Model correction of diagnostic coding-based RSV incidence for children 0-4 years in the US.

BACKGROUND: Although administrative claims data have a high degree of completeness, not all medically attended Respiratory Syncytial Virus-associated lower respiratory tract infections (RSV-LRTIs) are tested or coded for their causative agent. We sought to determine the attribution of RSV to LRTI in claims data via modeling of temporal changes in LRTI rates against surveillance data. METHODS: We estimated the weekly incidence of LRTI (inpatient, outpatient, and total) for children 0-4 years using 2011-2019 commercial insurance claims, stratified by HHS region, matched to the corresponding weekly NREVSS RSV and influenza positivity data for each region, and modelled against RSV, influenza positivity rates, and harmonic functions of time assuming negative binomial distribution. LRTI events attributable to RSV were estimated as predicted events from the full model minus predicted events with RSV positivity rate set to 0. RESULTS: Approximately 42% of predicted RSV cases were coded in claims data. Across all regions, the percentage of LRTI attributable to RSV were 15-43%, 10-31%, and 10-31% of inpatient, outpatient, and combined settings, respectively. However, when compared to coded inpatient RSV-LRTI, 9 of 10 regions had improbable corrected inpatient LRTI estimates (predicted RSV/coded RSV ratio < 1). Sensitivity analysis based on separate models for PCR and antigen-based positivity showed similar results. CONCLUSIONS: Underestimation based on coding in claims data may be addressed by NREVSS-based adjustment of claims-based RSV incidence. However, where setting-specific positivity rates is unavailable, we recommend modeling across settings to mirror NREVSS's positivity rates which are similarly aggregated, to avoid inaccurate adjustments.

Quinolone Ear Drops and Achilles Tendon Rupture.

BACKGROUND: Delayed eardrum healing has been observed in the ear opposite to the ear treated with otic quinolones (OQ) in rats. Case reports describe tendinopathies after OQ treatment, suggesting adverse systemic effects. METHODS: We studied patients aged 19 to 64 years with diagnosis of otitis externa or media in private insurance between 2005 and 2015. We compared OQ treatment against otic neomycin, oral amoxicillin, or azithromycin. Outcomes included Achilles tendon rupture (ATR), Achilles tendinitis (AT), and all-type tendon rupture (ATTR). We applied an active comparator, new-user design with 1-year look-back and ceased follow-up at initiation of systemic steroids or oral quinolones, external injury, hospitalization, and after 35 days. We used trimmed stabilized inverse probability of treatment weights to balance comparison groups in a survival framework. Negative outcomes (clavicle fractures or sports injuries) were examined to rule out differences from varied physical activity (unmeasured confounding). RESULTS: We examined 1 501 009 treated otitis episodes. Hazard ratios (HR) for OQ exposure associated with ATR were 4.49 (95% confidence interval [CI], 1.83-11.02), AT 1.04 (95% CI, 0.73-1.50), and ATTR 1.71 (95% CI, 1.21-2.41). Weighted risk differences (RD) per 100 000 episodes for OQ exposure were ATR 7.80 (95% CI, 0.72-14.89), AT 1.01 (95% CI, -12.80 to 14.81), and ATTR 18.57 (95% CI, 3.60-33.53). Corresponding HRs for clavicle fractures and sports injuries were HR,1.71 (95% CI, 0.55-5.27) and HR,1.45 (95% CI, 0.64-3.30), suggesting limited residual confounding. CONCLUSIONS: OQ exposure may lead to systemic consequences. Clinicians should consider this potential risk and counsel patients accordingly. Risk factors and mechanisms for this rare, adverse effect deserve further evaluation. Mechanistic and other clinical studies are warranted to corroborate this finding.

Performance Analysis of Multihop Full-Duplex NOMA Systems with Imperfect Interference Cancellation and Near-Field Path-Loss.

Outage probability (OP) and potential throughput (PT) of multihop full-duplex (FD) nonorthogonal multiple access (NOMA) systems are addressed in the present paper. More precisely, two metrics are derived in the closed-form expressions under the impact of both imperfect successive interference cancellation (SIC) and imperfect self-interference cancellation. Moreover, to model short transmission distance from the transmit and receive antennae at relays, the near-field path-loss is taken into consideration. Additionally, the impact of the total transmit power on the performance of these metrics is rigorously derived. Furthermore, the mathematical framework of the baseline systems is provided too. Computer-based simulations via the Monte Carlo method are given to verify the accuracy of the proposed framework, confirm our findings, and highlight the benefits of the proposed systems compared with the baseline one.

