PROPHYLAXIS FOR HEMOPHILIA PATIENT IN KOREA
(Abstract release date: 05/19/16)
EHA Library. Hyun S. 06/09/16; 134653; PB1753
Prof. Dr. Shinyoung Hyun
Contributions
Contributions
Abstract
Abstract: PB1753
Type: Publication Only
Background
Prophylaxis compare to on-demand treatment for severe hemophilia patients has a great profit for not only life quality but also life itself.
Aims
We have started prophylaxis for 5 intracranial hemorrhage patients after recovery since 1996. Fifteen years later, prophylaxis for hemophilia A and B patients only under 15 y/o have been approved by National Health Insurance Review and Assessment from January, 2011.
Methods
Intracranial hemorrhage was observed in 16 episodes from 9 patients between 1996 and 2002 at our hospital. All patients received FVIII concentrates with initial loading dose of FVIII 50 U/kg and then the continuous infusion, 3 U/kg/hr for 3 days, and then gradually decreased the volume for 7-10 days. We persuaded the patient/parent for life long prophylaxis to prevent further life-threatening hemorrhage. Five among 8 patients followed the prophylaxis (30 U/kg, 3 days/week).
Results
Age distribution was 2 mo-26 yr (mean; 8.8 yr). Severity was severe (4), moderate (4) and mild (1). Trauma history was in only 1 case. Initial symptom was vomiting (8), headache (4), seizure (3), and irritability (1). Time interval between first symptom and arrival at ER were 15 min to 10 days (mean; 1.7days). Intracranial bleeding site was variety. Inhibitor was in 1 case and 2 cases were transient type. Previous life threatening hemorrhage history was observed in 5 cases (ICH in 4 and GI bleeding in 1). Family history was observed in 7 cases. Eight patients received FVIII concentrates with initial loading dose of FVIII 50 U/kg and then the continuous infusion, 3 U/kg/hr for 3 days, and then gradually decrease the volume for 7-10 days. Inhibitor patient was treated with by-passing agent. Craniotomy was done in 4 cases and no operation was in 4 cases. All recovered except one (who died within 30 minutes after arrival, bleeding site was cerebellar vermis & 3rd ventricle) and are alive without any neurologic sequelae except one with mild limping gate for more than 10 years.
Conclusion
Prophylaxis for severe hemophilia has to expand to all age, to moderate hemophilia with previous life threatening bleeding history and to inhibitor patients in Korea.
Session topic: E-poster
Keyword(s): Hemophilia, Prophylaxis
Type: Publication Only
Background
Prophylaxis compare to on-demand treatment for severe hemophilia patients has a great profit for not only life quality but also life itself.
Aims
We have started prophylaxis for 5 intracranial hemorrhage patients after recovery since 1996. Fifteen years later, prophylaxis for hemophilia A and B patients only under 15 y/o have been approved by National Health Insurance Review and Assessment from January, 2011.
Methods
Intracranial hemorrhage was observed in 16 episodes from 9 patients between 1996 and 2002 at our hospital. All patients received FVIII concentrates with initial loading dose of FVIII 50 U/kg and then the continuous infusion, 3 U/kg/hr for 3 days, and then gradually decreased the volume for 7-10 days. We persuaded the patient/parent for life long prophylaxis to prevent further life-threatening hemorrhage. Five among 8 patients followed the prophylaxis (30 U/kg, 3 days/week).
Results
Age distribution was 2 mo-26 yr (mean; 8.8 yr). Severity was severe (4), moderate (4) and mild (1). Trauma history was in only 1 case. Initial symptom was vomiting (8), headache (4), seizure (3), and irritability (1). Time interval between first symptom and arrival at ER were 15 min to 10 days (mean; 1.7days). Intracranial bleeding site was variety. Inhibitor was in 1 case and 2 cases were transient type. Previous life threatening hemorrhage history was observed in 5 cases (ICH in 4 and GI bleeding in 1). Family history was observed in 7 cases. Eight patients received FVIII concentrates with initial loading dose of FVIII 50 U/kg and then the continuous infusion, 3 U/kg/hr for 3 days, and then gradually decrease the volume for 7-10 days. Inhibitor patient was treated with by-passing agent. Craniotomy was done in 4 cases and no operation was in 4 cases. All recovered except one (who died within 30 minutes after arrival, bleeding site was cerebellar vermis & 3rd ventricle) and are alive without any neurologic sequelae except one with mild limping gate for more than 10 years.
Conclusion
Prophylaxis for severe hemophilia has to expand to all age, to moderate hemophilia with previous life threatening bleeding history and to inhibitor patients in Korea.
