title: [Severe congenital factor V deficiency: case report].
abstract: BACKGROUND
  Congential deficiency of factor V is a rare condition, transmitted in autosomal recessive way. Heterozygote patients generally have no symptoms, homozygotes present with spontaneous and postoperative bleedings. About one-half  of patients are diagnosed in adulthood.
  
  
  METHODS
  The presented case is a double heterozygote, which was confirmed using moleculare biological methods. The authors found many carriers in his family.
  
  
  CONCLUSIONS
  This rare and sometimes severe disorder needs regular haematological controls. Carriers must have more attention during surgical procedures and labour, and the family doctor should be informed about there condition.
authors.affiliation: Fovárosi Onkormányzat Heim Pál Gyermekkórház, Budapest, Hematológiai Osztály.
authors.forename: Marosi
authors.initials: M
authors.lastname: Anikó
authors.forename: Apjok
authors.initials: A
authors.lastname: Eniko
authors.forename: Magyarosy
authors.initials: M
authors.lastname: Edina
authors.forename: Szabó
authors.initials: S
authors.lastname: Teréz
authors.forename: Ajzner
authors.initials: A
authors.lastname: Eva
mesh_terms: D001780:Blood Coagulation Tests; D003937:Diagnosis, Differential; D005166:Factor V Deficiency / Q000097:blood / Q000151:congenital* / Q000175:diagnosis*; D006579:Heterozygote; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D010375:Pedigree