Hemofilia
DOI:
https://doi.org/10.29303/jku.v11i3.666Kata Kunci:
Hemofilia, perdarahan, faktor koagulasiAbstrak
Hemofilia merupakan penyakit turunan yang ditandai adanya pendarahan berat. Penyakit ini muncul sebagai perdarahan pasca trauma minor atau perdarahan spontan. Hemofilia disebabkan karena kelainan pada faktor protein koagulasi. Faktor VIII (FVIII) menyebabkan hemofilia A, dan faktor IX (FIX) menyebabkan hemofilia B. Hemofilia dapat didiagnosis melalui sekuensing gen dengan cara mengidentifikasi status pembawa. Pada saat prenatal, hemofila dapat dideteksi pada saat janin usia 12 minggu, tetapi memiliki resiko keguguran 0,5-1,0 %. Hemofilia diobati dengan dua pendekatan yakni pengelolaan pendarahan akut dan profilaksis. Pengobatan hemofilia harus memperhatikan dosis, jadwal, dan level target. Pengobatan hemofilia dapat mengunakan faktor VIII rekombinan dengan melibatkan inaktivasi virus dan teknologi skrining yang dinilai lebih aman. Komplikasi dapat terjadi karena kehadiran inhibitor penekan fungsi kerja faktor protein koagulasi, artropati hemofilik, dan pseudotumor pemicu pendarahan jaringan lunak yang berpotensi cedera organ dalam. Upaya pencegahan dilakukan dengan cara menjauhkan penderita dari aktivitas pemicu luka pendarahan fisik, serta memastikan penderita memiliki sistem kekebalan normal untuk menerima vaksinasi rutin.
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