi-square and Fischer’s exact tests were performed to investigate differences between racial groups. Post-hoc analysis was performed with Dunn’s test to directly compare individual racial groups. Of 772 patients in the registry, the majority had linear morphea (47%), and were white (73%). Overall, patients of color comprised 27% of the entire cohort, with 5% Black, 4% Asian, 14% Hispanic, and 4% Other. Age of morphea onset showed significant differences between racial groups (p=0.004). Black patients were more likely to have a later disease onset compared to non-Black patients and were less likely to have linear morphea (p=0.005). Asian and Hispanic patients were found to have similar subtype distribution to white patients. Years to diagnosis from disease onset also varied between racial groups (p=0.0058). Black and Hispanic morphea patients had a delayed diagnosis compared to White morphea patients [2.85 and 2.73 years in Black (p=0.015) and Hispanic (p=0.011) patients respectively vs 2.08 years in white patients]. Hispanic patients had higher disease damage by PGA-D, when compared to compared to White patients (Median: 30, IQR: 20-50 v. Median: 20, IQR: 10-40, p=<0.001). Our study demonstrates that morphea is infrequent in patients with skin of color, but still occurs in a substantial number of patients. We underscore the need for dermatologists to carefully consider distinct disease characteristics in patients of color, especially given the delay in diagnosis time experienced by Black and Hispanic patients compared to White patients.

AB012. Clinical and demographic features of morphea patients with mucocutaneous involvement: a cross sectional study from the morphea of adults and children (MAC cohort)

While many clinical findings of morphea have been described by our group and others, mucocutaneous findings, including genital or oral lesions, have not been well characterized. To date, we don’t know the frequency, demographic and clinical features associated with mucocutaneus lesions. To address this knowledge gap, we performed a cross-sectional analysis of patients enrolled in the Morphea in Adults and Children Registry from 2007 to 2018. Of the 737 patients analyzed, 48% (n=353) had linear morphea, 31% (n=232) had generalized morphea, 12% (n=87) had plaque morphea and 6% (n=45) had mixed. Oral lesions were present in 23 patients (3%), out of which 20 (87%) had linear morphea, nine had En Coup de Sabre (39%) and 12 had Parry Romberg syndrome (PRS) (52%). Genital lesions were present in 28 patients, the majority of which (24/28, 86%) had generalized morphea. Patients with oral involvement had a younger age of onset when compared to patients with genital involvement (12 and 58 years old, respectively; P<0.001). Seventy-nine percent (n=22) of morphea patients with genital involvement had lichen sclerosus et atrophicus (LsA) overlap, compared to 17% (n=4) with oral involvement (P<0.001). Eighty-three percent (n=19) patients with oral involvement were classified as having morphea profunda, or deep involvement, compared to 14% (n=4) of patients with genital involvement (P<0.001). Median mLoSSI ad PGA-A scores for patients with oral involvement (0, IQR 0–4 and 0, IQR 0–24, respectively)) was lower than patients with genital involvement (10, IQR 6–27 and 23, IQR 15–40, respectively) (P<0.001 and P=0.002, respectively). PGA-D scores were higher in patients with oral involvement (50, IQR 30–60) than in patients with genital involvement (20, IQR 10–25) (P<0.001). In conclusion, have demonstrated that mucocutaneous involvement of morphea lesions occurs most frequently in patients with linear and generalized morphea. Providers may not be aware of the prevalence of these findings and thus they may go undiagnosed for many months or years, leading to the development of potentially permanent and devastating sequalae.

