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Abstrak :
Latar belakang: Pasien Talasemia Mayor (TM) anak menderita defisiensi nutrisi karena asupan nutrisi yang tidak mencukupi. Penghindaran makanan kaya zat besi seringkali bersamaan dengan pembatasan asupan protein. Asupan mikronutrien termasuk magnesium lebih rendah dibandingkan anak normal. Fungsi otot lebih awal terganggu akibat defisiensi nutrisi daripada massa otot. Penilaian massa otot dan Hand Grip Strength (HGS) menjadi penting untuk mengevaluasi status gizi. Hingga saat ini belum ada penelitian di Indonesia yang mengevaluasi hubungan antara HGS dengan asupan kalori, protein dan magnesium, LILA dan massa otot pasien anak TM. Metode: Penelitian dengan desain studi potong lintang melibatkan 70 pasien TM anak, berusia 6-18 tahun di Pusat Talasemia RSUPN Cipto Mangunkusumo. Status gizi dievaluasi disertai pengukuran lingkar lengan atas (LILA). Asupan kalori, protein dan magnesium diperoleh melalui metode analisis diet semi-kuantitatif Food Frequency Questionnaires (FFQ) dan Magnesium FFQ (MgFFQ). Kadar Mg serum dinilai dengan menggunakan metode enzimatik-kalorimetri. Massa otot diukur menggunakan Bioelectrical Impedance Analysis (BIA) dan HGS dinilai menggunakan Dinamometer tangan Jamar Hasil: Status gizi berdasarkan LILA/U sebagian besar berstatus gizi baik 42,9% dan malnutrisi 57,1% yakni gizi kurang (30,0%), gizi buruk (25,7%), dan obesitas (1,4%). Rerata kecukupan energi pada anak TM lelaki 100% (SB 17), sedangkan anak perempuan sebesar 112% (SB 27). Rerata asupan protein dan magnesium pada kedua kelompok lebih tinggi dibanding kebutuhan AKG. HGS berkorelasi kuat dengan massa otot (r=0,82), berkorelasi sedang dengan LILA (r=0,60), dan berkorelasi lemah dengan asupan kalori (r=-0,27), protein (r=-0,33) dan magnesium (r=-0,23), serta kadar magnesium (r=0,26). Hipermagnesemia dijumpai pada 23% subyek penelitian. Simpulan: Lebih dari separuh anak Talasemia mengalami malnutrisi walaupun asupan cukup. HGS berkorelasi dengan asupan nutrisi, LILA, dan massa otot. ......Background: Pediatric Thalassemia Major (TM) patients suffer from nutritional deficiencies due to insufficient nutritional intake. Avoidance of iron-rich foods often coincides with limiting protein intake. Micronutrient intake including magnesium is lower than in normal children. Muscle function is impaired earlier due to nutritional deficiencies than muscle mass. Assessment of muscle mass and Hand Grip Strength (HGS) is important for evaluating nutritional status. Until now there has been no research in Indonesia that evaluates the relationship between HGS and calorie, protein, and magnesium intake, LILA, and muscle mass in pediatric TM patients. Methods: This research with a cross-sectional study design involved 70 pediatric TM patients, aged 6-18 years at the Thalassemia Center of RSUPN Cipto Mangunkusumo. Nutritional status is evaluated by measurement of mid-upper arm circumference (MUAC). Calorie, protein, and magnesium intake was obtained through semi- quantitative dietary analysis methods Food Frequency Questionnaires (FFQ) and Magnesium FFQ (MgFFQ). Serum Mg levels were assessed using the enzymatic calorimetric method. Muscle mass was measured using Bioelectrical Impedance Analysis (BIA) and HGS was assessed using a Jamar hand dynamometer. Results: Nutritional status based on LILA/U was mostly good nutritional status 42.9% and malnutrition 57.1%, namely undernutrition (30.0%), poor nutrition (25.7%), and obesity (1.4%). The average energy adequacy for TM boys is 100% (SD 17), while for girls it is 112% (SD 27). The average intake of protein and magnesium in both groups was higher than the RDA requirements. HGS is strongly correlated with muscle mass (r=0.82), moderately correlated with LILA (r=0.60), and weakly correlated with calorie intake (r=-0.27), protein (r=-0.33), and magnesium (r=-0.23), as well as magnesium levels (r=0.26). Hypermagnesemia was found in 23% of study subjects. Conclusion: More than half of Thalassemia children experience malnutrition despite adequate intake. HGS correlates with nutritional intake, MUAC, and muscle mass.

