Abstract |
The rare inherited coagulation disorders (RICD) are uncommon and thus not well-defined in terms of severity or management. Inheritance is autosomal; in some of these disorders in the heterozygote state affected individuals may be mildly symptomatic. Severe deficiencies are more common in association with consanguinity. Factor X and factor XIII deficiency have the most severe manifestations, while factor XI deficiency is the least severe. Factor VII and factor XI deficiencies show a poor relationship between the factor level and bleeding risk. Unlike hemophilia, women are equally affected by these RICD and can have problems related to menstruation and childbirth.
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Authors | Paula H B Bolton-Maggs |
Journal | Pediatric blood & cancer
(Pediatr Blood Cancer)
Vol. 60 Suppl 1
Pg. S37-40
( 2013)
ISSN: 1545-5017 [Electronic] United States |
PMID | 23109366
(Publication Type: Journal Article, Review)
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Copyright | Copyright © 2012 Wiley Periodicals, Inc. |
Chemical References |
- Factor VII
- Factor VIII
- Factor X
- Factor XI
- Factor XIII
|
Topics |
- Blood Coagulation Disorders, Inherited
(diagnosis, epidemiology, genetics)
- Blood Coagulation Tests
- Factor VII
(genetics)
- Factor VIII
(genetics)
- Factor X
(genetics)
- Factor XI
(genetics)
- Factor XIII
(genetics)
- Female
- Hemorrhage
- Humans
- Male
|