Electronic Theses and Dissertation

Latar Belakang: Talasemia β mayor merupakan kelainan genetik akibat defek pada gen β-globin yang menyebabkan anemia kronis. Penatalaksanaan utama adalah transfusi darah rutin yang dapat menyebabkan akumulasi zat besi berlebih (hemosiderosis), sehingga diperlukan terapi kelasi besi untuk mengurangi dampaknya. Namun, terapi kelasi besi seperti Deferasirox (DFX) dan Deferiprone (DFP) berpotensi menyebabkan gangguan pendengaran. Oleh karena itu, deteksi dini gangguan pendengaran penting untuk mencegah dampak permanen.
Tujuan: Mengetahui hasil pemeriksaan audiometri nada murni pada anak dengan
talasemia β mayor yang menerima terapi kelasi besi DFX dan DFP.
Metode: Penelitian analitik observasional dengan desain potong lintang dilakukan pada anak dengan talasemia β mayor yang menjalani terapi kelasi besi DFX dan DFP di Poliklinik talasemia RSUD dr. Zainoel Abidin Banda Aceh pada Juli-Desember 2024. Pengambilan sampel menggunakan teknik total sampling.
Hasil: Jumlah sampel yang didapatkan yaitu 36 sampel yaitu perempuan 55.6% dan laki- laki 44.4% dengan usia rata-rata 14 tahun,, usia rata-rata saat didianosis talasemia β mayor 3 tahun, usia rata-rata pertama kali menggunakan terapi kelasi besi DFX atau DFP 5 tahun dan mayoritas hasil pemeriksaan pendengaran normal pada kedua telinga. Hasil analisis menunjukkan subjek penelitian yang menerima terapi kelasi besi DFP menunjukkan dosis yang lebih besar dengan durasi yang lebih singkat. Rerata dosis bulanan terapi kelasi besi DFX berhubungan terbalik dengan hasil audiometri dimana peningkatan dosis bulanan akan dapat menyebabkan penurunan dari intensitas pada beberapa frekuensi pada telinga kiri saja (500 Hz, dan 1000 Hz), namun tidak pada frekuensi yang lain (r=-0.385, p

Background: β major talasemia is a genetic disorder caused by a defect in the β-globin gene that causes chronic anemia. The main management is routine blood transfusion which can lead to excess iron accumulation (hemosiderosis), so iron chelation therapy is needed to reduce its impact. However, iron chelation therapies such as Deferasirox (DFX) and Deferiprone (DFP) have the potential to cause hearing loss. Therefore, early detection of hearing loss is important to prevent permanent effects.TalasemiaHb Objective: Knowing the results of pure tone audiometry examination in children with talasemia β major who received DFX and DFP iron chelation therapy. Methods: An observational analytic study with a cross-sectional design was conducted on children with talasemia β major who underwent DFX and DFP iron chelation therapy at the talasemia Polyclinic of RSUD dr. Zainoel Abidin Banda Aceh in July-December 2024. Sampling using total sampling technique. talasemiaTalasemia Results: The number of samples obtained was 36, consisting of 55.6% females and 44.4% males with an average age of 14 years, an average age of 3 years at diagnosis of major β thalassaemia, an average age of 5 years at first use of DFX or DFP iron chelation therapy, and the majority of hearing test results were normal in both ears. The analysis results showed that study subjects receiving iron chelation therapy with DFP had higher doses with shorter durations. The average monthly dose of iron chelation therapy with DFX was inversely correlated with audiometric results, where an increase in the monthly dose could cause a decrease in intensity at certain frequencies (500 Hz and 1000 Hz) in the left ear only, but not at other frequencies (r = -0.385, p < 0.05). The average monthly dose of DFP iron chelation therapy was inversely related to intensity at specific frequencies (1000 Hz and 2000 Hz) in the right ear (r = -0.334, p < 0.05). The duration of iron chelation therapy with DFX and DFP was not associated with a decrease or increase in intensity values at all frequencies in both ears (p>0.05). Discussion: The dosage of DFP and DFX iron chelation therapy was weakly correlated with pure tone audiometry results. The duration of DFP and DFX iron chelation therapy was not associated with pure tone audiometry results. This was because the subjects did not follow the dosage of DFP and DFX iron chelation therapy prescribed by the health insurance system. However, regular pure-tone audiometry examinations are necessary for children with β-thalassemia major receiving iron chelation therapy with DFX and DFP, especially before it is administered as a screening procedure for the prevention of hearing disorders. Keywords: β-major thalassemia, transfusion, iron chelation, Deferasirox, Deferiprone