543 Clinical and demographic features of morphea patients with mucocutaneous involvement: A cross sectional study from The Morphea of Adults and Children (MAC Cohort)

While clinical findings of morphea have been described by our group and others, genital and oral lesions have not been well characterized. To address this knowledge gap, a cross-sectional analysis was performed of patients enrolled in The Morphea in Adults and Children Registry from 2007 to 2018. Of 737 patients analyzed, 48% (n=353) had linear morphea, 31% (n=232) had generalized morphea, 12% (n=87) had plaque morphea, and 6% (n=45) had mixed. Oral lesions were present in 23 patients (3%), of which 20 (87%) had linear morphea, nine had En Coup de Sabre (39%) and 12 had Parry Romberg Syndrome (PRS) (52%). Genital lesions were present in 28 patients, the majority of which (86%) had generalized morphea. Patients with oral morphea involvement had a younger age of onset compared to genital involvement (12 and 58 years old, respectively; p < 0.001). Genital morphea patients had extra-genital lichen sclerosus et atrophicus (LsA) in 79% (n=22) as compared to 17% (n= 4) with oral involvement (p < 0.001). Morphea profunda, or deep involvement, was seen in 83% (n = 19) patients with oral involvement versus 14% (n=4) of patients with genital involvement (p < 0.001). Median mLoSSI and PGA-A scores for patients with oral involvement (0, IQR 0-4 and 0, IQR 0-24, respectively) was lower than patients with genital involvement (10, IQR 6-27 and 23, IQR 15-40, respectively) (p < 0.001 and p = 0.002, respectively). PGA-D scores were higher in patients with oral involvement (50, IQR 30-60) than in patients with genital involvement (20, IQR 10-25) (p < 0.001). Our results show that while mucocutaneous lesions are rare in morphea, oral involvement is predominant in patients with facial linear lesions, particularly PRS, while genital lesions predominate in post-menopausal women with overlying extra-genital LsA. Given the function limiting nature of these lesions, practitioners should know clinical signs that put patients at risk mucocutaneous morphea.

Information-analytical assessment systems for perinatal outcomes and children’s health status born by assisted reproductive technologies

Purpose: To integrate clinical databases based on the information and analytical systems of medical organizations (MO) and to evaluate outcomes and health of children born by assisted reproductive technologies (ART).Research methods: To form database by integrating information and analytical systems of the medical organizations. To search and unify the data by means of freeware relational database management system (DBMS) – MySQL.Results. A prototype application for the management and support of a unified clinical database to analyze and evaluate the outcomes of ART. To compare information from individual databases of MO one need to match fields in the form of unique personal records, field validation, processing of missing data. Significant (p &lt;0.001) differences in gestational age and anthropometric data in the databases of medical organizations (76% of full-term children from the Automated System “Regional obstetric monitoring” (AS ROM) database compared with 18% according to the IS “Register of Children requiring Early Care”) were determined by the introduction of the conception of ART, along with the presence of perinatal risk factors in children. The created software can be used to create registers of medical organizations, as well as to support clinical decision-making in forecasting, modeling outcomes and children’s health after ART, developing personalized treatment and rehabilitation programs.Conclusion. It is necessary to develop and implement information systems, to create ART registers on the basis of regional and national registers, to analyze the outcomes of the use of reproduction methods.

Prospective Multicenter Nationwide Registry of Children With Eosinophilic Esophagitis

Prospective Multicenter Nationwide Registry of Children With Eosinophilic Esophagitis

How Would Children Register Their Own Births? Insights from a Survey of Students Regarding Birth Registration Knowledge and Policy Suggestions in Kenya.

Birth registration and obtaining physical birth certificates impose major challenges in developing countries, with impact on child and community health, education, planning, and all levels of development. However despite initiatives, universal registration is elusive, leading to calls for new approaches to understanding the decisions of parents. In this paper, we report results of a survey of students in grades six to eight (age ~12–16) in an under-registered area of Kenya regarding their own understanding of registration issues and their suggestions for improvement. These students were selected because they themselves were also nearing the age for high school enrollment/entrance examinations, which specifically requires possession of a birth certificate. This assessment was also a companion to our previous representative survey of adults in the same Kenyan region, allowing for parent-child comparison. Results supported previous research, showing that only 43% had birth certificates. At the same time, despite these low totals, students were themselves quite aware of registration factors and purposes. The students also made quite prescient sources for understanding their households’ motivations, with many of their suggestions—for focus on communication of pragmatic benefits, or automatic measures shifting responsibility from parents—mirroring our own previous suggestions, and showing a level of pragmatism not witnessed when surveying their parents. This paper therefore adds evidence to the discussion of registration policy planning. More generally, it also builds on an important trend regarding the treatment of children as stakeholders and important sources of information, and raising an intriguing new avenue for future research.

