Clotting factor activity levels and bleeding risk in people with haemophilia playing sports.

Background: Improved treatment options for people with haemophilia (PWH) have increased the possibilities for sports participation, but the risk of sports-induced bleeding (SIB) is still considered considerable by many.

Aim: To assess sports associated injury- and bleeding risk in PWH and to assess clotting levels associated with safe sports participation.

Methods: Sports injuries and SIBs were prospectively collected for 12 months in PWH aged 6-49 without inhibitors playing sports at least once weekly.

Can motor proficiency testing predict sports injuries and sports-induced bleeds in people with haemophilia?

Introduction: Predicting the risk of sports injuries and sports-induced bleeds (SIBs) in people with haemophilia (PWH) may support clinical counselling.

Aim: To assess the association between motor proficiency testing and sports injuries and SIBs and to identify a specific set of tests for predicting injury risk in PWH.

Methods: In a single centre, prospective study male PWH aged 6-49 playing sports ≥1x/week were tested for running speed and agility, balance, strength and endurance.

Validation of the pedHAL and HAL in Dutch children and adults with haemophilia.

Introduction: The Haemophilia Activities List (HAL) and paediatric HAL assess self-reported limitations in various daily activities. To reduce patient burden, shorter versions of the pedHAL (22 items) and HAL (18 items) have been developed.

Aim: This study aimed to determine the agreement between the pedHAL/HAL and pedHAL/HAL and construct validity and internal consistency of the pedHAL HAL in persons with haemophilia (PWH).

Longitudinal Motor-Developmental Outcomes in Infants with a Critical Congenital Heart Defect.

Infants with critical congenital heart defects (CCHDs) are at increased risk for neurodevelopmental delays. The early identification of motor delays is clinically relevant to prevent or reduce long-term consequences. The current study aims to describe the motor-developmental pathways of infants with a CCHD.

Bimanual performance in children with unilateral perinatal arterial ischaemic stroke or periventricular haemorrhagic infarction.

Background: Long term outcome data on bimanual performance in children with perinatal arterial ischaemic stroke (PAIS) and periventricular haemorrhagic infarction (PVHI) with and without unilateral spastic cerebral palsy (USCP) is sparse.

Aims: To assess bimanual performance in children with PAIS or PVHI with and without USCP and to explore the relationship with unilateral hand function and full-scale IQ (FSIQ) in a cross-sectional study.

Methods: Fifty-two children with PAIS (n = 27) or PVHI (n = 25) participated at a median age of 12 years and 1 month (range 6-20 years).

Motor Developmental Delay After Cardiac Surgery in Children With a Critical Congenital Heart Defect: A Systematic Literature Review and Meta-analysis.

Purpose: To systematically review evidence regarding the severity and prevalence of motor development in children with a critical congenital heart defect (CCHD) without underlying genetic anomalies.

Summary Of Key Points: Twelve percent of all included studies reported abnormal mean motor developmental scores, and 38% reported below average motor scores. Children with single-ventricle physiology, especially those with hypoplastic left heart syndrome, had the highest severity and prevalence of motor delay, particularly at 0 to 12 months.

Shortening the Haemophilia Activities List (HAL) from 42 items to 18 items.

Introduction: The Haemophilia Activities List (HAL) was developed to measure activities and participation in persons with haemophilia (PWH). Shortening the questionnaire may facilitate use of the HAL.

Aim: The aim of this study was to determine which items of the HAL are redundant, to construct a shorter version of the HAL, and to determine the construct validity of the HAL .

Early motor outcomes in infants with critical congenital heart disease are related to neonatal brain development and brain injury.

Aim: To assess the relationship between neonatal brain development and injury with early motor outcomes in infants with critical congenital heart disease (CCHD).

Method: Neonatal brain magnetic resonance imaging was performed after open-heart surgery with cardiopulmonary bypass. Cortical grey matter (CGM), unmyelinated white matter, and cerebellar volumes, as well as white matter motor tract fractional anisotropy and mean diffusivity were assessed.

Similar sports participation as the general population in Dutch persons with haemophilia; results from a nationwide study.

Introduction: Although sports participation is advocated in people with haemophilia (PWH), detailed data concerning sports participation in Dutch PWH is lacking.

Aim: to assess sports participation in Dutch PWH (6-65 years) compared to the Dutch general population (GP).

Methods: Data from a nationwide, cross-sectional study in PWH were analysed.

Shortening the paediatric Haemophilia Activities List (pedHAL) based on pooled data from international studies.

Introduction: The paediatric Haemophilia Activities List (pedHAL) was developed to measure activities and participation in children and youth with haemophilia. Results from international studies provide an opportunity to determine which items are universally important.

Aim: The aim of this study was to determine which items of the pedHAL are redundant to construct a shorter version of the pedHAL.

Assessing the test-retest reliability and smallest detectable change of the Haemophilia Activities List.

Introduction: The Haemophilia Activities List (HAL) is a preferred instrument to measure self-reported limitations in activities in persons with haemophilia (PWH). Information on reliability and interpretability of HAL scores is lacking.

Aim: To examine the test-retest reliability and smallest detectable change (SDC) of the HAL in adult PWH.

Parents' experiences of VOICE: A novel support programme in the NICU.

