title: Morbidity and mortality in a large number of Iranian patients with severe congenital factor XIII deficiency.
abstract: With 473 patients, Iran has about one third of the world's patients with severe congenital factor XIII (FXIII) deficiency. A considerable number of patients with FXIII deficiency (FXIIID) are affected by life-threatening bleeding episodes, such as central nervous system (CNS) bleeding or recurrent miscarriage and umbilical cord bleeding (UCB), that cause a high rate of morbidity and mortality in Iranian patients with FXIIID. Among 317 Iranian patients with FXIIID, 145 cases experienced 166 CNS bleeds (CNSBs) that recurred in 21 cases. CNSB caused different types of neurological complications in 69 patients. A total of 62 miscarriages were observed in 24 women of childbearing age, and 21 deaths were observed due to umbilical cord bleeding or mucosal bleeding. In fact, 49 deaths (15.4 %) were observed in these patients, which highlight the importance of early diagnosis and intensive health care among patients with FXIIID.
authors.affiliation: Department of Hematology and Blood Transfusion, School of Allied Medical Sciences, Iran University of Medical Sciences, Tehran, Iran. dorgalaleha@gmail.com.
authors.forename: Akbar
authors.initials: A
authors.lastname: Dorgalaleh
authors.affiliation: Department of Pediatrics Hematology & Oncology, Ali Ebn-e Abitaleb Hospital Research Center For Children And Adolescents Health [RCCAH], Zahedan University of Medical Sciences, Zahedan, Iran. naderifactorxiii@yahoo.com.
authors.forename: Majid
authors.initials: M
authors.lastname: Naderi
authors.affiliation: Department of Nursing and Midwifery, Shahroud University of Medical Sciences, Shahroud, Iran.
authors.forename: Morteza
authors.initials: M
authors.lastname: Shamsizadeh
mesh_terms: D000026:Abortion, Habitual / Q000175:diagnosis / Q000453:epidemiology / Q000401:mortality; D005177:Factor XIII Deficiency / Q000175:diagnosis* / Q000453:epidemiology / Q000401:mortality*; D005260:Female; D006470:Hemorrhage / Q000175:diagnosis / Q000453:epidemiology / Q000401:mortality; D006801:Humans; D007492:Iran / Q000453:epidemiology; D008297:Male; D009017:Morbidity; D011247:Pregnancy; D012720:Severity of Illness Index
keywords: Central nervous system bleeding; Factor XIII deficiency; Morbidity; Mortality