PROPHYLAXIS IN RARE FACTOR DEFICIENCIES OF CHILDREN
(Abstract release date: 05/19/16)
EHA Library. Salcioglu Z. 06/09/16; 134657; PB1757
Dr. Zafer Salcioglu
Contributions
Contributions
Abstract
Abstract: PB1757
Type: Publication Only
Background
Rare factor deficiencies (RFD) cover the deficiencies of fibrinogen, Factor II, Factor V, Factor V+VIII, Factor VII, Factor X, Factor XI, Factor XIII, and vitamin K associated factors. Many national and international study groups conducted studies regarding the diagnosis and treatment of rare factor deficiencies in 2000s. The existence a mortality and morbidity risk associated with severe bleedings has brought the search for prophylaxes related to these disorders into the agenda. In addition, the good results obtained with primary and secondary prophylaxes in haemophilia cases have attracted the attention to the possibility that the same consideration could also be applied to rare factor deficiencies. Prophylaxis is recommended in these patients if such severe bleedings as central nervous system bleedings, gastrointestinal bleedings, and articular hemorrhages recur.
Aims
Our prophylaxis application related to 13 congenital rare factor deficiency cases being followed at our clinic comprising of five patients with Factor VII deficiency, four patients with Factor X deficiency, three patients with afibrinogenemia, and one patient with FV deficiency, were evaluated in our article.
Methods
Information concerning our patients was compiled from patient files and the data contained in the electronic information processing environment created after 2005.
Results
Presenting ages of our patients ranged between one week and 7 years of age. Seven of them were boys and 6 were girls. Prophylaxis was applied due to intracranial bleeding to three of our Factor VII deficiency patients, for gastrointestinal system bleeding to one of them, and for the development of chronic hemarthrosis and hemarthrosis that requires the application of radioisotope synovectomy to one patient. 20 mcg/kg active recombinant FVII was used once a week in the prophylaxis of our FVII deficiency cases. Transition to two doses a week was required for one patient. Prophylaxis is being applied with 20 IU/kg prothrombin complex concentrate two days a week to our patients with Factor X deficiency for central nervous system bleedings and with 50 mg/kg fibrinogen every two weeks to our patients with afibrinogenemia for recurring intramuscular bleedings causing severe pain and difficulty of walking. Our patient with FV deficiency to whom prophylaxis is applied due to intracranial bleeding, on the other hand, receives 20 ml/kg fresh frozen plasma once a week. The prophylaxis periods of our patients range between 2 months and 9 years.
Conclusion
The observation as well as clinical and laboratory findings of our patients together with the details related to their prophylaxis processes were presented in our proceedings accompanied with the relevant literature.
Session topic: E-poster
Keyword(s): Bleeding disorder, Children, Factors
Type: Publication Only
Background
Rare factor deficiencies (RFD) cover the deficiencies of fibrinogen, Factor II, Factor V, Factor V+VIII, Factor VII, Factor X, Factor XI, Factor XIII, and vitamin K associated factors. Many national and international study groups conducted studies regarding the diagnosis and treatment of rare factor deficiencies in 2000s. The existence a mortality and morbidity risk associated with severe bleedings has brought the search for prophylaxes related to these disorders into the agenda. In addition, the good results obtained with primary and secondary prophylaxes in haemophilia cases have attracted the attention to the possibility that the same consideration could also be applied to rare factor deficiencies. Prophylaxis is recommended in these patients if such severe bleedings as central nervous system bleedings, gastrointestinal bleedings, and articular hemorrhages recur.
Aims
Our prophylaxis application related to 13 congenital rare factor deficiency cases being followed at our clinic comprising of five patients with Factor VII deficiency, four patients with Factor X deficiency, three patients with afibrinogenemia, and one patient with FV deficiency, were evaluated in our article.
Methods
Information concerning our patients was compiled from patient files and the data contained in the electronic information processing environment created after 2005.
Results
Presenting ages of our patients ranged between one week and 7 years of age. Seven of them were boys and 6 were girls. Prophylaxis was applied due to intracranial bleeding to three of our Factor VII deficiency patients, for gastrointestinal system bleeding to one of them, and for the development of chronic hemarthrosis and hemarthrosis that requires the application of radioisotope synovectomy to one patient. 20 mcg/kg active recombinant FVII was used once a week in the prophylaxis of our FVII deficiency cases. Transition to two doses a week was required for one patient. Prophylaxis is being applied with 20 IU/kg prothrombin complex concentrate two days a week to our patients with Factor X deficiency for central nervous system bleedings and with 50 mg/kg fibrinogen every two weeks to our patients with afibrinogenemia for recurring intramuscular bleedings causing severe pain and difficulty of walking. Our patient with FV deficiency to whom prophylaxis is applied due to intracranial bleeding, on the other hand, receives 20 ml/kg fresh frozen plasma once a week. The prophylaxis periods of our patients range between 2 months and 9 years.
Conclusion
The observation as well as clinical and laboratory findings of our patients together with the details related to their prophylaxis processes were presented in our proceedings accompanied with the relevant literature.
