title: The rare inherited coagulation disorders.
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.
authors.affiliation: University of Manchester, Manchester Blood Centre, Plymouth Grove, Manchester, UK. paula.bolton-maggs@manchester.ac.uk
authors.forename: Paula H B
authors.initials: PH
authors.lastname: Bolton-Maggs
mesh_terms: D025861:Blood Coagulation Disorders, Inherited / Q000175:diagnosis / Q000453:epidemiology* / Q000235:genetics*; D001780:Blood Coagulation Tests; D005167:Factor VII / Q000235:genetics; D005169:Factor VIII / Q000235:genetics; D005170:Factor X / Q000235:genetics; D005172:Factor XI / Q000235:genetics; D005176:Factor XIII / Q000235:genetics; D005260:Female; D006470:Hemorrhage; D006801:Humans; D008297:Male
chemical_list: D005167:Factor VII; D005169:Factor VIII; D005170:Factor X; D005172:Factor XI; D005176:Factor XIII