title: Intracranial haemorrhage in patients with congenital haemostatic defects.
abstract: We investigated 52 of 457 patients with congenital factor deficiencies with 57 episodes of intracranial haemorrhage (ICH) between 1998 and 2007. There were 38 severe haemophiliacs, 6 with factor XIII deficiency, 5 with factor X deficiency, 2 factor V-deficient patients, and 1 with type 3 von Willebrand disease (VWD). The median age was 8 years (range 1 month-22 years). Most patients were below 15 years of age (86.5%). All patients with factor X deficiency were between 1 and 5 months of age. ICH was the primary bleeding episode leading to detection of factor deficiency in 19.2% (five patients with severe haemophilia and all patients with factor X deficiency). Trauma caused bleeding in 66%. None of the patients with factor X deficiency had history of prior trauma. Surgery was performed in five patients with subdural haematomas, all of whom survived. Conservative factor replacement with 100% correction for 3 days followed by 50-60% correction for 7 days was possible in 60% patients. Seizures requiring prolonged therapy were noted in eight patients. Death was recorded in 15 patients (29%). Inadequate therapy in the form of delay or insufficient replacement was noted in 7/15 deaths. ICH was seen in 11.3% of all patients with coagulation factor deficiencies. Factor X deficiency presented with ICH at an earlier age. Inadequate replacement therapy including delayed treatment caused nearly 50% of all deaths. Most patients can be managed satisfactorily with adequate replacement therapy alone, with surgery being reserved for those with worsening neurological conditions.
authors.affiliation: Department of Hematology, All India Institute of Medical Sciences, New Delhi, India. pravas_mishra@rediffmail.com
authors.forename: P
authors.initials: P
authors.lastname: Mishra
authors.forename: R
authors.initials: R
authors.lastname: Naithani
authors.forename: T
authors.initials: T
authors.lastname: Dolai
authors.forename: R
authors.initials: R
authors.lastname: Bhargava
authors.forename: M
authors.initials: M
authors.lastname: Mahapatra
authors.forename: A
authors.initials: A
authors.lastname: Dixit
authors.forename: T
authors.initials: T
authors.lastname: Seth
authors.forename: R
authors.initials: R
authors.lastname: Kumar
authors.forename: R
authors.initials: R
authors.lastname: Saxena
mesh_terms: D000293:Adolescent; D025861:Blood Coagulation Disorders, Inherited / Q000150:complications*; D002648:Child; D002675:Child, Preschool; D005166:Factor V Deficiency / Q000150:complications; D005171:Factor X Deficiency / Q000150:complications; D006467:Hemophilia A / Q000150:complications; D006801:Humans; D007223:Infant; D020300:Intracranial Hemorrhages / Q000209:etiology* / Q000628:therapy; D008297:Male; D012189:Retrospective Studies; D055815:Young Adult