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• W2046242521 abstract "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.The Turkish Society of Pediatric Hematology-Hemophilia-Thrombosis-Hemostasissubcommittee designed a Microsoft Excel-based questionnaire for standardized data collection and sent it to participatinginstitutions.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.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.Amaç: Türkiye’de son 10 yılda nadir koagülasyon eksikliği olan çocukların klinik bulguları, laboratuvar değerleri ve tedavilerini geriye yönelik olarak değerlendirdik.Gereç ve Yöntemler: Türk Pediatrik Hematoloji Derneği, Tromboz-Hemostaz-Hemofili Alt Komitesi tarafından standardize edilmiş excel sorgulama dosyaları her merkeze gönderilerek yanıt veren merkezlerin verileri toplandı. Bulgular: Oniki çocuk hematoloji merkezinden toplam 156 hasta bu çalışmaya alındı. Çalışmamızda en sık görülen nadir koagülasyon bozuklukları FVII (n: 53 olgu, %34), FV (n: 24 olgu, %15.4), FX (n: 23 olgu, %14.7) olarak dağılım gösterdi. Tüm nadir koagülasyon bozuklukları içinde en sık görülen başlangıç semptomları sırası ile burun kanaması, ekimoz ve diş eti kanamasıydı. Sonuç: Mukozal kanamalar ilk semptomları oluştururken, halen en sık kullanılan tedavi seçenekleri ise taze donmuş plazma ve treneksamik asitti. Ağır klinik semptomları olan ve şiddetli kanamaya neden olabilecek nadir koagülasyon bozukluklarının erken dönemlerinde de hemofili hastalarında kullandığımız proflaktik tedavinin düşünülmesi gerektiğini öneriyoruz." @default.
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• W2046242521 date "2012-01-01" @default.
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• W2046242521 title "RARE COAGULATION DISORDERS. Retrospective analyses of 156 patients in TURKEY" @default.
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• W2046242521 doi "https://doi.org/10.5505/tjh.2012.02418" @default.
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