Session topic: E-poster
Keyword(s): Hemophilia, Prophylaxis
Abstract: PB1753
Type: Publication Only
Background
Prophylaxis compare to on-demand treatment for severe hemophilia patients has a great profit for not only life quality but also life itself.
Aims
We have started prophylaxis for 5 intracranial hemorrhage patients after recovery since 1996. Fifteen years later, prophylaxis for hemophilia A and B patients only under 15 y/o have been approved by National Health Insurance Review and Assessment from January, 2011.
Methods
Intracranial hemorrhage was observed in 16 episodes from 9 patients between 1996 and 2002 at our hospital. All patients received FVIII concentrates with initial loading dose of FVIII 50 U/kg and then the continuous infusion, 3 U/kg/hr for 3 days, and then gradually decreased the volume for 7-10 days. We persuaded the patient/parent for life long prophylaxis to prevent further life-threatening hemorrhage. Five among 8 patients followed the prophylaxis (30 U/kg, 3 days/week).
Results
Age distribution was 2 mo-26 yr (mean; 8.8 yr). Severity was severe (4), moderate (4) and mild (1). Trauma history was in only 1 case. Initial symptom was vomiting (8), headache (4), seizure (3), and irritability (1). Time interval between first symptom and arrival at ER were 15 min to 10 days (mean; 1.7days). Intracranial bleeding site was variety. Inhibitor was in 1 case and 2 cases were transient type. Previous life threatening hemorrhage history was observed in 5 cases (ICH in 4 and GI bleeding in 1). Family history was observed in 7 cases. Eight patients received FVIII concentrates with initial loading dose of FVIII 50 U/kg and then the continuous infusion, 3 U/kg/hr for 3 days, and then gradually decrease the volume for 7-10 days. Inhibitor patient was treated with by-passing agent. Craniotomy was done in 4 cases and no operation was in 4 cases. All recovered except one (who died within 30 minutes after arrival, bleeding site was cerebellar vermis & 3rd ventricle) and are alive without any neurologic sequelae except one with mild limping gate for more than 10 years.
Conclusion
Prophylaxis for severe hemophilia has to expand to all age, to moderate hemophilia with previous life threatening bleeding history and to inhibitor patients in Korea.
Session topic: E-poster
Keyword(s): Hemophilia, Prophylaxis
Type: Publication Only
Background
Prophylaxis compare to on-demand treatment for severe hemophilia patients has a great profit for not only life quality but also life itself.
Aims
We have started prophylaxis for 5 intracranial hemorrhage patients after recovery since 1996. Fifteen years later, prophylaxis for hemophilia A and B patients only under 15 y/o have been approved by National Health Insurance Review and Assessment from January, 2011.
Methods
Intracranial hemorrhage was observed in 16 episodes from 9 patients between 1996 and 2002 at our hospital. All patients received FVIII concentrates with initial loading dose of FVIII 50 U/kg and then the continuous infusion, 3 U/kg/hr for 3 days, and then gradually decreased the volume for 7-10 days. We persuaded the patient/parent for life long prophylaxis to prevent further life-threatening hemorrhage. Five among 8 patients followed the prophylaxis (30 U/kg, 3 days/week).
Results
Age distribution was 2 mo-26 yr (mean; 8.8 yr). Severity was severe (4), moderate (4) and mild (1). Trauma history was in only 1 case. Initial symptom was vomiting (8), headache (4), seizure (3), and irritability (1). Time interval between first symptom and arrival at ER were 15 min to 10 days (mean; 1.7days). Intracranial bleeding site was variety. Inhibitor was in 1 case and 2 cases were transient type. Previous life threatening hemorrhage history was observed in 5 cases (ICH in 4 and GI bleeding in 1). Family history was observed in 7 cases. Eight patients received FVIII concentrates with initial loading dose of FVIII 50 U/kg and then the continuous infusion, 3 U/kg/hr for 3 days, and then gradually decrease the volume for 7-10 days. Inhibitor patient was treated with by-passing agent. Craniotomy was done in 4 cases and no operation was in 4 cases. All recovered except one (who died within 30 minutes after arrival, bleeding site was cerebellar vermis & 3rd ventricle) and are alive without any neurologic sequelae except one with mild limping gate for more than 10 years.
Conclusion
Prophylaxis for severe hemophilia has to expand to all age, to moderate hemophilia with previous life threatening bleeding history and to inhibitor patients in Korea.
Session topic: E-poster
Keyword(s): Hemophilia, Prophylaxis
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