BACKGROUND: To estimate health-related quality-of-life changes in @entity1 with @entity1659 due to @entity19459 ( @entity38524 ) deficiency who received subcutaneous @entity38524 with recombinant hyaluronidase (rHuPH20) for attack prophylaxis in a randomized, double-blind, dose-ranging, cross-over study. METHODS: @entity1 with type I/II @entity1659 received 1000 U of C1- @entity19459 with 24,000 U of rHuPH20 or 2000 U of C1- @entity19459 with 48,000 U of rHuPH20 every 3-4 days for 8 weeks and then crossed over for another 8-week period. The study was terminated early as a precaution related to non-neutralizing antibodies to rHuPH20. The @entity113 Quality of Life questionnaire (AE-QoL) was administered at weeks 1 and 5 of both periods, and at 1 week after the second treatment period. Changes in AE-QoL scores were calculated over both treatment periods and within each treatment period for @entity1 with greater then or equal to 4 weeks of treatment. RESULTS: Forty-one @entity1 had evaluable AE-QoL data, and 22 @entity1 completed treatment. At screening, 43% of the @entity1 were receiving intravenous @entity38524 . A significant average AE-QoL total score decline (improvement) of -8.1 (95% confidence interval, -13.7 to -2.5) was observed from baseline to the end of the study, and significant AE-QoL score declines were observed in the Functioning, Fear/Shame, and Nutrition domains. @entity1 on 2000 U reported higher mean AE-QoL score declines in Functioning and Nutrition domains relative to the 1000 U dose. Overall, 43.9% of all the @entity1 , 45.5% of the study completers, and 46.7% of the nonprophylaxis users at baseline on high treatment doses achieved a reduction in the AE-QoL total score of six points. CONCLUSION: Despite early termination and prestudy prophylactic intravenous @entity38524 use by 43% of the @entity1 , improved AE-QoL scores were observed after less than or equal to 16 weeks of subcutaneous @entity38524 -rHuPH20 prophylaxis.

Health-related quality of life with @entity1659 following prophylaxis with subcutaneous XXXX with recombinant hyaluronidase.

@entity1 :: ('9606', 'Species') :: ['Patients', 'patients'] @entity1659 :: ('MESH:D054179', 'Disease') :: ['hereditary angioedema'] @entity19459 :: ('710', 'Gene') :: ['C1-inhibitor', 'INH'] @entity113 :: ('MESH:D000799', 'Disease') :: ['Angioedema'] @entity38524 :: ('710;3183', 'Gene') :: ['C1-INH']
@entity19459 :: (710,Gene) :: ['C1-inhibitor']