Abstrak :
Latar belakang: Pasien Talasemia Mayor (TM) anak menderita defisiensi nutrisi karena asupan nutrisi yang tidak mencukupi. Penghindaran makanan kaya zat besi seringkali bersamaan dengan pembatasan asupan protein. Asupan mikronutrien termasuk magnesium lebih rendah dibandingkan anak normal. Fungsi otot lebih awal terganggu akibat defisiensi nutrisi daripada massa otot. Penilaian massa otot dan Hand Grip Strength (HGS) menjadi penting untuk mengevaluasi status gizi. Hingga saat ini belum ada penelitian di Indonesia yang mengevaluasi hubungan antara HGS dengan asupan kalori, protein dan magnesium, LILA dan massa otot pasien anak TM. Metode: Penelitian dengan desain studi potong lintang melibatkan 70 pasien TM anak, berusia 6-18 tahun di Pusat Talasemia RSUPN Cipto Mangunkusumo. Status gizi dievaluasi disertai pengukuran lingkar lengan atas (LILA). Asupan kalori, protein dan magnesium diperoleh melalui metode analisis diet semi-kuantitatif Food Frequency Questionnaires (FFQ) dan Magnesium FFQ (MgFFQ). Kadar Mg serum dinilai dengan menggunakan metode enzimatik-kalorimetri. Massa otot diukur menggunakan Bioelectrical Impedance Analysis (BIA) dan HGS dinilai menggunakan Dinamometer tangan Jamar. Hasil: Status gizi berdasarkan LILA/U sebagian besar berstatus gizi baik 42,9% dan malnutrisi 57,1% yakni gizi kurang (30,0%), gizi buruk (25,7%), dan obesitas (1,4%). Rerata kecukupan energi pada anak TM lelaki 100% (SB 17), sedangkan anak perempuan sebesar 112% (SB 27). Rerata asupan protein dan magnesium pada kedua kelompok lebih tinggi dibanding kebutuhan AKG. HGS berkorelasi kuat dengan massa otot (r=0,82), berkorelasi sedang dengan LILA (r=0,60), dan berkorelasi lemah dengan asupan kalori (r=-0,27), protein (r=-0,33) dan magnesium (r=-0,23), serta kadar magnesium (r=0,26). Hipermagnesemia dijumpai pada 23% subyek penelitian. Simpulan: Lebih dari separuh anak Talasemia mengalami malnutrisi walaupun asupan cukup. HGS berkorelasi dengan asupan nutrisi, LILA, dan massa otot. ......Background: Pediatric Thalassemia Major (TM) patients suffer from nutritional deficiencies due to insufficient nutritional intake. Avoidance of iron-rich foods often coincides with limiting protein intake. Micronutrient intake including magnesium is lower than in normal children. Muscle function is impaired earlier due to nutritional deficiencies than muscle mass. Assessment of muscle mass and Hand Grip Strength (HGS) is important for evaluating nutritional status. Until now there has been no research in Indonesia that evaluates the relationship between HGS and calorie, protein, and magnesium intake, LILA, and muscle mass in pediatric TM patients. Methods: This research with a cross-sectional study design involved 70 pediatric TM patients, aged 6-18 years at the Thalassemia Center of RSUPN Cipto Mangunkusumo. Nutritional status is evaluated by measurement of mid-upper arm circumference (MUAC). Calorie, protein, and magnesium intake was obtained through semi- quantitative dietary analysis methods Food Frequency Questionnaires (FFQ) and Magnesium FFQ (MgFFQ). Serum Mg levels were assessed using the enzymatic calorimetric method. Muscle mass was measured using Bioelectrical Impedance Analysis (BIA) and HGS was assessed using a Jamar hand dynamometer. Results: Nutritional status based on LILA/U was mostly good nutritional status 42.9% and malnutrition 57.1%, namely undernutrition (30.0%), poor nutrition (25.7%), and obesity (1.4%). The average energy adequacy for TM boys is 100% (SD 17), while for girls it is 112% (SD 27). The average intake of protein and magnesium in both groups was higher than the RDA requirements. HGS is strongly correlated with muscle mass (r=0.82), moderately correlated with LILA (r=0.60), and weakly correlated with calorie intake (r=-0.27), protein (r=-0.33), and magnesium (r=-0.23), as well as magnesium levels (r=0.26). Hypermagnesemia was found in 23% of study subjects. Conclusion: More than half of Thalassemia children experience malnutrition despite adequate intake. HGS correlates with nutritional intake, MUAC, and muscle mass.

