title: [Haemophilia A and intracranial bleedings in infants].
abstract: OBJECTIVE
  Haemophilia is a well-known X-linked recessive bleeding disorder related to a deficiency of factor VIII. The aim of the paper is to point out the role of an early diagnosis in cases of intracranial bleeding, especially in neonates and to highlight the bad prognosis.
  
  
  METHODS
  Three patients were included in the study from our prospective data. All had severe factor VIII deficiency (less than 1%).
  
  
  RESULTS
  The first of them was a newborn without familial history of haemophilia which presented with hypotonia and seizures after a delivery with vacuum. CT showed a subdural haematoma and MRI multiple ischemic lesions. Two months later, he died from a new intracranial haemorrhagic episode resistent to infusion of factor VIII related to development of an anti-factor VIII antiboby. The second neonate had no familial history more and experienced hypothermia and areactive mydriasis because of an acute subdural haematoma. He died eight days later of ischemic lesions in spite of a craniotomy. The third was a 5-month-old boy with a familial history. Physical examination revealed lethargy, seizure and a recent cranial perimeter increase. He underwent a craniotomy with a good result.
  
  
  CONCLUSIONS
  The authors stress the need for immediate factor VIII infusion and appropriate neurosurgical work-up. The rapid medical management is mandatory if morbidity and mortality are to be minimized.
authors.affiliation: Service de neurochirurgie, CHU d'Amiens, place Victor-Pauchet, 80054 Amiens cedex 1, France. peltier.johann@chu-amiens.fr
authors.forename: J
authors.initials: J
authors.lastname: Peltier
authors.forename: M
authors.initials: M
authors.lastname: Baroncini
authors.forename: L
authors.initials: L
authors.lastname: Thines
authors.forename: S
authors.initials: S
authors.lastname: Leteurtre
authors.forename: A
authors.initials: A
authors.lastname: Marey
authors.forename: M
authors.initials: M
authors.lastname: Vinchon
mesh_terms: D003399:Craniotomy / Q000009:adverse effects; D005169:Factor VIII / Q000235:genetics; D006467:Hemophilia A / Q000150:complications* / Q000175:diagnosis* / Q000601:surgery; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D020300:Intracranial Hemorrhages / Q000175:diagnosis / Q000209:etiology* / Q000401:mortality / Q000601:surgery; D008279:Magnetic Resonance Imaging; D008297:Male; D014057:Tomography, X-Ray Computed
chemical_list: D005169:Factor VIII