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单中心成人重型血友病A低/中剂量凝血因子Ⅷ预防治疗30例疗效分析

OBJECTIVE: To evaluate the clinical effects of low- and intermediate-dose factor Ⅷ(FⅧ)prophylaxis in Chinese adult patients with severe hemophilia A. METHODS: Thirty adult patients with severe hemophilia A who received low-(n=20)/intermediate-dose(n=10)FⅧ prophylaxis at Nanjing Drum Tower Hospital a...

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Formato: Online Artículo Texto
Lenguaje:English
Publicado: Editorial office of Chinese Journal of Hematology 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10067379/
https://www.ncbi.nlm.nih.gov/pubmed/36987721
http://dx.doi.org/10.3760/cma.j.issn.0253-2727.2023.01.007
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description OBJECTIVE: To evaluate the clinical effects of low- and intermediate-dose factor Ⅷ(FⅧ)prophylaxis in Chinese adult patients with severe hemophilia A. METHODS: Thirty adult patients with severe hemophilia A who received low-(n=20)/intermediate-dose(n=10)FⅧ prophylaxis at Nanjing Drum Tower Hospital affiliated with Nanjing University Medical College were included in the study. The annual bleeding rate(ABR), annual joint bleeding rate(AJBR), number of target joints, functional independence score of hemophilia(FISH), quality of life score, and health status score(SF-36)before and after preventive treatment were retrospectively analyzed and compared. RESULTS: The median follow-up was 48 months. Compared with on-demand treatment, low- and intermediate-dose prophylaxis significantly reduced ABR, AJBR, and the number of target joints(P<0.05); the improvement in the intermediate-dose prophylaxis group was better than that in the low-dose prophylaxis group(P<0.05). Compared with on-demand treatment, the FISH score, quality of life score, and SF-36 score significantly improved in both groups(P<0.05), but there was no significant difference between the two groups(P>0.05). CONCLUSION: In Chinese adults with severe hemophilia A, low- and intermediate-dose prophylaxis can significantly reduce bleeding frequency, delay the progression of joint lesions, and improve the quality of life of patients as compared with on-demand treatment. The improvement in clinical bleeding was better with intermediate-dose prophylaxis than low-dose prophylaxis.
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spelling pubmed-100673792023-04-04 单中心成人重型血友病A低/中剂量凝血因子Ⅷ预防治疗30例疗效分析 Zhonghua Xue Ye Xue Za Zhi 论著 OBJECTIVE: To evaluate the clinical effects of low- and intermediate-dose factor Ⅷ(FⅧ)prophylaxis in Chinese adult patients with severe hemophilia A. METHODS: Thirty adult patients with severe hemophilia A who received low-(n=20)/intermediate-dose(n=10)FⅧ prophylaxis at Nanjing Drum Tower Hospital affiliated with Nanjing University Medical College were included in the study. The annual bleeding rate(ABR), annual joint bleeding rate(AJBR), number of target joints, functional independence score of hemophilia(FISH), quality of life score, and health status score(SF-36)before and after preventive treatment were retrospectively analyzed and compared. RESULTS: The median follow-up was 48 months. Compared with on-demand treatment, low- and intermediate-dose prophylaxis significantly reduced ABR, AJBR, and the number of target joints(P<0.05); the improvement in the intermediate-dose prophylaxis group was better than that in the low-dose prophylaxis group(P<0.05). Compared with on-demand treatment, the FISH score, quality of life score, and SF-36 score significantly improved in both groups(P<0.05), but there was no significant difference between the two groups(P>0.05). CONCLUSION: In Chinese adults with severe hemophilia A, low- and intermediate-dose prophylaxis can significantly reduce bleeding frequency, delay the progression of joint lesions, and improve the quality of life of patients as compared with on-demand treatment. The improvement in clinical bleeding was better with intermediate-dose prophylaxis than low-dose prophylaxis. Editorial office of Chinese Journal of Hematology 2023-01 /pmc/articles/PMC10067379/ /pubmed/36987721 http://dx.doi.org/10.3760/cma.j.issn.0253-2727.2023.01.007 Text en 2023年版权归中华医学会所有 https://creativecommons.org/licenses/by/3.0/This work is licensed under a Creative Commons Attribution 3.0 License.
spellingShingle 论著
单中心成人重型血友病A低/中剂量凝血因子Ⅷ预防治疗30例疗效分析
title 单中心成人重型血友病A低/中剂量凝血因子Ⅷ预防治疗30例疗效分析
title_full 单中心成人重型血友病A低/中剂量凝血因子Ⅷ预防治疗30例疗效分析
title_fullStr 单中心成人重型血友病A低/中剂量凝血因子Ⅷ预防治疗30例疗效分析
title_full_unstemmed 单中心成人重型血友病A低/中剂量凝血因子Ⅷ预防治疗30例疗效分析
title_short 单中心成人重型血友病A低/中剂量凝血因子Ⅷ预防治疗30例疗效分析
title_sort 单中心成人重型血友病a低/中剂量凝血因子ⅷ预防治疗30例疗效分析
topic 论著
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10067379/
https://www.ncbi.nlm.nih.gov/pubmed/36987721
http://dx.doi.org/10.3760/cma.j.issn.0253-2727.2023.01.007
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