Sentia: A Spanish Systematic Online Monitoring Registry for Children And Adolescents Treated With Antipsychotics: Results from A 2-Year, Naturalistic Follow-Up Study

Sentia: A Spanish Systematic Online Monitoring Registry for Children And Adolescents Treated With Antipsychotics: Results from A 2-Year, Naturalistic Follow-Up Study

Bone health of patients with juvenile idiopathic arthritis: a comparison between dual-energy X-ray absorptiometry and digital X-ray radiogrammetry

ObjectivesJuvenile idiopathic arthritis (JIA) affects bone mineral density (BMD). Dual-energy X-ray absorptiometry (DXA) is the most widely used technique to determine BMD. Digital X-ray radiogrammetry (DXR) is a feasible method for determination of cortical BMD on hand radiographs. This study aimed to compare DXR and DXA in the assessment of BMD in JIA patients. MethodsThirty-five JIA patients with available DXA and hand radiograph within the same time period were included from the Dutch Arthritis and Biologicals in Children register. Outcome measures for BMD were Bone Health Index from DXR and BMD total body, BMD lumbar spine and Bone Mineral Apparent Density from DXA. All measures were transformed to Z-scores. Correlations were assessed with Pearson correlation coefficients. ResultsMedian age of the patients (60% female) was 11.7 years. Pearson correlation coefficient was significant for the absolute scores: 0.568–0.770 (p<0.001). No significant correlation was found between the Z-scores of DXA and DXR. ConclusionsThe BMD assessment from the DXR was correlated to DXA measures in a cohort of JIA patients, although only in absolute scores. Future steps for implementation of DXR in clinical practice include evaluation of responsiveness to change, predictive value and comparison with other imaging techniques.

Long-term quality of life and functional outcome of patients with juvenile idiopathic arthritis in the biologic era: a longitudinal follow-up study in the Dutch Arthritis and Biologicals in Children Register.

To carry out a longitudinal investigation of functional outcome, health-related quality of life (HRQoL) and treatment strategies in JIA patients who started etanercept >5 years ago. We approached patients whose HRQoL changes were described previously in a subanalysis of the Dutch Arthritis and Biologicals in Children register. Recent disease status, co-morbidities and structural damage were retrieved. Disability and HRQoL were assessed by (Childhood) HAQ [(C)HAQ], Child Health Questionnaire, Short Form 36 and Health Utilities Index Mark 3. Changes over time were analysed with linear mixed models. Forty-three patients (81% response) started etanercept a median 8.5 years ago. At the time of this long-term analysis, median age was 22 years (interquartile range: 18-24 years). HRQoL outcome was similar to HRQoL 15-27 months after the initiation of etanercept; 42% had a (C)HAQ of 0.00 and 67% had achieved inactive disease. Patients reported increasing levels of bodily pain compared with earlier measurements. Unemployment (12%) was comparable to the general population; educational level was higher. Use of biologic agents was as follows: 40% etanercept; 40% other biologic agents; and 20% none. Joint surgery occurred in 14% of patients. At a median 8.5 years after the commencement of etanercept treatment, JIA patients maintain most of the acquired improvement in HRQoL. Although disability and disease activity are low, chronic pain remains an issue. Persistence and possible deterioration of radiological damage emphasize the importance of early treatment. The fact that 20% of patients do not use any anti-rheumatic medication shows that clinical remission of medication might be an achievable goal.

