The Problem of Unrecognized "Mild Hemophilia"

Pappas, Arthur M.; Barr, Joseph S.; Salzman, Edwin W.; Britten, Anthony; Riseborough, Edward J.

doi:10.1001/jama.1964.03060230100031

Cited by 13 publications

(1 citation statement)

References 8 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Mild hemophilia is defined by factor levels between 5% and 40%. While individuals with the moderate (1–5%) and severe (<1%) forms of hemophilia generally have a higher risk of spontaneous and life‐threatening bleeds, the bleeding associated with mild hemophilia A is usually reported as episodic, occurring during surgery or following trauma [1–3]. Mild hemophilia may also be characterized by joint and muscle hemorrhage, leading over time to chronic pain and hemoarthropathy with eventual joint replacement surgeries [4].…”

Section: Introductionmentioning

confidence: 99%

Health‐related quality of life in a cohort of adult patients with mild hemophilia A

Walsh

Macgregor

Stuckless

et al. 2008

Journal of Thrombosis and Haemostasis

View full text Add to dashboard Cite

Summary. Objectives: To compare the health-related quality of life among adult males affected with mild hemophilia A due to the same mutation (Val2016ala) to that of unaffected age and sex matched controls from the same general population. Methods: The Short-Form 36 (SF-36) and Health Assessment Questionnaire (HAQ) were used to measure health-related quality of life and physical function. Other measures included bleeding history, a measure of joint damage, body mass index, age, and viral infection status. Cross-sectional data were collected through research clinics and a retrospective chart audit over a two-year period. Results and Conclusions: The study included 47 affected males and 33 controls. The affected males had a higher level of co-morbidity, prior bleeding, and existing joint damage than controls. With the exception of the social function and health transition scales, mean scores for each of the SF-36 domains were worse among affected males. Mean differences were more than a clinically important five points in five of eight domains, with the general health scale showing more than a 10-point difference. Despite the degree of difference noted, only two of the differences were statistically significant (general health and role emotional scales) because of the small sample size and considerable individual variation in SF-36 scale scores. Multiple regression analyses suggested existing joint damage and presence of heart disease as the strongest associates of lower physical healthrelated quality of life. Joint damage in turn was partly related to prior hemarthroses. Compared to the Canadian population, affected males had lower scores in six out of eight SF-36 domains as well as the physical component summary score. There were no significant differences found in the HAQ scores between the two groups. So-called mild hemophilia A was associated with a negative effect on physical health-related quality of life, contributed to by joint damage as a result of prior bleeding.

show abstract

Section: Introductionmentioning

confidence: 99%