Background: Admission of an infant to a neonatal intensive care unit (NICU) is often a stressful experience for parents and can be associated with feelings of inadequacy to fulfil the desirable parental role. The values, opportunities, integration, control, and evaluation (VOICE) programme was developed to engage parents in care, to decrease stress, and to increase empowerment.

Aim: To explore the experiences of parents regarding involvement in the VOICE programme during admission of their infant to the NICU.

Evaluating international Haemophilia Joint Health Score (HJHS) results combined with expert opinion: Options for a shorter HJHS.

Introduction: The Hemophilia Joint Health Score (HJHS) was developed to detect early changes in joint health in children and adolescents with haemophilia. The HJHS is considered by some to be too time consuming for clinical use and this may limit broad adoption.

Aim: This study was a first step to develop a shorter and/or more convenient version of the HJHS for the measurement of joint function in children and young adults with haemophilia, by combining real-life data and expert opinion.

Sports participation and sports injuries in Dutch boys with haemophilia.

Introduction: Sports participation in children with hemophilia is generally considered to be associated with increased injury risk, which is generally considered highest in severe hemophilia.

Aim: To assess sports participation according to age and severity in children with hemophilia and its association with sports injuries.

Methods: In a retrospective single-center study, sports participation, injuries, and bleeding data from three consecutive annual clinic visits were collected for young patients with hemophilia (PWH, aged 6-18).

The Paediatric Haemophilia Activities List (pedHAL) in routine assessment: changes over time, child-parent agreement and informative domains.

Introduction: The Paediatric Haemophilia Activities List (pedHAL) assesses self-reported limitations in activities and participation in children with haemophilia.

Aim: To assess longitudinal changes, child-parent agreement and to identify which pedHAL domains yielded most information in boys with access to early prophylaxis.

Methods: The pedHAL (53 items, 7 domains, optimum 100) was completed annually at the Van Creveldkliniek by boys aged 4-18 years with moderate/severe haemophilia and their parents.

Healthy play, better coping: The importance of play for the development of children in health and disease.

Play is of vital importance for the healthy development of children. From a developmental perspective, play offers ample physical, emotional, cognitive, and social benefits. It allows children and adolescents to develop motor skills, experiment with their (social) behavioural repertoire, simulate alternative scenarios, and address the various positive and negative consequences of their behaviour in a safe and engaging context.

Dutch juvenile idiopathic arthritis patients, carers and clinicians create a research agenda together following the James Lind Alliance method: a study protocol.

Background: Research on Juvenile Idiopathic Arthritis (JIA) should support patients, caregivers/parents (carers) and clinicians to make important decisions in the consulting room and eventually to improve the lives of patients with JIA. Thus far these end-users of JIA-research have rarely been involved in the prioritisation of future research.

Main Body: Dutch organisations of patients, carers and clinicians will collaboratively develop a research agenda for JIA, following the James Lind Alliance (JLA) methodology.

2016 ACR-EULAR adult dermatomyositis and polymyositis and juvenile dermatomyositis response criteria-methodological aspects.

Objective: The objective was to describe the methodology used to develop new response criteria for adult DM/PM and JDM.

Methods: Patient profiles from prospective natural history data and clinical trials were rated by myositis specialists to develop consensus gold-standard ratings of minimal, moderate and major improvement. Experts completed a survey regarding clinically meaningful improvement in the core set measures (CSM) and a conjoint-analysis survey (using 1000Minds software) to derive relative weights of CSM and candidate definitions.

The effect of an integrated perceived competence and motor intervention in children with developmental coordination disorder.

Background And Aims: Children with DCD have lower self-perceptions and are less physically active than typically developing children. The aim of this quasi-experimental study was to investigate whether an integrated perceived competence and motor intervention affects DCD children's motor performance, self-perceptions, and physical activity compared with a motor intervention only.

Methods And Procedures: The intervention group consisted of 20 children and the care-as-usual group consisted of 11 children, all aged 7-10 years.

Consensus-based recommendations for the management of juvenile dermatomyositis.

Background: In 2012, a European initiative called Single Hub and Access point for pediatric Rheumatology in Europe (SHARE) was launched to optimise and disseminate diagnostic and management regimens in Europe for children and young adults with rheumatic diseases. Juvenile dermatomyositis (JDM) is a rare disease within the group of paediatric rheumatic diseases (PRDs) and can lead to significant morbidity. Evidence-based guidelines are sparse and management is mostly based on physicians' experience.

The change in perceived motor competence and motor task values during elementary school: A longitudinal cohort study.

Participation in motor activities is essential for social interaction and life satisfaction in children. Self-perceptions and task values have a central position in why children do or do not participate in (motor) activities. Investigating developmental changes in motor self-perceptions and motor task values in elementary school children would provide vital information about their participation in motor activities.

Proposal for a Candidate Core Set of Fitness and Strength Tests for Patients with Childhood or Adult Idiopathic Inflammatory Myopathies.

Objective: Currently there are no evidence-based recommendations regarding fitness and strength tests for patients with childhood or adult idiopathic inflammatory myopathies (IIM). This hinders clinicians and researchers in choosing the appropriate fitness- or muscle strength-related outcome measures for these patients. Through a Delphi survey, we aimed to identify a candidate core set of fitness and strength tests for children and adults with IIM.