RSV testing practice and positivity by patient demographics in the United States: integrated analyses of MarketScan and NREVSS databases.

BACKGROUND: RSV-incidence estimates obtained from routinely-collected healthcare data (e.g., MarketScan) are commonly adjusted for under-reporting using test positivity reported in national Surveillance Systems (NREVSS). However, NREVSS lacks detail on patient-level characteristics and the validity of applying a single positivity estimate across diverse patient groups is uncertain. We aimed to describe testing practices and test positivity across subgroups of private health insurance enrollees in the US and illustrate the possible magnitude of misclassification when using NREVSS to correct for RSV under ascertainment. METHODS: Using billing records, we determined distributions of RSV-test claims and test positivity among a national sample of private insurance enrollees. Tests were considered positive if they coincided with an RSV-diagnosis. We illustrated the influence of positivity variation across sub-populations when accounting for untested acute respiratory infections. RESULTS: Most tests were for children (age 0-4: 65.8%) and outpatient encounters (78.3%). Test positivity varied across age (0-4: 19.8%, 5-17: 1.8%, adults: 0.7%), regions (7.6-16.1%), settings (inpatient 4.7%, outpatient 14.2%), and test indication (5.0-35.9%). When compared to age, setting or indication-specific positivity, bias due to using NREVSS positivity to correct for untested ARIs ranged from - 76% to 3556%. CONCLUSIONS: RSV-test positivity depends on the characteristics of patients for whom those tests were ordered. NREVSS-based correction for RSV-under-ascertainment underestimates the true incidence among children and overestimate rates among adults. Demographic-specific detail on testing practice and positivity can improve the accuracy of RSV-incidence estimates.

Assessment and mitigation of toddlers' personal exposure to black carbon before and during the COVID-19 pandemic: A case study in Singapore.

Black carbon (BC), an important indicator of traffic-related air pollution (TRAP) in urban environments, is receiving increased attention because of its adverse health effects. Personal exposure (PE) of adults to BC has been widely studied, but little is known about the exposure of young children (toddlers) to BC in cities. We carried out a pilot study to investigate the integrated daily PE of toddlers to BC in a city-state with a high population density (Singapore). We studied the impact of urban traffic on the PE of toddlers to BC by comparing and contrasting on-road traffic flow (i.e., volume and composition) in Singapore in 2019 (before the COVID-19 pandemic) and in 2020 (during the COVID-19 pandemic). Our observations indicate that the daily BC exposure levels and inhaled doses increased by about 25% in 2020 (2.9 ± 0.3 µg m-3 and 35.5 µg day-1) compared to that in 2019 (2.3 ± 0.4 µg m-3 and 28.5 µg day-1 for exposure concentration and inhaled dose, respectively). The increased BC levels were associated with the increased traffic volume on both weekdays and weekends in 2020 compared to the same time period in 2019. Specifically, we observed an increase in the number of trucks as well as cars/taxis and motorcycles (private transport) and a decline in the number of buses (public transport) in 2020. The implementation of lockdown measures in 2020 resulted in significant changes in the time, place and duration of PE of toddlers to BC. The recorded daily time-activity patterns indicated that toddlers spent almost all the time in indoor environments during the measurement period in 2020. When we compared different ventilation options (natural ventilation (NV), air conditioning (AC), and portable air cleaner (PAC)) for mitigation of PE to BC in the home environment, we found a significant decrease (>30%) in daily BC exposure levels while using the PAC compared to the NV scenario. Our case study shows that the PE of toddlers to BC is of health concern in indoor environments in 2020 because of the migration of the increased TRAP into naturally ventilated residential homes and more time spent indoors than outdoors. Since toddlers' immune system is weak, technological intervention is necessary to protect their health against inhalation exposure to air pollutants.

Air quality changes in cities during the COVID-19 lockdown: A critical review.

