Congenital Factor XIII Deficiency With the Presence of Inhibitor: A Case Study.
J Pediatr Hematol Oncol
; 43(1): e99-e102, 2021 01.
Article
en En
| MEDLINE
| ID: mdl-31764516
ABSTRACT
Coagulation factor XIII (FXIII) is a fibrin-stabilizing factor with additional roles in wound healing and interactions between the decidua and fetus. Congenital FXIII deficiency is rare bleeding disorder. Inhibitor development against FXIII in inherited FXIII deficency is also uncommon, but may cause severe, life-threatening bleeding. FXIII is the last step in the coagulation cascade with normal coagulation paramaters (PT, aPTT), the detection of inhibitor to FXIII is quite difficult. The treatment of inhibitor-positive congenital FXIII deficiency is challenging due to the lack of a role of by-pass agents such as FVII. The best known ways of treatment in these cases are the use of high-dose FXIII concentrates and immunosuppression. Herein, we report the management of postoperative bleeding diathesis in a patient with FXIII deficiency who developed inhibitors, and to follow the clinical course of the disease with FXIII concentrate and immunosuppression.
Texto completo:
1
Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Factor XIII
/
Inhibidores de Factor de Coagulación Sanguínea
/
Deficiencia del Factor XIII
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Anticuerpos Neutralizantes
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Trastornos Hemorrágicos
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Inmunosupresores
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Isoanticuerpos
Tipo de estudio:
Etiology_studies
/
Prognostic_studies
Límite:
Child
/
Humans
/
Male
Idioma:
En
Revista:
J Pediatr Hematol Oncol
Asunto de la revista:
HEMATOLOGIA
/
NEOPLASIAS
/
PEDIATRIA
Año:
2021
Tipo del documento:
Article