RARE COAGULATION DISORDERS. Retrospective analyses of 156 patients in TURKEY
Makaleler > Tam Makale · 2012
Yazarlar: Tunc Fisgin, Can BALKAN, Tiraje Celkan, Yurdanur Kilinc, Meral Turker, Cetin Timur, Turkiz Gursel, Emin Kurekci, Feride Duru, Alphan Kupesiz, Lale Olcay, Sebnem Yilmaz, Unsal Ozgen, Aysegul Unuvar
Turkish Journal of Hematology, Uluslararası, Wos, 2012, cilt 29, ss 48-54
Özet
OBJECTIVE: To retrospectively evaluate the clinical findings, laboratory data, management, and outcome in a group ofTurkish children diagnosed with rare coagulation deficiencies (RCDs) between January 1999 and June 2009. MATERIAL AND METHODS: The Turkish Society of Pediatric Hematology-Hemophilia-Thrombosis-Hemostasissubcommittee designed a Microsoft Excel-based questionnaire for standardized data collection and sent it to participatinginstitutions. RESULTS: In total, 156 patients from 12 pediatric referral centers were included in the study. The cost common RCDswere as follows: FVII (n = 53 [34%]), FV (n = 24 [15.4%]), and FX (n = 23 [14.7%]) deficiency. The most common initialfinding in the patients was epistaxis, followed by ecchymosis, and gingival bleeding. CONCLUSION: Initial symptoms were mucosal bleeding, and fresh frozen plasma (FFP) and tranexamic acid werethe most commonly used treatments. We think that prophylactic treatment used for hemophilia patients should beconsidered as an initial therapeutic option for patients with rare factor deficiencies and a severe clinical course, and forthose with a factor deficiency that can lead to severe bleeding.