In response to the rapid spread of coronavirus disease-2019 (COVID-19) within and across countries and the need to protect public health, governments worldwide introduced unprecedented measures such as restricted road and air travel and reduced human mobility in 2020. The curtailment of personal travel and economic activity provided a unique opportunity for researchers to assess the interplay between anthropogenic emissions of primary air pollutants, their physical transport, chemical transformation, ultimate fate and potential health impacts. In general, reductions in the atmospheric levels of outdoor air pollutants such as particulate matter (PM), nitrogen dioxide (NO2), carbon monoxide (CO), sulfur dioxide (SO2), and volatile organic compounds (VOCs) were observed in many countries during the lockdowns. However, the levels of ozone (O3), a secondary air pollutant linked to asthma and respiratory ailments, and secondary PM were frequently reported to remain unchanged or even increase. An increase in O3 can enhance the formation of secondary PM2.5, especially secondary organic aerosols, through the atmospheric oxidation of VOCs. Given that the gaseous precursors of O3 (VOCs and NOx) are also involved in the formation of secondary PM2.5, an integrated control strategy should focus on reducing the emission of the common precursors for the co-mitigation of PM2.5 and O3 with an emphasis on their complex photochemical interactions. Compared to outdoor air quality, comprehensive investigations of indoor air quality (IAQ) are relatively sparse. People spend more than 80% of their time indoors with exposure to air pollutants of both outdoor and indoor origins. Consequently, an integrated assessment of exposure to air pollutants in both outdoor and indoor microenvironments is needed for effective urban air quality management and for mitigation of health risk. To provide further insights into air quality, we do a critical review of scientific articles, published from January 2020 to December 2020 across the globe. Finally, we discuss policy implications of our review in the context of global air quality improvement.

Factors affecting quality of life in children and adolescents with hypermobile Ehlers-Danlos syndrome/hypermobility spectrum disorders.

Hypermobile Ehlers-Danlos syndrome (hEDS) is a hereditary disorder of connective tissue, often presenting with complex symptoms can include chronic pain, fatigue, and dysautonomia. Factors influencing functional disability in the pediatric hEDS population are incompletely studied. This study's aims were to assess factors that affect quality of life in children and adolescents with hEDS. Individuals with hEDS between the ages 12-20 years and matched parents were recruited through retrospective chart review at two genetics clinics. Participants completed a questionnaire that included the Pediatric Quality of Life Inventory (PedsQL™), PedsQL Multidimentional Fatigue Scale, Functional Disability Inventory, Pain-Frequency-Severity-Duration Scale, the Brief Illness Perception Questionnaire, measures of anxiety and depression, and helpful interventions. Survey responses were completed for 47 children and adolescents with hEDS/hypermobility spectrum disorder (81% female, mean age 16 years), some by the affected individual, some by their parent, and some by both. Clinical data derived from chart review were compared statistically to survey responses. All outcomes correlated moderately to strongly with each other. Using multiple regression, general fatigue and pain scores were the best predictors of the PedsQL total score. Additionally, presence of any psychiatric diagnosis was correlated with a lower PedsQL score. Current management guidelines recommend early intervention to prevent disability from deconditioning; these results may help identify target interventions in this vulnerable population.

Secrecy Performance of TAS/SC-Based Multi-Hop Harvest-to-Transmit Cognitive WSNs Under Joint Constraint of Interference and Hardware Imperfection.

In this paper, we evaluate the secrecy performance of multi-hop cognitive wireless sensor networks (WSNs). In the secondary network, a source transmits its data to a destination via the multi-hop relaying model using the transmit antenna selection (TAS)/selection combining (SC) technique at each hop, in the presence of an eavesdropper who wants to receive the data illegally. The secondary transmitters, including the source and intermediate relays, have to harvest energy from radio-frequency signals of a power beacon for transmitting the source data. Moreover, their transmit power must be adjusted to satisfy the quality of service (QoS) of the primary network. Under the joint impact of hardware imperfection and interference constraint, expressions for the transmit power for the secondary transmitters are derived. We also derive exact and asymptotic expressions of secrecy outage probability (SOP) and probability of non-zero secrecy capacity (PNSC) for the proposed protocol over Rayleigh fading channel. The derivations are then verified by Monte Carlo simulations.

Rateless Codes-Based Secure Communication Employing Transmit Antenna Selection and Harvest-To-Jam under Joint Effect of Interference and Hardware Impairments.

