[Factor VII deficiency revealed by intracranial hemorrhage].
Constitutional factor VII deficiency is a hereditary disease with recessive autosomic transmission. Its incidence is estimated to be 1/1,000,000 in the general population. We report a case of severe factor VII deficiency in infancy revealed by an intracranial hemorrhage in a 2-month-old infant. We describe the clinical, biological and therapeutic characteristics of this disease.
Sfaihi Ben Mansour
L
L
Service de pédiatrie, CHU Hédi Chaker, 3029 Sfax, Tunisie. lamia_benmansour@yahoo.fr
Thabet
A
A
Aloulou
H
H
Turki
H
H
Chabchoub
I
I
Mhiri
F
F
Mnif
Z
Z
Ben Ali
H
H
Kammoun
T
T
Hachicha
M
M
D061085:Agenesis of Corpus Callosum; D000941:Antigens / Q000627:therapeutic use; D002552:Cerebral Ventricles / Q000473:pathology; D002869:Chromosome Aberrations; D003131:Combined Modality Therapy; D003241:Consanguinity; D003337:Corpus Callosum / Q000473:pathology; D004453:Echoencephalography; D005167:Factor VII / Q000627:therapeutic use; D005168:Factor VII Deficiency / Q000175:diagnosis* / Q000235:genetics; D005808:Genes, Recessive; D006580:Genetic Carrier Screening; D006801:Humans; D007223:Infant; D020300:Intracranial Hemorrhages / Q000209:etiology* / Q000235:genetics / Q000601:surgery; D008297:Male; D009778:Occipital Lobe / Q000473:pathology; D011994:Recombinant Proteins / Q000627:therapeutic use; D012008:Recurrence; D014057:Tomography, X-Ray Computed; D017287:Ventriculoperitoneal Shunt
D000941:Antigens; D011994:Recombinant Proteins; C042580:factor VII clotting antigen; D005167:Factor VII