1/20. Clinical efficacy and recovery levels of recombinant FVIIa (NovoSeven) in the treatment of intracranial haemorrhage in severe neonatal FVII deficiency.The use of replacement FVII is critical to the successful treatment of life-threatening bleeds in newborns and infants with severe FVII deficiency (<1%). However, the clinical efficacy, optimum dosage and pharmacologic recovery of rFVIIa in such children has not been studied systematically. This report is a case of an infant with severe FVII deficiency (FVII:C at 0%) and massive intracranial haemorrhage in which successful use of rFVIIa (NovoSeven) was carefully monitored. The drug was administered by intravenous bolus through a central line every 4 h at each of three dose levels: 15 microg kg-1, 22 microg kg-1 and 30 microg kg-1. FVII:C was >100% between 30 and 180 min after each infusion with mean trough levels above 25% for all three dose levels. There was no evidence of hyper-coagulation as indicated by measurements of the platelet count, D-dimer, plasma protamine paracoagulant and fibrinogen levels in spite of high FVII:C concentration. In this infant, rFVIIa was well-tolerated, maintained effective haemostasis with good clinical outcome, and produced consistent therapeutic mean trough levels above 25% FVII:C even at 15 microg kg-1 every 4 h.- - - - - - - - - - ranking = 1keywords = coagulation (Clic here for more details about this article) |
2/20. Possible linkage of amprenavir with intracranial bleeding in an hiv-infected hemophiliac.The use of protease inhibitors (PI) has been associated with many adverse effects including increased tendency to bleed, which is particularly problematic in individuals with congenital coagulation disorders. We report the occurrence of spontaneous intracranial bleeding in an human immunodeficiency virus (hiv)-infected adolescent with hemophilia a who was receiving amprenavir (APV). The bleeding resolved on discontinuation of APV. This case report highlights a need for awareness of increased bleeding as a potentially serious complication associated with the use of all currently licensed PIs in individuals with hemophilia.- - - - - - - - - - ranking = 49.016890176389keywords = coagulation disorder, coagulation (Clic here for more details about this article) |
3/20. Intrauterine correction of factor viii (FVIII) deficiency.This is the first published report documenting the successful use of intrauterine infusion of factor viii (FVIII) in order to reduce the risk of intracranial haemorrhage in a foetus with documented haemophilia A. This approach provides another option for management of newborns with documented coagulation factor deficiency. The subsequent development of an inhibitor directed against FVIII is believed to be related to exposure to exogenous FVIII in the presence of an inversion mutation and not to the intrauterine procedure.- - - - - - - - - - ranking = 1keywords = coagulation (Clic here for more details about this article) |
4/20. A rare cause of intracranial hemorrhage: factor x deficiency.Congenital factor x deficiency is a rare inherited coagulation disorder, characterized by prolonged prothrombin time and partial thromboplastin time. For the definite diagnosis, specific factor X level should be investigated. We describe a patient with factor x deficiency who had intracranial hemorrhage. hematologic tests showed prolonged prothrombin time, partial thromboplastin time, and a factor X level of 5%. The patient's hemorrhage resolved with fresh frozen plasma replacement. In this article, we discuss the clinical features and management of factor x deficiency.- - - - - - - - - - ranking = 49.016890176389keywords = coagulation disorder, coagulation (Clic here for more details about this article) |
5/20. angioplasty and stenting for symptomatic basilar artery stenosis.A 71-year-old man with symptomatic basilar artery stenosis refractory to antiplatelet therapy and anticoagulation underwent angioplasty and stenting of the basilar artery. The patient was discharged symptom-free on clodiprogel 75 mg qd and aspirin 325 mg qd. One month later, he presented with a right frontal intracerebral hemorrhage. angioplasty with stenting for symptomatic basilar artery stenosis is feasible. Its role in the therapy of symptomatic basilar artery stenosis is not yet defined. Furthermore, the best medical treatment postprocedure is yet to be established.- - - - - - - - - - ranking = 1keywords = coagulation (Clic here for more details about this article) |
6/20. Treatment of dural arteriovenous fistulae (dAVF's) at the superior sagittal sinus (SSS) using embolisation combined with micro- or radiosurgery.DAVF's at the SSS are extremely rare and usually present with intracranial haemorrhage (ICH) or a progressive neurological deficit. Due to their midline location and multiple arterial supply, endovascular treatment alone often fails in eliminating the fistula. Therefore, endovascular, combined with neurosurgical and/or radiosurgical treatment is often needed to cure the patient. We summarized our experience with three male patients over a ten-year period who suffered from dAVF's involving the middle and posterior third of the SSS. Two of them presented with an ICH during the clinical course. Despite multiple transarterial embolisations, complete fistula occlusion could not be achieved in any of them. Nevertheless, neurological symptoms improved in all cases. One patient refused further treatment and died six years later due to an ICH. Another patient was finally cured by microsurgical coagulation of the fistula. In the remaining patient stereotactic radiosurgery (SRS) was performed after nearly complete endovascular occlusion. We strongly recommend microsurgery and/or radiosurgery as adjunctive measures, if embolisation alone fails to eliminate these dangerous fistulae.- - - - - - - - - - ranking = 1keywords = coagulation (Clic here for more details about this article) |