In this paper, we propose a rateless codes-based communication protocol to provide security for wireless systems. In the proposed protocol, a source uses the transmit antenna selection (TAS) technique to transmit Fountain-encoded packets to a destination in presence of an eavesdropper. Moreover, a cooperative jammer node harvests energy from radio frequency (RF) signals of the source and the interference sources to generate jamming noises on the eavesdropper. The data transmission terminates as soon as the destination can receive a sufficient number of the encoded packets for decoding the original data of the source. To obtain secure communication, the destination must receive sufficient encoded packets before the eavesdropper. The combination of the TAS and harvest-to-jam techniques obtains the security and efficient energy via reducing the number of the data transmission, increasing the quality of the data channel, decreasing the quality of the eavesdropping channel, and supporting the energy for the jammer. The main contribution of this paper is to derive exact closed-form expressions of outage probability (OP), probability of successful and secure communication (SS), intercept probability (IP) and average number of time slots used by the source over Rayleigh fading channel under the joint impact of co-channel interference and hardware impairments. Then, Monte Carlo simulations are presented to verify the theoretical results.

Pain and sleep quality in children with non-vascular Ehlers-Danlos syndromes.

The objective of this study was to explore the factors contributing to quality of life in pediatric patients with non-vascular Ehlers-Danlos syndromes (EDS). Data were analyzed on 41 children with a diagnosis of non-vascular EDS from the de-identified data available from the National Institute on Aging (NIA) study of heritable disorders of connective tissue. Children under age 19 years were seen as part of a long-term evaluation project from 2003 to 2013 on a larger natural history of patients with heritable disorders of connective tissue. Data collected included medical history, physical examination findings, diagnostic study results, and responses on validated questionnaires. We reviewed a sub-cohort of children with a diagnosis of non-vascular EDS and explored pain severity and interference via the Brief Pain Inventory, and sleep quality via the Pittsburgh Sleep Quality Index. Pain severity had a strong correlation with pain interference, and both were similar to other disorders that include chronic pain reported in the literature. Sleep quality did not correlate with pain severity or interference, but all patients had poor sleep quality in comparison to historical controls. We conclude that pain and sleep are significant issues in the pediatric non-vascular EDS population, and future research may be directed toward these issues.

Energy Harvesting over Rician Fading Channel: A Performance Analysis for Half-Duplex Bidirectional Sensor Networks under Hardware Impairments.

In this paper, a rigorous analysis of the performance of time-switching energy harvesting strategy that is applied for a half-duplex bidirectional wireless sensor network with intermediate relay over a Rician fading channel is presented to provide the exact-form expressions of the outage probability, achievable throughput and the symbol-error-rate (SER) of the system under the hardware impairment condition. Using the proposed probabilistic models for wireless channels between mobile nodes as well as for the hardware noises, we derive the outage probability of the system, and then the throughput and SER can be obtained as a result. Both exact analysis and asymptotic analysis at high signal-power-to-noise-ratio regime are provided. Monte Carlo simulation is also conducted to verify the analysis. This work confirms the effectiveness of energy harvesting applied in wireless sensor networks over a Rician fading channel, and can provide an insightful understanding about the effect of various parameters on the system performance.

Hybrid TSR⁻PSR Alternate Energy Harvesting Relay Network over Rician Fading Channels: Outage Probability and SER Analysis.

In this research, we investigate a hybrid time-switching relay (TSR)⁻power-splitting relay (PSR) alternate energy harvesting (EH) relaying network over the Rician fading channels. For this purpose, the amplify-and-forward (AF) mode is considered for the alternative hybrid time TSR⁻PSR. The system model consists of one source, one destination and two alternative relays for signal transmission from the source to the destination. In the first step, the exact and asymptotic expressions of the outage probability and the symbol errors ratio (SER) are derived. Then, the influence of all system parameters on the system performance is investigated, and the Monte Carlo simulation verifies all results. Finally, the system performances of TSR⁻PSR, TSR, and PSR cases are compared in connection with all system parameters.

α-Glucosidase Inhibitory Xanthones from the Roots of Garcinia fusca.

Two new compounds, fuscaxanthones J (1) and K (2), together with eight known xanthones (3 - 10) were isolated from an ethyl acetate extract of the roots of Garcinia fusca. Their structures were determined using spectroscopic methods, mainly 1D- and 2D-NMR. α-Glucosidase inhibitory activity of the isolated compounds was evaluated and fuscaxanthone J (1) showed the most significant effect with an IC50 value of 8.3 ± 1.8 µm (compared with acarbose, IC50 = 214.5 ± 2.3 µm).

Respiratory syncytial virus reinfections among infants and young children in the United States, 2011-2019.

