Aerobic Capacity in Children with Hemophilia

Engelbert, Raoul H.H.; Plantinga, Martine; Van der Net, Janjaap; Van Genderen, Frank R.; Van den Berg, Marijke H.; Helders, Paul J.M.; Takken, Tim

doi:10.1016/j.jpeds.2007.12.016

« Previous Next »The Journal of Pediatrics
Volume 152, Issue 6 , Pages 833-838.e1, June 2008

Aerobic Capacity in Children with Hemophilia

Raoul H.H. Engelbert, PT, PCS, PhD
- Pediatric Physiotherapy and Exercise Physiology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, the Netherlands
Martine Plantinga, MSc
- Pediatric Physiotherapy and Exercise Physiology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, the Netherlands
Janjaap Van der Net, PT, PCS, PhD
- Pediatric Physiotherapy and Exercise Physiology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, the Netherlands
Frank R. Van Genderen, MSc, PhD
- Pediatric Physiotherapy and Exercise Physiology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, the Netherlands
- Van Creveldkliniek/Department of Haematology, University Medical Center Utrecht, Utrecht, the Netherlands.
Marijke H. Van den Berg, MD, PhD
- Van Creveldkliniek/Department of Haematology, University Medical Center Utrecht, Utrecht, the Netherlands.
Paul J.M. Helders, PT, PCS, MSc, PhD
- Pediatric Physiotherapy and Exercise Physiology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, the Netherlands
Tim Takken, MSc, PhD
- Pediatric Physiotherapy and Exercise Physiology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, the Netherlands
- Reprint requests: Tim Takken, MSc, PhD, Department of Pediatric Physical Therapy and Exercise Physiology, University Hospital for Children and Youth “Wilhelmina Kinderziekenhuis,” University Medical Center Utrecht, Room KB.02.056, PO Box 85090, 3508 AB Utrecht, the Netherlands.

Received 13 July 2007; received in revised form 2 October 2007; accepted 5 December 2007. published online 25 February 2008.

Objective

To determine whether aerobic capacity is normal in boys with different types of hemophilia compared with healthy peers and whether the level of aerobic capacity correlates with the amount of physical activity, joint health status, muscle strength, and anthropometrics.

Study design

47 patients (mean [SD] age, 12.9 [3.2] years; age range, 8.2-17.4 years) from the “Van Creveldkliniek” of the University Medical Center Utrecht, participated. Anthropometry, muscle strength, joint impairment, functional ability, and aerobic capacity were measured. The amount of energy expenditure during daily living was assessed.

Results

All boys were able to perform at maximal or near-maximal level on exercise tests, and none of them reported bleeds or other adverse events. Relative peak oxygen, peak heart rate, and peak working capaicty were significantly lower compared with healthy control subjects. 30% had Z-scores >2 for weight. Total muscle strength was normal, and almost no joint impairment and no decrease in functional ability were found.

Conclusion

The aerobic capacity of children with hemophilia is still lower than the normal population, whereas their overall muscle strength is comparable with healthy peers. The functional ability does not differ from healthy peers, and joint health status showed very minor impairments. A substantial proportion of Dutch children with hemophilia was overweight, without showing a reduction in the amount of self-reported physical activities.

Abbreviations: Σ4SF, Sum of 4 skin-fold measurements, BMI, Body mass index, HJHS, Hemophilia Joint Health Score, HR, Heart rate, MAQ, Modifiable Activity Questionnaire, MET, Metabolic equivalent score, MET-h/week, Average MET/ hour per week, RCHAQ₃₈, Revised Childhood Health Assessment Questionnaire, RER, Respiratory exchange ratio, VO_2peak, Peak oxygen consumption, VO_2peak/kg, Relative VO_2peak, W_max, Maximal working capacity