Abstrak :
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.

Abstrak :
Latar Belakang: Pencapaian target transfusi darah pada pasien thalassemia beta bergantung transfusi dipengaruhi oleh berbagai faktor diantaranya adalah genotip, hipersplenisme, kompatibilitas darah, kecukupan darah donor dan interval transfusi. Ukuran limpa dapat dijadikan salah satu indikator keberhasilan pencapaian target transfusi darah selain kadar hemoglobin. Tujuan: Mengetahui proporsi pasien yang mencapai target optimal kadar hemoglobin pra dan pascatransfusi, menentukan faktor-faktor yang terkait dengan pencapaian target kadar hemoglobin pra dan pascatransfusi dan menilai hubungan antara pencapaian target kadar hemoglobin pra dan pascatransfusi dengan ukuran limpa pada pasien dewasa thalassemia beta bergantung transfusi. Metode: Penelitian cohort retrospective dengan pengambilan 200 subjek secara total sampling pada pasien dewasa rawat jalan Poliklinik thalassemia Rumah Sakit Cipto Mangunkusumo. Data dianalisis dari 110 subjek berupa anamnesis, pemeriksaan fisis dan laboratorium. Hasil: Sebanyak 200 pasien thalassemia beta bergantung transfusi yang rutin kontrol ke poliklinik thalassemia Kiara RSCM, diikuti secara kohort sejak bulan Juni 2017 sampai Juni 2018. 110 subjek penelitian memenuhi kriteria inklusi diantaranya subjek thalassemia beta mayor 53 (48,2%) dan beta HbE bergantung transfusi 57 (51,8%). Proporsi subjek yang mencapai target kadar Hb pratransfusi yaitu 18 (16,4%) dan 22 (20,0%) subjek yang mencapai target Hb pasca. Sebanyak 8 (7,3%) subjek mencapai target kadar Hb pra dan pascatransfusi darah. Faktor kecukupan darah donor berhubungan dengan pencapaian target kadar Hb pra dan pascatransfusi (p=0,008) yaitu subjek yang hanya memiliki selisih permintaan darah < 30ml/KgBB/tahun. Pada 93 subjek penelitian tahap 2, didapatkan perbedaan bermakna antara kelompok yang tercapai kadar Hb pra dan pascatransfusi darah dengan yang tidak tercapai terhadap delta ukuran limpa (p <0,001). Simpulan: Faktor kecukupan darah donor berhubungan dengan pencapaian target kadar hemoglobin pra dan pascatransfusi. Pencapaian target kadar hemoglobin pra dan pascatransfusi berhubungan dengan ukuran limpa. ......Background: Achieving the target of blood transfusion in transfusion-dependent beta thalassemia patients is influenced by various factors including genotype, hypersplenism, blood compatibility, donor blood adequacy and transfusion interval. The size of the spleen can be one indicator of the success of achieving blood transfusion targets in addition to hemoglobin levels. Objective: Determine the proportion of patients who achieve the optimal target hemoglobin level pre and post transfusion, determine the factors that are related to achieving pre and post transfusion hemoglobin levels and assess the relationship between achieving pre and post transfusion hemoglobin levels with spleen size in adult beta thalassemia transfusion dependent patients. Methods: A cohort retrospective study, with total sampling of 200 adult thalassemia transfusion dependent patient at Cipto Mangunkusumo Hospital. Data taken from 110 eligible subject in the form of medical history, physical examination and laboratory. Result: 200 transfusion-dependent beta thalassemia patients who routinely visit the RSCM thalassemia Kiara polyclinic, followed in cohort from June 2017 to June 2018. 110 study subjects fulfilled the inclusion criteria including 53 (48.2%) major beta thalassemia subjects and transfusion-dependent HbE beta 57 (51.8%). The proportion of subjects who achieved pre-transfusion Hb target levels was 18 (16.4%) and 22 (20.0%) subjects who achieved the post Hb target. A total of 8 (7.3%) subjects achieved pre and post transfusion Hb levels. The donor blood adequacy factor is related to the achievement of pre and post transfusion Hb target levels (p = 0.008), namely subjects who only have a blood demand difference of <30ml/KgBB/year. In 93 research subjects, there was a significant difference between groups who achieved pre and post-transfusion Hb levels with those that were not reached against the delta of spleen size (p <0.001). Conclusion: Adequacy factor of donors blood is related to achieving target pre and post transfusion hemoglobin levels. The achievement of the target pre and post transfusion hemoglobin levels is related to the size of the spleen.