BACKGROUND: Although respiratory syncytial virus (RSV) immunoprophylaxis is recommended for high-risk infants, the American Academy of Pediatrics (AAP) recommends against immunoprophylaxis in the same season following a breakthrough hospitalization due to limited risk for a second hospitalization. Evidence in support of this recommendation is limited. We estimated population-based re-infection rates from 2011-2019 in children <5 years since RSV risk remains relatively high in this age group. MATERIALS AND METHODS: Using claims data from private insurance enrollees, we established cohorts of children <5 years who were followed to ascertain annual (July 1-June 30) and seasonal (November 1- February 28/29) RSV recurrence estimates. Unique RSV episodes included inpatient encounters with RSV diagnosis ≥30 days apart, and outpatient encounters ≥30 days apart from each other as well as from inpatient encounters. The risk of annual and seasonal re-infection was calculated as the proportion of children with a subsequent RSV episode in the same RSV year/season. RESULTS: Over the 8 assessed seasons/years (N = 6,705,979) and across all age groups annual inpatient and outpatient infection rates were 0.14% and 1.29%, respectively. Among children with a first infection, annual inpatient and outpatient re-infection rates were 0.25% (95% confidence interval (CI) = 0.22-0.28) and 3.44% (95% CI = 3.33-3.56), respectively. Both infection and re-infection rates declined with age. CONCLUSION: While medically-attended re-infections contributed numerically only a fraction of the total RSV infections, re-infections among those with previous infection in the same season were of similar magnitude as the general infection risk, suggesting that a previous infection may not attenuate the risk for a re-infection.

Proceedings of the 2023 Cannabis Clinical Outcomes Research Conference.

The Consortium for Medical Marijuana Clinical Outcomes Research, a multi-university collaboration established by the state of Florida in the USA, hosted its third annual Cannabis Clinical Outcomes Research Conference (CCORC) in May 2023. CCORC was held as a hybrid conference, with a scientific program consisting of in-person sessions, with some sessions livestreamed to virtual attendees. CCORC facilitated and promoted up-to-date research on the clinical effects of medical cannabis, fostering collaboration and active involvement among scientists, policymakers, industry professionals, clinicians, and other stakeholders. Three themes emerged from conference sessions and speaker presentations: (1) disentangling conflicting evidence for the effects of medical cannabis on public health, (2) seeking solutions to address barriers faced when conducting clinical cannabis research - especially with medical cannabis use in special populations such as those who are pregnant, and (3) unpacking the data behind cannabis use and mental health outcomes. The fourth annual CCORC is planned for the summer of 2024 in Florida, USA.

Polyisoprenylated benzophenones and xanthones from the pericarp of Garcinia planchonii Pierre.

Two new polyisoprenylated benzophenones, planchoniones A (1) and B (2), together with two known benzophenones (3, 4) and six known xanthones (5-10), were isolated from an ethyl acetate extract of the pericarp of Garcinia planchonii Pierre. Their structures were established using spectroscopic methods, mainly 1D and 2D NMR. The four benzophenones were evaluated for their cytotoxicity against MCF-7 human breast cancer cells, and showed almost no activity. Meanwhile, compounds 5-10 were investigated for their inhibitory effects towards α-glucosidase, and γ-mangostin (5) exhibited the most remarkable effect with IC50 value of 15.3 ± 0.9 µM (compared with acarbose, IC50 = 224.9 ± 3.6 µM).

Two new antidiabetic xanthones from the twigs of Garcinia oblongifolia.

Two new xanthones, oblongixanthones I (1) and J (2), and seven known compounds (3-9), were isolated from an EtOAc extract of the twigs of Garcinia oblongifolia. Their structures were elucidated using spectroscopic methods, mainly 1 D and 2 D NMR. The antidiabetic effects of the two new compounds were evaluated using α-glucosidase and PTP1B inhibition assays. Both compounds displayed strong inhibition towards α-glucosidase with IC50 values of 258.7 ± 49.3 and 187.1 ± 27.5 µM, respectively (compared with acarbose, IC50 = 900.0 ± 3.0 µM) and moderate effects against PTP1B with IC50 values of 93.9 ± 12.3 and 64.1 ± 5.8 µM, respectively (compared with RK682, IC50 = 4.4 ± 0.3 l µM).

An Umbrella Review of the Cost Effectiveness of Human Papillomavirus Vaccines.

