title: [Low-dose Tertiary Prophylactic Therapy for Severe Haemophilia A Adults in A Single Medical Center in Beijing Area].
abstract: OBJECTIVE
  To investigate the benefit of low-dose tertiary prophylaxis in adults with severe haemophilia A(SHA).
  
  
  METHODS
  Twenty-two SHA patients aged 18 to 60 years from the Haemophilia Centre of Peking Union Medical College Hospital, Beijing, China, were retrospectively observed from their one year on-demand treatment to one year tertiary prophylaxis using plasma derived factor 8 concentrates at 5-10 IU/kg 2-3x per week. All the patients had already developed arthropathy. Gilbert and the functional independence scores in hemophilia were used to assess the joint status and the ability in the activities of daily living of the patients.
  
  
  RESULTS
  Comparing with on-demand therapy,the annual bleeding frequency during low-dose tertiary prophylaxis decreased significantly by 72.7%,from 39.9 ± 21.5 to 11.1 ± 7.2 (P<0.0001),the total Gilbert score decreased from 50.5±32.1 to 45.2±29.6(P<0.05),and the total functional independence score in hemophilia score increased from 18.6 ± 5.2 to 21.7 ± 4.1 (P<0.05). CONCLUSION Low-dose tertiary prophylaxis in adults with SHA is beneficial by reducing bleeding frequency, improving the health status of joints, and improving the activities of daily living, thus raising the quality of life.
authors.affiliation: 1. Department of Hematology,PUMC Hospital,CAMS and PUMC,Beijing 100730,China2Department of Hematology,Shaanxi Provincial People's Hospital,Xi'an 710068,China;
authors.forename: Xiao-yun
authors.initials: XY
authors.lastname: Lian
authors.affiliation: Department of Hematology,PUMC Hospital,CAMS and PUMC,Beijing 100730,China2Department of Hematology,Shaanxi Provincial People's Hospital,Xi'an 710068,China.
authors.forename: Kui-xing
authors.initials: KX
authors.lastname: Li
authors.affiliation: Department of Hematology,PUMC Hospital,CAMS and PUMC,Beijing 100730,China2Department of Hematology,Shaanxi Provincial People's Hospital,Xi'an 710068,China.
authors.forename: Bao-lai
authors.initials: BL
authors.lastname: Hua
authors.affiliation: Department of Hematology,PUMC Hospital,CAMS and PUMC,Beijing 100730,China2Department of Hematology,Shaanxi Provincial People's Hospital,Xi'an 710068,China.
authors.forename: Yong-qiang
authors.initials: YQ
authors.lastname: Zhao
mesh_terms: D000203:Activities of Daily Living; D000293:Adolescent; D000328:Adult; D000068476:Beijing; D006395:Hemarthrosis; D006467:Hemophilia A; D006470:Hemorrhage; D006801:Humans; D008875:Middle Aged; D011788:Quality of Life; D012189:Retrospective Studies; D055815:Young Adult