Year: 2012
Type: article
Study design: Observational Study
Abstract: 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.
Source: Turkish Journal of Hematology
Institutions: Ondokuz Mayıs University, Ege University, Istanbul University-Cerrahpaşa, Istanbul University, Cukurova University, Izmir Tepecik Eğitim ve Araştırma Hastanesi, Medeniyet Üniversitesi Göztepe Eğitim ve Araştırma Hastanesi, Gazi University, Gülhane Askerî Tıp Akademisi, Akdeniz University, Ankara Dr. Abdurrahman Yurtaslan Oncology Training and Research Hospital, Dokuz Eylül University, Inonu University
Language: English
Subfield: Hematology
Field: Medicine
Domain: Health Sciences
Open Access status: diamond
OpenAlex ID: W2046242521