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"Latar Belakang: Harapan hidup pasien thalasemia bergantung transfusi bertambah baik karena transfusi darah dan terapi kelasi besi yang sesuai. Penyakit jantung akibat toksisitas besi tetap menjadi penyebab utama kematian pada pasien thalasemia bergantung transfusi. MRI T2* jantung dapat mendeteksi dini toksisitas besi di jantung dan dapat mengevaluasi hasil pengobatan dengan membandingkan nilai T2* pra dan pasca terapi kelasi besi.Tujuan Penelitian: Penelitian ini bertujuan mendapatkan profil perbaikan toksisitas besi di jantung pada pasien thalasemia dewasa bergantung transfusi. Penelitian ini juga bertujuan untuk melihat kesesuaian antara perbaikan nilai T2* jantung dengan perbaikan feritin serum dan saturasi transferin.Metode Penelitian: pre and post test dengan data sekunder retrospektif pada pasien dewasa thalasemia bergantung transfusi yang kontrol di poliklinik thalasemia Kiara dan poliklinik dewasa hematologi-onkologi medik RSUPN Cipto Mangukusumo. Penelitian dilakukan pada bulan Juli-Desember 2019. Data sekunder diperoleh dari rekam medis dan registri pasien thalasemia berupa riwayat medis, jenis obat kelasi besi, nilai T2* jantung satu tahun berturut-turut, kadar feritin serum dan saturasi transferin. Analisis data berupa data deskriptif dan uji marginal homogeneity serta uji kappa.Hasil: Sebanyak 115 pasien dilibatkan dalam penelitian ini. Terdapat perbaikan T2* jantung sebanyak 7,0% dan menetap baik (T2* jantung tetap >20 milidetik) sebanyak 72,2%. Tidak terdapat kesesuaian antara perbaikan nilai T2* jantung dengan perbaikan feritin serum (nilai kappa = 0,044) dan perbaikan nilai T2* jantung dengan saturasi transferin ( nilai kappa = 0,011).Simpulan: Perbaikan toksisitas besi di jantung pasca terapi kelasi besi sebanyak 7,0% dan menetap baik sebanyak 72,2%. Tidak terdapat kesesuaian antara perbaikan nilai T2* jantung dengan perbaikan kadar feritin serum dan saturasi transferin.

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Background: Life expectancy of the transfusion dependent thalassemia patients is getting better because of blood transfusion and appropriate iron chelation therapy. Heart disease due to iron toxicity remains the leading cause of death in thalassemia patients who need transfusion. MRI T2* can allow to detect premature iron toxicity in the heart and can evaluate the results by comparing myocardial T2* pre and post iron chelation therapy.Objectives: This study aims to obtain a profile of improvement in cardiac iron toxicity in adult thalassemia patients who need transfusion. This study also supports to see aggrement between improvement in myocardial T2* with improved serum ferritin level and transferrin saturation.Methods: pre and post test with retrospective secondary data in adult thalassemia patients requiring controlled transfusions in Kiara thalassemia clinic and hematology-medical oncology clinic Cipto Mangukusumo General Hospital. The study was conducted in July-Desember 2019. Data were obtained from medical records and thalassemia registry, which consisted of medical history, type of chelation, myocardial T2* within one year, serum ferritin level and transferrin saturation. Data analysis was performed in descriptive data and marginal homogeneity test and Kappa test.Results: A total of 115 patients were included in this study. There was an improvement of a myocardial T2* in 7.0% patients and persistently good (myocardial T2* remains >20 milliseconds) in 72.2%. There was no agreement between improvement in myocardial T2* with improvement in serum ferritin level (kappa value 0.044) and improvement in myocardial T2* with transferrin saturation (kappa value 0.011).Conclusion: Improvement of cardiac iron toxicity after iron chelation therapy was 7.0% and persistently good in 72.2%. There was no agreement between the improvement in myocardial T2* with improvement in serum ferritin level and transferrin saturation."

"Pendahuluan: Beta thalassemia mayor (BTM) merupakan kelainan sintesis rantai beta globin yang menyebabkan penderitanya harus menjalani transfusi berulang untuk mempertahankan kadar hemoglobin. Hal tersebut dapat menyebabkan hemokromatosis di berbagai organ yang dapat menyebabkan komplikasi, termasuk diabetes melitus (DM). Terapi kelasi besi ditujukan untuk mengurangi hemokromatosis dan mencegah komplikasi, namun seringkali penderita tidak patuh. Tujuan dari penelitian ini adalah untuk mengetahui hubungan tingkat kepatuhan terapi kelasi terhadap kejadian DM pada pasien BTM. Metode: Penelitian ini merupakan penelitian dengan desain potong-lintang. Tingkat kepatuhan terapi kelasi ditentukan menggunakan Morisky Medication Adherence Scale – 8 (MMAS-8), sementara data pemeriksaan laboratorium terkait DM (kadar glukosa darah sewaktu, puasa, dan 2 jam postprandial) didapatkan dari rekam medis. Subjek penelitian adalah penderita BTM di Pusat Kesehatan Ibu & Anak (PKIA) Rumah Sakit Cipto Mangunkusumo (RSCM) Kiara yang bersedia mengisi kuesioner dan memiliki data pemeriksaan laboratorium terkait. Analisis data dilakukan dengan Uji One- Way ANOVA dan Uji T Tidak Berpasangan. Hasil: Dari 50 penderita BTM yang menjadi subjek penelitian, sebagian besar (74%) memiliki tingkat kepatuhan terapi kelasi yang rendah. Ditemukan 1 (2%) subjek yang memiliki kondisi prediabetes. Tidak ditemukan hubungan yang bermakna antara tingkat kepatuhan terapi kelasi terhadap kadar glukosa darah sewaktu (p = 0,843, n = 35), kadar glukosa darah puasa (p = 0,776, n = 17), maupun kadar glukosa darah 2 jam postprandial (p = 0,863, n = 17). Kesimpulan: Tingkat kepatuhan terapi kelasi tidak berhubungan dengan kejadian DM yang ditentukan melalui kadar glukosa darah sewaktu, puasa, dan 2 jam postprandial

