Rare coagulation disorders: A retrospective analysis of 156 patients in Turkey
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Dosyalar
Tarih
2012
Dergi Başlığı
Dergi ISSN
Cilt Başlığı
Yayıncı
Turkish Journal of Hematology
Erişim Hakkı
info:eu-repo/semantics/openAccess
Özet
Öz: 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.
Abstract: Objective: To retrospectively evaluate the clinical findings, laboratory data, management, and outcome in a group of Turkish children diagnosed with rare coagulation deficiencies (RCDs) between January 1999 and June 2009. Material and Methods: The Turkish Society of Pediatric Hematology-Hemophilia-Thrombosis-Hemostasis subcommittee designed a Microsoft Excel-based questionnaire for standardized data collection and sent it to participating institutions. Results: In total, 156 patients from 12 pediatric referral centers were included in the study. The cost common RCDs were as follows: FVII (n = 53 [34%]), FV (n = 24 [15.4%]), and FX (n = 23 [14.7%]) deficiency. The most common initial finding 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 were the most commonly used treatments. We think that prophylactic treatment used for hemophilia patients should be considered as an initial therapeutic option for patients with rare factor deficiencies and a severe clinical course, and for those with a factor deficiency that can lead to severe bleeding.
Abstract: Objective: To retrospectively evaluate the clinical findings, laboratory data, management, and outcome in a group of Turkish children diagnosed with rare coagulation deficiencies (RCDs) between January 1999 and June 2009. Material and Methods: The Turkish Society of Pediatric Hematology-Hemophilia-Thrombosis-Hemostasis subcommittee designed a Microsoft Excel-based questionnaire for standardized data collection and sent it to participating institutions. Results: In total, 156 patients from 12 pediatric referral centers were included in the study. The cost common RCDs were as follows: FVII (n = 53 [34%]), FV (n = 24 [15.4%]), and FX (n = 23 [14.7%]) deficiency. The most common initial finding 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 were the most commonly used treatments. We think that prophylactic treatment used for hemophilia patients should be considered as an initial therapeutic option for patients with rare factor deficiencies and a severe clinical course, and for those with a factor deficiency that can lead to severe bleeding.
Açıklama
Yıl: 2012Cilt: 29Sayı: 1ISSN: 1300-7777 / 1308-5263Sayfa Aralığı: 48 - 54Metin Dili:İngilizce
Anahtar Kelimeler
Kaynak
Turkish Journal of Hematology
WoS Q Değeri
Q4
Scopus Q Değeri
Cilt
29
Sayı
1
Künye
FIŞGIN T,BALKAN C,CELKAN T,KILINÇ Y,TÜRKER M,TİMUR Ç,GÜRSEL T,KÜREKÇİ E,DURU F,KÜPESİZ O. A,OLCAY L,YILMAZ Ş,ÖZGEN Ü,ÜNÜVAR A,ÖREN H,KAVAKLI K (2012). Rare coagulation disorders: A retrospective analysis of 156 patients in Turkey. Turkish Journal of Hematology, 29(1), 48 - 54.