Session topic: E-poster
Keyword(s): Bleeding disorder, Children, Factors
Abstract: PB1757
Type: Publication Only
Background
Rare factor deficiencies (RFD) cover the deficiencies of fibrinogen, Factor II, Factor V, Factor V+VIII, Factor VII, Factor X, Factor XI, Factor XIII, and vitamin K associated factors. Many national and international study groups conducted studies regarding the diagnosis and treatment of rare factor deficiencies in 2000s. The existence a mortality and morbidity risk associated with severe bleedings has brought the search for prophylaxes related to these disorders into the agenda. In addition, the good results obtained with primary and secondary prophylaxes in haemophilia cases have attracted the attention to the possibility that the same consideration could also be applied to rare factor deficiencies. Prophylaxis is recommended in these patients if such severe bleedings as central nervous system bleedings, gastrointestinal bleedings, and articular hemorrhages recur.
Aims
Our prophylaxis application related to 13 congenital rare factor deficiency cases being followed at our clinic comprising of five patients with Factor VII deficiency, four patients with Factor X deficiency, three patients with afibrinogenemia, and one patient with FV deficiency, were evaluated in our article.
Methods
Information concerning our patients was compiled from patient files and the data contained in the electronic information processing environment created after 2005.
Results
Presenting ages of our patients ranged between one week and 7 years of age. Seven of them were boys and 6 were girls. Prophylaxis was applied due to intracranial bleeding to three of our Factor VII deficiency patients, for gastrointestinal system bleeding to one of them, and for the development of chronic hemarthrosis and hemarthrosis that requires the application of radioisotope synovectomy to one patient. 20 mcg/kg active recombinant FVII was used once a week in the prophylaxis of our FVII deficiency cases. Transition to two doses a week was required for one patient. Prophylaxis is being applied with 20 IU/kg prothrombin complex concentrate two days a week to our patients with Factor X deficiency for central nervous system bleedings and with 50 mg/kg fibrinogen every two weeks to our patients with afibrinogenemia for recurring intramuscular bleedings causing severe pain and difficulty of walking. Our patient with FV deficiency to whom prophylaxis is applied due to intracranial bleeding, on the other hand, receives 20 ml/kg fresh frozen plasma once a week. The prophylaxis periods of our patients range between 2 months and 9 years.
Conclusion
The observation as well as clinical and laboratory findings of our patients together with the details related to their prophylaxis processes were presented in our proceedings accompanied with the relevant literature.
Session topic: E-poster
Keyword(s): Bleeding disorder, Children, Factors
Type: Publication Only
Background
Rare factor deficiencies (RFD) cover the deficiencies of fibrinogen, Factor II, Factor V, Factor V+VIII, Factor VII, Factor X, Factor XI, Factor XIII, and vitamin K associated factors. Many national and international study groups conducted studies regarding the diagnosis and treatment of rare factor deficiencies in 2000s. The existence a mortality and morbidity risk associated with severe bleedings has brought the search for prophylaxes related to these disorders into the agenda. In addition, the good results obtained with primary and secondary prophylaxes in haemophilia cases have attracted the attention to the possibility that the same consideration could also be applied to rare factor deficiencies. Prophylaxis is recommended in these patients if such severe bleedings as central nervous system bleedings, gastrointestinal bleedings, and articular hemorrhages recur.
Aims
Our prophylaxis application related to 13 congenital rare factor deficiency cases being followed at our clinic comprising of five patients with Factor VII deficiency, four patients with Factor X deficiency, three patients with afibrinogenemia, and one patient with FV deficiency, were evaluated in our article.
Methods
Information concerning our patients was compiled from patient files and the data contained in the electronic information processing environment created after 2005.
Results
Presenting ages of our patients ranged between one week and 7 years of age. Seven of them were boys and 6 were girls. Prophylaxis was applied due to intracranial bleeding to three of our Factor VII deficiency patients, for gastrointestinal system bleeding to one of them, and for the development of chronic hemarthrosis and hemarthrosis that requires the application of radioisotope synovectomy to one patient. 20 mcg/kg active recombinant FVII was used once a week in the prophylaxis of our FVII deficiency cases. Transition to two doses a week was required for one patient. Prophylaxis is being applied with 20 IU/kg prothrombin complex concentrate two days a week to our patients with Factor X deficiency for central nervous system bleedings and with 50 mg/kg fibrinogen every two weeks to our patients with afibrinogenemia for recurring intramuscular bleedings causing severe pain and difficulty of walking. Our patient with FV deficiency to whom prophylaxis is applied due to intracranial bleeding, on the other hand, receives 20 ml/kg fresh frozen plasma once a week. The prophylaxis periods of our patients range between 2 months and 9 years.
Conclusion
The observation as well as clinical and laboratory findings of our patients together with the details related to their prophylaxis processes were presented in our proceedings accompanied with the relevant literature.
Session topic: E-poster
Keyword(s): Bleeding disorder, Children, Factors
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