41 PROMs in autosomal inherited bleeding disorders: A systematic literature review 2 Author, (year), country Study design and characteristics of population (n; gender; disease type; age) PROMs Measured PROs PROs and conclusions* Govorov, I., et al. (2016). Sweden 41 Prospective, single center 30 patients with VWD (all female) • Type 1: 15 • Type 2: 11 • Type 3: 4 Age (years; SD) • mean: 35.1 (8.1) • range: 19-51 SF-36 • HRQoL • Impact of menstruation on overall life activities • Menstruation affects patients on mood (90.9%), family life (72.7%), vacations (68.2%), and the ability to go to work (45.5%). • Patients have similar levels of HRQoL compared to normative data (p>0.10). • Patients with VWD type 1, 2 and 3 have similar scores of HRQoL (p>0.10). • Patients with HMB have more bodily pain compared to normative data (p<0.10). Haghpanah, S., et al. (2017). Iran 42 Prospective, single center 52 patients with rare factor deficiencies (46% female) • FI def.: 9 • FV def.: 2 • FVII def.: 15 • FX def.: 7 • FXI def.: 8 • FXIII def.: 8 • FV & FVIII def.: 3 Age (years; SD) • mean: 13.96 (4.50) • range: 4-18 Haemo-QOL • HRQoL • Global health status • More than half of patients (63%) have a good or higher global health status. • Women have similar HRQoL compared to men (p=0.122). • Bleeding severity (p=0.032), health status (p=0.011), and being bothered by disease (p<0.001) are negatively associated with HRQoL.
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