An international national emergency airway registry for children: Comparison of tracheal intubation practices across international PICUs

An international national emergency airway registry for children: Comparisonof tracheal intubationpractices across international PICUs Jan hau Lee, Gabrielle Nuthall, Takanari Ikeyama, Osamu Saito, Yee hui Mok, Vinay M. Nadkarni, Akira Nishisaki KK Women's and Children's Hospital, Singapore Starship Children's Hospital, New Zealand Aichi Children's Health and Medical Center, Japan Tokyo Metropolitan Children's Medical Center, Japan The Children's Hospital of Philadelphia, USA Background/Purpose: Tracheal intubation (TI) is a common, lifesaving intervention in pediatric intensive care units (PICUs). There is heterogeneity in approaches to TI across PICUs. This variation may be more marked across different geographical regions and may lead to difference in safety outcomes. We hypothesized that there would be differences in the process of care and safety outcomes for TI across PICUs in 4 different geographical regions (Japan, New Zealand, Singapore, and North America). Methods: The multicenter TI safety database National Emergency Airway Registry for Children (NEAR4KIDS) expanded to include international PICUs since 2012. Currently, there are 3 international PICUs outside of North America. We analyzed data from the NEAR4KIDS database from 3 PICUs (1 in Japan, 1 in New Zealand, and 1 in Singapore) from January 2013 to September 2014 and compared against North America (25 ICUs). We included all initial TI courses performed in PICUs. Outcome measures included adverse tracheal intubation associated events (TIAEs) and severe TIAEs (eg, cardiac arrest, aspiration, esophageal intubation not immediately recognized). Univariate analysis was performed with χ test and Kruskal-Wallis test, with P b .05 taken as statistical difference. Results: We identified a total of 515 TIs across the 3 international PICUs. Median age of patients (years) was 0 (IQR, 0-2) in Japan, 0 (0-1) in New Zealand, and 1 (0-5) in Singapore, significantly different (P = .005). The most common TI indication was respiratory failure (74% in Japan, 62% in New Zealand, 68% in Singapore, P = .17), similar to TIs from North America (62%). Direct laryngoscopy was the most common TI method in both international and North America (96% vs 85%, P b .001). International PICUs used uncuffed tracheal tube more often (62% vs 7%, P b .001). Among the reported TIs, tube change was more common in international sites (36% vs 12%, P b .001). The occurrence of adverse TIAE was 20% in Japan, 14% in New Zealand, and 10% in Singapore, not different from North America (international 15% vs North America 16%, P= .64). The occurrence of severe TIAEswas 11% in Japan, 6% in New Zealand, and 2% in Singapore, not different fromNorth America (international 7% vs North America 6%, P = .73). Conclusions: NEAR4KIDS TI registry successfully captured similarities and differences in TI practice and outcomes across international PICUs. International sites used uncuffed tracheal tube more often and had higher proportionof tracheal tube change. Theoccurrenceof TIAEand severeTIAEs were similar between international and North American sites.

Long term functional outcome and quality of life of patients with refractory juvenile idiopathic arthritis treated with etanercept: results of the dutch arthritis and biologicals in children register

Juvenile idiopathic arthritis patients refractory to methotrexate are eligible for treatment with biologic agents. A longitudinal sub-analysis (n=53) of the Dutch Arthritis and Biologicals in Children register previously showed that disability and health related quality of life (HRQoL) improved shortly after treatment with etanercept.

Automated determination of bone age and bone mineral density in patients with juvenile idiopathic arthritis: a feasibility study.

IntroductionChronic inflammation combined with glucocorticoid treatment and immobilization puts juvenile idiopathic arthritis (JIA) patients at risk of impaired growth and reduced bone mineral density (BMD). Conventional methods for evaluating bone age and BMD are time-consuming or come with additional costs and radiation exposure. In addition, an automated measurement of bone age and BMD is likely to be more consistent than visual evaluation. In this study, we aimed to evaluate the feasibility of an automated method for determination of bone age and (cortical) bone mineral density (cBMD) in severely affected JIA patients. A secondary objective was to describe bone age and cBMD in this specific JIA population eligible for biologic treatment.MethodsIn total, 69 patients with standard hand radiographs at the start of etanercept treatment and of calendar age within the reliability ranges (2.5 to 17 years for boys and 2 to 15 years for girls) were extracted from the Dutch Arthritis and Biologicals in Children register. Radiographs were analyzed using the BoneXpert method, thus automatically determining bone age and cBMD expressed as bone health index (BHI). Agreement between measurements of the left- and right-hand radiographs and a repeated measurement of the left hand were assessed with the intraclass correlation coefficient (ICC). Regression analysis was used to identify variables associated with Z-scores of bone age and BHI.ResultsThe BoneXpert method was reliable in the evaluation of radiographs of 67 patients (radiographs of 2 patients were rejected because of poor image quality). Agreement between left- and right-hand radiographs (ICC = 0.838 to 0.996) and repeated measurements (ICC = 0.999 to 1.000) was good. Mean Z-scores of bone age (−0.36, P = 0.051) and BHI (−0.85, P < 0.001) were lower compared to the healthy population. Glucocorticoid use was associated with delayed bone age (0.79 standard deviation (SD), P = 0.028), and male gender was associated with a lower Z-score of BHI (0.65 SD, P = 0.021).ConclusionsBoneXpert is an easy-to-use method for assessing bone age and cBMD in patients with JIA, provided that radiographs are of reasonable quality and patients’ bone age lies within the age ranges of the program. The population investigated had delayed bone maturation and lower cBMD than healthy children.Electronic supplementary materialThe online version of this article (doi:10.1186/s13075-014-0424-1) contains supplementary material, which is available to authorized users.

