Congenital factor XIII deficiency: comprehensive overview of the FranceCoag cohort.
epidemiology
factor XIII
prophylaxis
rare bleeding disorder
registry
Journal
British journal of haematology
ISSN: 1365-2141
Titre abrégé: Br J Haematol
Pays: England
ID NLM: 0372544
Informations de publication
Date de publication:
01 2020
01 2020
Historique:
received:
13
04
2019
accepted:
13
06
2019
pubmed:
16
8
2019
medline:
6
8
2020
entrez:
16
8
2019
Statut:
ppublish
Résumé
This FranceCoag network study assessed 33 patients with congenital factor XIII (FXIII) deficiency presenting FXIII levels <10 iu/dl. Diagnosis was based on abnormal bleeding in 29 patients, a positive family history in 2, recurrent miscarriages in 1 and was fortuitous in 1. Eighteen patients (62·1%) presented life-threatening umbilical or intracranial haemorrhages (ICH). Seven of the 15 patients who experienced ICH were diagnosed but untreated, including 3 with secondary neurological sequelae. All pregnancies without prophylaxis (26/26) led to miscarriages versus 3/16 with prophylaxis. In patients exhibiting FXIII levels <10 iu/dl, prophylaxis could be discussed at diagnosis and at pregnancy. Further controlled prospective studies are needed.
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
317-320Informations de copyright
© 2019 British Society for Haematology and John Wiley & Sons Ltd.
Références
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