Dergiler / Fiziksel Tıp ve Rehabilitasyon Bilimleri Dergisi / 2019 / Cilt: 22 - Sayı: 2

The Evaluation of the Joint Status of Hemophiliac Adults: Single Center Adult Hematology Clinic Experience

Hemofilik Erişkinlerde Eklem Durumunun Değerlendirilmesi: Tek Merkez Erişkin Hematoloji Kliniği Deneyimi

Sayfa
48–52
DOI
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⚡ TL;DR(1 Cümlede Hızlı Çıkarım)

Prophylaxis in patients with severe hemophilia may be planned earlier because of the more frequent occurrence of target joint development.

📌 3 Maddede Bu Makale

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Amaç & Hipotez:Objective: Hereditary hemophilia A is the result of genetic alterations that cause deficiencies in clotting factor VIII, obstructing the process of hemostasis and predisposing hemophiliacs to spontaneous or post-traumatic bleeding.
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Temel Bulgular & Veri:Material and Methods: We analyzed the target joint of 33 patients followed as hemophilia A in adult hematology outpatient clinic between 2000 and 2018 years.
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Sonuç & Katkı:Prophylaxis in patients with severe hemophilia may be planned earlier because of the more frequent occurrence of target joint development.
🔬Tür:Araştırma Makalesi
📍Kapsam:2000 Verileri
⚙️Değişkenler:Alan Parametreleri

Özet

Objective: Hereditary hemophilia A is the result of genetic alterations that cause deficiencies in clotting factor VIII, obstructing the process of hemostasis and predisposing hemophiliacs to spontaneous or post-traumatic bleeding. The aim of this study was to identify the target joint conditions of adult hemophilia A patients followed in adult hematology outpatient clinic. Material and Methods: We analyzed the target joint of 33 patients followed as hemophilia A in adult hematology outpatient clinic between 2000 and 2018 years. Results: Knee joint was the predominant joint affected by hemarthrosis in 57.5% cases. Ankle joint was involved in 42.4% cases and elbow joint was involved in 27.2% cases. Hip joint and shoulder joint involvement was seen in 9% and 6% cases, respectively. 48.5% of hemophilia A patients had developed target joint. Knee joint was the predominant target joint in 56.2% cases and ankle joint was the target joint in 18.8% cases. In this study, a positive association was observed between inhibitor presence and target joint development. Inhibitor was detected in 5 of 6 patients who developed target joint. Conclusion: In conclusion, the most affected joints were knee, ankle and elbow joints. Prophylaxis in patients with severe hemophilia may be planned earlier because of the more frequent occurrence of target joint development.

Abstract

Amaç: Kalıtsal hemofili A, pıhtılaşma faktörü VIII eksikliğine yol açan genetik değişikliklerin sonucudur, hemostaz sürecini engeller ve hastalarda spontan veya travma sonrası kanamaya sebep olur. Bu çalışmanın amacı, erişkin hematoloji polikliniğinde izlenen erişkin hemofili A hastalarının hedef eklem durumlarını değerlendirmektir. Gereç ve Yöntemler: Çalışmada, 2000-2018 yılları arasında Hacettepe Üniversitesi Hastanesi’ndeki erişkin hematoloji polikliniğinde hemofili A ile takip edilen 33 hastanın hedef eklemi değerlendirildi. Bulgular: Diz eklemi, %57,5 olguda hemartrozdan en sık etkilenen eklemdi. Ayak bileği eklemi %42,4 olguda, dirsek eklemi %27,2 olguda etkilenmişti. Kalça eklemi ve omuz eklemi tutulumu sırasıyla %9 ve %6 olguda saptandı. Hemofili A hastalarının %48,5'inde hedef eklem gelişti. Diz eklemi %56,2 olguda en sık izlenen hedef eklemdi ve %18,8 olguda ise ayak bileği eklemi hedef eklemdi. Bu çalışmada, inhibitör varlığı ve hedef eklem gelişimi arasında pozitif bir ilişki olduğu gözlendi. Hedef eklem gelişen 6 hastanın 5’inde inhibitör saptandı. Sonuç: Sonuç olarak en fazla etkilenen eklemler diz, ayak bileği ve dirsek eklemleri idi. Ağır hemofili hastalarında profilaksi, hedef eklem gelişiminin daha sık görülmesi nedeniyle daha erken planlanabilir.

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Bu Makaleye Atıf Yap

ÇİFTÇİLER, R., AKSU, S. (2019). The Evaluation of the Joint Status of Hemophiliac Adults: Single Center Adult Hematology Clinic Experience. Fiziksel Tıp ve Rehabilitasyon Bilimleri Dergisi, Cilt: 22 - Sayı: 2, 48–52. https://ajindex.com/articles/160367

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Kaynakça (16)

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