7/20. Ipsilateral intracerebral hemorrhage following carotid stent-assisted angioplasty: a manifestation of hyperperfusion syndrome--a case report.A case of hyperperfusion syndrome manifested as intracerebral hemorrhage following carotid stent-assisted angioplasty while using intravenous abciximab is described. review of literature regarding hyperperfusion syndrome in patients undergoing carotid artery revascularization is presented. Possible mechanisms of hyperperfusion and the role of arterial hypertension, anticoagulation, and antiplatelet treatment in the genesis of hyperperfusion syndrome are discussed. Widening use of percutaneous carotid revascularization necessitates physicians' awareness of early recognition of this complication.- - - - - - - - - - ranking = 1keywords = coagulation (Clic here for more details about this article) |
8/20. Valproate induces reversible factor xiii deficiency with risk of perioperative bleeding.The antiepileptic drug valproic acid (VPA) induces subclinical changes in both the intrinsic and extrinsic coagulation system. However, fatal bleeding is very rare. This study reports a 39-year-old patient who underwent selective amygdalohippocampectomy because of drug-resistant temporal lobe epilepsy. Preoperatively, the patient was on a combined therapy with VPA and topiramate, and routine coagulation laboratory parameters were entirely normal. epilepsy surgery was immediately followed by severe intracranial bleeding events which promped repeated craniectomy. Extensive laboratory analyses revealed a factor XIII activity level of 17%, indicating factor xiii deficiency confirmed by a reduced XIIIA-antigen. After termination of treatment with VPA, factor XIII levels returned to normal. Control examinations after 9 and 24 months showed normal range values for all coagulation parameters, including factor XIII, platelet function, and von willebrand factor. To our knowledge, this case is the first description of a well-documented, clinically relevant transient factor XIII-deficiency syndrome related to VPA treatment.- - - - - - - - - - ranking = 3keywords = coagulation (Clic here for more details about this article) |
9/20. brain haemorrhage in five infants with coagulopathy.Most intracranial haemorrhages in infants after the neonatal period are secondary to non-accidental injury. Occasionally brain haemorrhages in non-mobile infants are due to an inherited coagulopathy. This may often be diagnosed with a coagulation screen on admission. Little is known about the neurological outcome of infants in the latter group. Five infants are described who presented with acute spontaneous brain haemorrhage secondary to an inherited coagulopathy (n = 3) and vitamin k deficiency in alpha(1) antitrypsin deficiency (n = 1) and Alagille's syndrome (n = 1). Despite the critical clinical presentation and the severe imaging findings, these five infants made a good neurological recovery. Infants presenting with spontaneous ICH due to a significant (inherited) coagulopathy are usually easy to differentiate from non-accidental shaking injury; their bleeding pattern within the brain seems different from non-accidental shaking injury and neurodevelopment outcome appears better.- - - - - - - - - - ranking = 1keywords = coagulation (Clic here for more details about this article) |
10/20. Drotrecogin alfa (activated) in an infant with gram-negative septic shock.The authors observed the effect of drotrecogin alfa (activated) in a case of pediatric severe sepsis. A 4-month-old male infant with serratia marcescens septic shock, multiple organ dysfunction syndrome (MODS), and consumptive coagulopathy was admitted. The safety and efficacy of drotrecogin alfa (activated) has not yet been established for patients younger than 18 years of age. This is the first published report of the use of drotrecogin alfa (activated) in an infant with severe sepsis. Within 6 hours of starting therapy, there was a significant improvement in hemodynamics, which was not maintained after the drotrecogin alfa (activated) infusion was temporarily discontinued. No significant bleeding complications occurred during the infusion. A brain MRI on day 22 after drotrecogin alfa (activated) infusion showed bilateral small occipital hemorrhages. Drotrecogin alfa (activated) in this infant was temporally related to significant improvement. It is unknown whether the MRI brain lesions are related to severe sepsis with disseminated intravascular coagulation or drotrecogin alfa (activated) infusion. The authors believe that drotrecogin alfa (activated) should be considered in select children with life-threatening severe sepsis.- - - - - - - - - - ranking = 1keywords = coagulation (Clic here for more details about this article) |
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