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Introduction: Beta thalassemia major (BTM) is a disorder of beta globin chain synthesis that causes sufferers to undergo repeated transfusions to maintain hemoglobin levels. This can cause hemochromatosis, and in various organs can cause complications, including diabetes melitus (DM). Iron chelation therapy is intended to reduce hemochromatosis and prevent complications, but often sufferers do not comply. The purpose of this study was to determine the relationship of the level of adherence of chelation therapy to the occurence of DM in BTM patients. Method: This research is a cross-sectional study. The level of chelation therapy adherence was determined using the Morisky Medication Adherence Scale - 8 (MMAS-8), while laboratory examination data related to DM (random, fasting, and two hours post-prandial plasma glucose level) were obtained from medical records. The subjects were BTM sufferers at the Cipto Mangunkusumo Hospital (RSCM) Kiara Hospital who were willing to fill out questionnaires and have relevant laboratory examination data. Data analysis was performed with One-Way ANOVA and Independent T-Test. Results: From BTM sufferers who were the subjects of the study, the majority (74%) had a low level of chelation therapy adherence. One (2%) subject were found to have prediabetes. No significant relationship was found between the level of chelation therapy adherence to random blood glucose levels (p = 0.843, n = 35), fasting blood glucose levels (p = 0.776, n = 17), and 2 hours post-prandial blood glucose levels (p = 0.863 , n = 17). Conclusion: The level of chelation therapy adherence is not related to the occurence of DM which is determined through random, fasting, and two hours post- prandial plasma glucose level."

"Thalassemia merupakan penyakit hereditas kronis dengan prevalensi tinggi di Indonesia. Studi cross-sectional ini melibatkan 109 pasein thalassemia beta mayor berusia 10 – 18 tahun yang menjalani terapi kelasi besi di RSUPN dr. Cipto Mangunkusumo untuk menilai hubungan antara persepsi penyakit (B-IPQ) dan kepatuhan terapi kelasi besi (MMAS-8). Sebagian besar responden berusia 10 – 14 tahun (50,5%) dan didominasi oleh laki-laki (51,4%). Mayoritas responden memiliki rumah sendiri (57,8%). Status pekerjaan orang tua menunjukkan 94,5% ayah responden bekerja dan 63,3% ibu responden tidak bekerja. 52,3% responden mengalami thalassemia > 12 tahun. Kadar Hb pratransfusi < 9 g/dL dialami oleh 55% responden. Frekuensi transfusi darah terbanyak dilakukan ≥ 2 kali dalam sebulan oleh 70,6% responden. Mayoritas responden (85,3%) tidak dapat mengendalikan kadar feritin. Seluruh responden minum obat terapi kelasi secara oral. Hasil menunjukkan 70,6% responden memiliki persepsi penyakit positif dan 57,8% responden memiliki kepatuhan yang rendah. Uji Chi-square menunjukkan hubungan signifikan antara usia dan kepatuhan (p = 0,025), tetapi tidak ditemukan hubungan signifikan antara persepsi penyakit atau faktor demografis lainnya dengan kepatuhan (p > 0,05). Penelitian ini menunjukkan bahwa kepatuhan terapi kelasi semakin rendah seiring bertambahnya usia. Dengan demikian, orang tua perlu memahami cara berkomunikasi efektif dengan remaja. Perawat anak perlu memberikan intervensi yang menyasar efikasi dan manajemen diri pasien untuk meningkatkan kepatuhan.

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Thalassemia is a chronic hereditary disease with high prevalence in Indonesia. This cross-sectional study involved 109 beta-thalassemia major patients aged 10-18 years undergoing iron chelation therapy at Dr. Cipto Mangunkusumo National Central Public Hospital. The study aimed to assess the association between illness perception (B-IPQ) and adherence to iron chelation therapy (MMAS-8). The majority of respondents were aged 10-14 years (50.5%) and predominantly male (51.4%). Most respondents lived in their own homes (57.8%). Parental employment status indicated that 94.5% of fathers were employed, while 63.3% of mothers were unemployed. Furthermore, 52.3% of respondents had experienced thalassemia for more than 12 years. Pre-transfusion hemoglobin levels of < 9 g/dL were observed in 55% of respondents. The most frequent blood transfusions (≥ 2 times per month) were reported by 70.6% of respondents. A significant majority (85.3%) of respondents were unable to control their ferritin levels. All respondents received oral chelation therapy. Results showed that 70.6% of respondents had a positive illness perception, while 57.8% demonstrated low adherence to therapy. Chi-square tests revealed a significant association between age and adherence (p = 0.025), but no significant association was found between illness perception or other demographic factors and adherence (p > 0.05). This study indicates that adherence to chelation therapy tends to decrease with increasing age. Therefore, parents need to understand effective communication strategies with adolescents. Pediatric nurses should implement interventions targeting patients' self-efficacy and self-management skills to improve adherence."