Clin Pediatr Hematol Oncol.  2018 Oct;25(2):108-115. 10.15264/cpho.2018.25.2.108.

Joint Health Status in Hemophilia Patients Using Hemophilia Joint Health Score and Pettersson Score

Affiliations
  • 1Department of Pediatric Hematology-Oncology, Yonsei Cancer Center, Yonsei University Health System, Seoul, Korea. jwhan@yuhs.ac
  • 2Department of Pediatrics, Yonsei University College of Medicine, Yonsei University Health System, Seoul, Korea.
  • 3Department of Nursing, Yonsei Cancer Center, Yonsei University Health System, Seoul, Korea.

Abstract

BACKGROUND
Comprehensive clinical and radiologic follow-up is needed to preserve joint functions and quality of life in hemophilia using clinimetric tools such as Hemophilia joint health score (HJHS) or Pettersson score (PS). We investigated the joint health status evaluated using the tools in Korean hemophilia patients.
METHODS
We reviewed retrospectively medical records to collect clinical parameters, HJHS and PS, who were followed up in Severance Hospital, Seoul, Korea. The correlation between HJHS and PS, and the effect of the prophylaxis for hemophilia on the outcomes measured with the scores were evaluated. The prophylaxis proportion (PP) was calculated as the proportion of prophylaxis duration to each patient's life time.
RESULTS
Total of 28 patients with severe hemophilia were enrolled. Twelve patients (42.8%) were less than 20 years old. Total of 23 patients had experienced prophylaxis during their lives, and median PP was 39.7%. There was significant correlation between HJHS and PS (P < 0.001). Each score was positively correlated with patient's age (P < 0.001). PP was negatively correlated with either HJHS or PS (P < 0.001, respectively). There was significant correlation between either HJHS or PS and the PP in the group of patients < 20 years old, but there was no correlation in the group of >20 years old.
CONCLUSION
HJHS and PS were positively correlated. Each score increased as the patient's age increased. The prophylaxis had protective effect on joint health. The prospective evaluation of HJHS and PS will be needed to prove the effect of proper management on the joint health status.

Keyword

Hemophilia; Arthropathies; Level of health; Patient outcome assessment

MeSH Terms

Follow-Up Studies
Health Status
Hemophilia A*
Humans
Joints*
Korea
Medical Records
Patient Outcome Assessment
Prospective Studies
Quality of Life
Retrospective Studies
Seoul
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