Analysis of individualized primary prophylactic treatment of 19 cases of children with severe hemophilia A

Analysis of individualized primary prophylactic treatment of 19 cases of children with severe hemophilia A

Objective: To study the current situation of primary prophylaxis in severe hemophilia A children and to explore rational regimen in order to provide evidence for the development of primary prophylaxis in China. Method: A retrospective clinical data collection and analysis was conducted for 19 severe...

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Veröffentlicht in:Zhonghua er ke za zhi = Chinese journal of pediatrics. - 1960. - 54(2016), 12 vom: 02. Dez., Seite 923-926
1. Verfasser: Liu, G Q (VerfasserIn)
Weitere Verfasser: Tang, L, Wu, X Y, Zhen, Y Z, Li, G, Chen, Z P, Wang, Y, Zhang, N N, Zhang, J S, Yu, G X, Wu, R H
Format: Online-Aufsatz
Sprache:Chinese
Veröffentlicht: 2016
Zugriff auf das übergeordnete Werk:Zhonghua er ke za zhi = Chinese journal of pediatrics
Schlagworte:Journal Article Coagulants Factor VIII 9001-27-8
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245 1 0 |a Analysis of individualized primary prophylactic treatment of 19 cases of children with severe hemophilia A 
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520 |a Objective: To study the current situation of primary prophylaxis in severe hemophilia A children and to explore rational regimen in order to provide evidence for the development of primary prophylaxis in China. Method: A retrospective clinical data collection and analysis was conducted for 19 severe hemophilia A children who received primary prophylaxis in Beijing Children's Hospital outpatient clinic between February 2011 and September 2015 and evaluated the regimen and efficacy. Result: (1) Primary prophylaxis regimen: the median beginning age 1.8 (range 0.5-2.9) years, the median FⅧ preparation using dosage 16.7 (8.0-23.5) U/(kg·time), the median using frequency was 1.0 (1.0-3.0) time/week. Eight cases among the patients received escalation of treatment intensity because of the poor bleeding control. (2) Efficacy: the median annual bleeding rate (ABR) was 1.9 (0-6.0) times/year, the median annual joint bleeding rate (AJBR) was 0 (0-3.3) times/year, without life threatening bleeding. All of them kept in 4th scale of Beijing Children Hospital daily activity level. The median annual factor consumption was 1 844 (840-5 040) U/kg. Conclusion: Low-dose primary prophylaxis regimen which were in low-dose /low frequencies and adjusted by bleeding frequency could decrease bleeding and joint bleeding frequency significantly, maintained the normal daily activity capacity and saved the factor consumption compared to standard regimen in severe hemophilia A children 
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700 1 |a Tang, L  |e verfasserin  |4 aut 
700 1 |a Wu, X Y  |e verfasserin  |4 aut 
700 1 |a Zhen, Y Z  |e verfasserin  |4 aut 
700 1 |a Li, G  |e verfasserin  |4 aut 
700 1 |a Chen, Z P  |e verfasserin  |4 aut 
700 1 |a Wang, Y  |e verfasserin  |4 aut 
700 1 |a Zhang, N N  |e verfasserin  |4 aut 
700 1 |a Zhang, J S  |e verfasserin  |4 aut 
700 1 |a Yu, G X  |e verfasserin  |4 aut 
700 1 |a Wu, R H  |e verfasserin  |4 aut 
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