AB0886 Bone Health Assessment of Patients with Juvenile Idiopathic Arthritis: A Comparison between DXA and Bonexpert

BackgroundChronic inflammatory activity in juvenile idiopathic arthritis (JIA) affects the bone, possibly resulting in growth abnormalities and decreased bone mineral density (BMD). Monitoring the bone status of JIA patients is...

Trends in prescription of biological agents and outcomes of juvenile idiopathic arthritis: results of the Dutch national Arthritis and Biologics in Children Register

BackgroundTreatment of juvenile idiopathic arthritis (JIA) has changed dramatically since the introduction of biological agents in 1999.ObjectiveTo evaluate trends in prescription patterns of biological agents and the subsequent outcome of...

Treatment choices of paediatric rheumatologists for juvenile idiopathic arthritis: etanercept or adalimumab?

To evaluate differences in baseline characteristics between etanercept- and adalimumab-treated JIA patients and to reveal factors that influence the choice between these TNF inhibitors, which are considered equally effective in the recent ACR recommendations for JIA treatment. Biologic-naïve JIA patients with active arthritis who started treatment with adalimumab or etanercept between March 2008 and December 2011 were selected from the Dutch Arthritis and Biologicals in Children register. Baseline characteristics were compared. Focus group interviews with paediatric rheumatologists were performed to evaluate factors determining treatment choices. A total of 193 patients started treatment with etanercept and 21 with adalimumab. Adalimumab-treated patients had longer disease duration prior to the start of biologics (median 5.7 vs 2.0 years) and more often a history of uveitis (71% vs 4%). Etanercept-treated patients had more disability at baseline (median Childhood Health Assessment Questionnaire score 1.1 vs 0.4) and more active arthritis (median number of active joints 6 vs 4). The presence of uveitis was the most important factor directing the choice towards adalimumab. Factors specific for the paediatric population-such as painful adalimumab injections-as well as the physician's familiarity with the drug accounted for the preference for etanercept. Although the two TNF inhibitors are considered equally effective, in daily practice etanercept is most often prescribed; adalimumab is mainly preferred when uveitis is present. In choosing the most suitable biologic treatment, paediatric rheumatologists take into account drug and patient factors, considering newly published data and cautiously implementing this into daily care.

AB1165 Tumour necrosis factor-blocking agents in persistent oligoarticular juvenile idiopathic arthritis: Results from the dutch arthritis and biologicals in children register

BackgroundCurrently, biological agents are not approved for the treatment of persistent oligoarticular juvenile idiopathic arthritis (oJIA). However, there are persistent oJIA patients unresponsive to the conventional treatment consisting of NSAIDs,...

OP0297 Trends in Prescription of Biologics and Outcomes of Juvenile Idiopathic Arthritis; Results of the Dutch National Arthritis and Biologicals in Children Register

BackgroundTreatment of juvenile idiopathic arthritis (JIA) has changed dramatically since the introduction of biologics in 1999. Because of more insight in the immunological and biological pathways involved in the development...