In:
British Journal of Haematology, Wiley, Vol. 188, No. 2 ( 2020-01), p. 317-320
Kurzfassung:
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.
Materialart:
Online-Ressource
ISSN:
0007-1048
,
1365-2141
Sprache:
Englisch
Verlag:
Wiley
Publikationsdatum:
2020
ZDB Id:
1475751-5