BACKGROUND AND OBJECTIVES: Although many systematic reviews for the human papillomavirus vaccines cost effectiveness have been published, they vary in perspectives, methods, and quality. We aimed to condense systematically such evidence to facilitate locating, processing, and learning, not only about the consensus of findings but also how models were built and their evolution over time and across settings. METHODS: We conducted an umbrella review of cost-effectiveness studies for human papillomavirus vaccines using three databases (PubMed, Embase, and Cochrane). Based on their objectives, we classified studies into three groups (human papillomavirus vaccines cost effectiveness, model characteristics, and all-type vaccines, including human papillomavirus vaccines). We used the AMTAR2 to assess the quality of the studies. Additionally, we provided a summary of study findings, discussions, and evidence gaps in the literature. RESULTS: Though most studies were critically low quality and had a low quality of reporting, the human papillomavirus vaccine was consistently cost effective in young girls and men who have sex with men. Stratified analyses by rated quality did not change the results. The quality assessment of the reviews did not necessarily reflect the quality assessment of underlying studies. The human papillomavirus vaccine models became more complex over time, capturing more realistic disease transmission with different human papillomavirus strains and herd immunities. CONCLUSIONS: Additional evidence is needed for vulnerable populations (e.g., childhood cancer survivors) who are at high risk for human papillomavirus vaccine-related cancers and, therefore, may be more cost effective when receiving human papillomavirus vaccines. Quantifying human papillomavirus vaccine cost effectiveness via meta-analyses is feasible if investigators can increase the homogeneity of their populations.

Disease Severity and Risk Factors of 30-Day Hospital Readmission in Pediatric Hospitalizations for Pneumonia.

Pneumonia is the leading cause of hospitalization in pediatric patients. Disease severity greatly influences pneumonia progression and adverse health outcomes such as hospital readmission. Hospital readmissions have become a measure of healthcare quality to reduce excess expenditures. The aim of this study was to examine 30-day all-cause readmission rates and evaluate the association between pneumonia severity and readmission among pediatric pneumonia hospitalizations. Using 2018 Nationwide Readmissions Database (NRD), we conducted a cross-sectional study of pediatric hospitalizations for pneumonia. Pneumonia severity was defined by the presence of respiratory failure, sepsis, mechanical ventilation, dependence on long-term supplemental oxygen, and/or respiratory intubation. Outcomes of interest were 30-day all-cause readmission, length of stay, and cost. The rate of 30-day readmission for the total sample was 5.9%, 4.7% for non-severe pneumonia, and 8.7% for severe pneumonia (p < 0.01). Among those who were readmitted, hospitalizations for severe pneumonia had a longer length of stay (6.5 vs. 5.4 days, p < 0.01) and higher daily cost (USD 3246 vs. USD 2679, p < 0.01) than admissions for non-severe pneumonia. Factors associated with 30-day readmission were pneumonia severity, immunosuppressive conditions, length of stay, and hospital case volume. To reduce potentially preventable readmissions, clinical interventions to improve the disease course and hospital system interventions are necessary.

Agreement between parent-proxy and child self-report in pediatric hypermobile Ehlers-Danlos syndrome.

Hypermobile Ehlers-Danlos syndrome (hEDS) is a common disorder in children and adolescents that negatively impacts health-related quality of life (HRQOL). It can include chronic pain, fatigue, autonomic dysfunction, and mood problems. The objective of this study was to examine levels of agreement between children and parents in the setting of hEDS and HRQOL. Individuals with hEDS, ages 10-20 years, and their parents were recruited to complete a series of surveys. Instruments included pediatric quality of life generic and multidimensional fatigue scales, Functional Disability Index, Pain-Frequency-Severity-Duration scale, Brief Illness Perception Questionnaire, and Herth Hope Index. Agreement on each measure was evaluated using statistical calculations. Thirty-six parent-child dyads completed the surveys. There were no significant differences between the means of parent and child scores. There was moderate to strong agreement on all survey scores. However, the proportion of dyads with disagreement was relatively high for each individual score. Eighteen dyads disagreed on at least half of the surveys. Body mass index centile and child perception of cognitive fatigue most strongly predicted disagreement in total HRQOL score. Proxy-reporters for children and adolescents with hEDS may agree with their child on average. However, due to significant frequency of clinically important disagreement, information from both children and their parents should be sought whenever possible.