Golestan University of Medical Science
Contributions
Type: Publication Only
Background
Congenital factor XIII (FXIII) deficiency is a rare autosomal recessive coagulation disorder. This congenital disorder is associated with poor wound healing, spontaneous abortion, life-long tendency towards spontaneous bleeding and a high risk of potentially life-threatening intracranial hemorrhage.
Aims
Routine prophilaxis with FXIII concentrate is recommended in all individuals with FXIII levels of <1 IU/dl from the time of diagnosis and in some severely affected patients with FXIII levels of 1-4 IU/dl.Therefore reliable assays for FXIII are necessary not only for the diagnosis of deficiency state but also to guide prophilaxis and replacement therapy in patients during times of increased risk. In Iran, ureaclot lysis test (FXIII screen) is the most available test and reliable assays are not accessible because of limitations of laboratory equipment. Hence, we recommended prophilaxis in any cases with abnormal FXIII screen and history of intracranial hemorrhage.
Methods
In here, it is reported the clinical course and outcome of 38-month old boy with intracranial hemorrhage that successfully managed and received prophilaxy with fibrogammin® P.
He had a history of recurrent spontaneous bleeding or bleeding after trauma in extremities from birth. He admitted at 27 month of age in hospital due to gluteous hematoma after muscle injection. He was tested for PLT, PT, PTT, INR, fibrinogen (clauss method) and FXIII screen (ureaclot lysis test). He had abnormal FXIII screen test and other tests were normal. He managed with fresh froze plasma before diagnosis and recommended for on demand therapy after diagnosis
Results
He admitted at 30 months of age due to headache and vomiting. Brain CT scan revealed intracranial hemorrhage. He was treated with fibrogammin® P. The prophylactic substitution therapy with fibrogammin® P 20 IU/kg every 4 weeks performed and he is free from bleeding episodes. Nugent et al, 2012, studied on 41 patients with congenital FXIII deficiency. Fibrogammin® P was administrated intravenously at an initial dose of 40 IU/kg every 4 weeks, with dosing adjusted to maintain a trough FXIII activity level of 5-20%. No spontaneous bleeding episodes requiring FXIII treatment were reported during the study.
Summary
According to inaccessibility to reliable assays of FXIII in Iran we recommended prophilaxy with fibrogammin® P in all cases with intracranial hemorrhage.
Keyword(s): Factor XIII, Hemorrhage
Type: Publication Only
Background
Congenital factor XIII (FXIII) deficiency is a rare autosomal recessive coagulation disorder. This congenital disorder is associated with poor wound healing, spontaneous abortion, life-long tendency towards spontaneous bleeding and a high risk of potentially life-threatening intracranial hemorrhage.
Aims
Routine prophilaxis with FXIII concentrate is recommended in all individuals with FXIII levels of <1 IU/dl from the time of diagnosis and in some severely affected patients with FXIII levels of 1-4 IU/dl.Therefore reliable assays for FXIII are necessary not only for the diagnosis of deficiency state but also to guide prophilaxis and replacement therapy in patients during times of increased risk. In Iran, ureaclot lysis test (FXIII screen) is the most available test and reliable assays are not accessible because of limitations of laboratory equipment. Hence, we recommended prophilaxis in any cases with abnormal FXIII screen and history of intracranial hemorrhage.
Methods
In here, it is reported the clinical course and outcome of 38-month old boy with intracranial hemorrhage that successfully managed and received prophilaxy with fibrogammin® P.
He had a history of recurrent spontaneous bleeding or bleeding after trauma in extremities from birth. He admitted at 27 month of age in hospital due to gluteous hematoma after muscle injection. He was tested for PLT, PT, PTT, INR, fibrinogen (clauss method) and FXIII screen (ureaclot lysis test). He had abnormal FXIII screen test and other tests were normal. He managed with fresh froze plasma before diagnosis and recommended for on demand therapy after diagnosis
Results
He admitted at 30 months of age due to headache and vomiting. Brain CT scan revealed intracranial hemorrhage. He was treated with fibrogammin® P. The prophylactic substitution therapy with fibrogammin® P 20 IU/kg every 4 weeks performed and he is free from bleeding episodes. Nugent et al, 2012, studied on 41 patients with congenital FXIII deficiency. Fibrogammin® P was administrated intravenously at an initial dose of 40 IU/kg every 4 weeks, with dosing adjusted to maintain a trough FXIII activity level of 5-20%. No spontaneous bleeding episodes requiring FXIII treatment were reported during the study.
Summary
According to inaccessibility to reliable assays of FXIII in Iran we recommended prophilaxy with fibrogammin® P in all cases with intracranial hemorrhage.
Keyword(s): Factor XIII, Hemorrhage