| Literature DB >> 31414482 |
Séverine Bouttefroy1, Sandrine Meunier1, Vanessa Milien2, Mohamed Boucekine2, Pierre Chamouni3, Dominique Desprez4, Annie Harroche5, Audrey Hochart6, Marie Françoise Thiercelin-Legrand7, Bénédicte Wibaut6, Hervé Chambost2,8, Lucia Rugeri1.
Abstract
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.Entities:
Keywords: epidemiology; factor XIII; prophylaxis; rare bleeding disorder; registry
Mesh：
Year: 2019 PMID： 31414482 DOI： 10.1111/bjh.16133
Source DB: PubMed Journal: Br J Haematol ISSN： 0007-1048 Impact factor: 6.998