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"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.

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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."

"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 JamarHasil: 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.

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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."

"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."

"ABSTRAKLatar belakang: Thalassemia merupakan kelainan genetik terbanyak di dunia, termasuk Indonesia. Pasien thalassemia mayor berisiko mengalami gangguan fungsi neurokognitif akibat anemia kronik dan penumpukan besi. Tujuan: mengetahui prevalens abnormalitas hasil EEG dan tes IQ, menganalisis faktor-faktor yang diduga berhubungan dengan gangguan fungsi neurokognitif pada anak dengan thalassemia mayor usia saat diagnosis, lama transfusi, pendidikan pasien, rerata Hb pra-transfusi, kadar feritin serum, saturasi transferin, dan komplians terhadap obat kelasi besi , serta untuk mengetahui apakah gangguan neurokognitif dapat memengaruhi fungsi sekolah. Metode: Penelitian potong lintang deskriptif analitik antara April 2016-April 2017. Pengukuran tes IQ menggunakan WISC-III. Hasil: Total subyek adalah 70 anak thalassemia mayor berusia antara 9 hingga 15,5 tahun. Prevalens hasil EEG abnormal adalah 60 dan prevalens skor IQ abnormal

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ABSTRACTBackground Thalassemia is the most common hereditary disorders worldwide, including Indonesia. Chronic anemia and iron overload in thalassemia major lead to several risk factors including neurocognitive problems. Aim To investigate the prevalence of abnormal EEG and IQ test, to identify the factors related to neurocognitive function in children with thalassemia major age at diagnosis, years of transfusion, patients education, pre transfusion haemoglobin level, ferritin, transferrin saturation, and compliance to chelation , and to identify whether neurocognitive dysfunction affects child rsquo s school performance. Methods A cross sectional descriptive analitic study. Subjects were recruited from April 2016 April 2017. Cognitive function assessed by the WISC III. Results A total 70 children aged from 9 to 15.5 years old were recruited. The prevalence of abnormal EEG and abnormal IQ score "

"Latar belakang dan tujuan: Thalassemia adalah penyakit anemia hemolitik yang diturunkan, merupakan penyakit genetik yang paling sering di dunia. Transfusi secara berkala pada pasien thalassemia dapat menyebabkan deposit besi pada berbagai organ seperti hipofisis. Deposit besi pada hipofisis dapat menyebabkan hipogonadotropik hipogonadisme. Biopsi merupakan pemeriksaan baku emas untuk menilai deposit besi pada organ, namun hal ini tidak dapat dilakukan pada hipofisis. Pemeriksaan MRI mulai digunakan unutuk mengukur kadar besi pada berbagai organ salah satunya hipofisis.Metode: Uji korelasi dengan pendekatan potong lintang untuk mengetahui nilai korelasi nilai MRI T2 dan T2 relaksometri serta SIR T2 hipofisis dengan kadar FSH dan LH pada pasien thalassemia mayor. Pemeriksaan dilakukan 28 subjek penelitian dalam kurun waktu Desember 2016 hingga Maret 2016.Hasil: Terdapat korelasi antara nilai relaksometri T2 hipofisis potongan koronal dengan kadar FSH dan LH, serta terdapat pula korelasi antara nilai SIR T2 hipofisis dengan kadar LH. Tidak terdapat korelasi antara nilai relaksometri T2 potongan koronal-sagital dengan kadar FSH dan LH, serta tidak terdapat pula korelasi antara SIR T2 hipofisis dengan kadar FSH.Kesimpulan: Nilai relaksometri T2 hipofisis potongan koronal dan SIR T2 hipofisis dapat digunakan sebagai acuan deposit besi pada hipofisis serta dapat memonitor terapi kelasi pada pasien thalassemia - mayor.

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Background and abjective Thalassemia is a hereditary hemolytic anemia disorder, it is one of the most common genetic disease in the world. Periodic transfusion for thalassemia patients may lead to iron deposit in various organs such as pituitary gland. Iron deposit in pituitary gland may induce hypogonadotropic hypogonadism. Biopsy and histopathology assessment is the gold standard examination to assess organ iron deposit, however this method is inapplicable for pituitary gland. MRI examination has been started to be used for measurement of iron level in various organ, such as pituitary gland.

Method: This study uses cross sectional method. MRI T2 and T2 relaxometry value as well as SIR T2 of pituitary gland was correlated with FSH and LH level in patients with major thalassemia. This study involves 28 subjects and conducted from December 2016 to March 2017.

Result: There is a correlation between relaxometry values of T2 pituitary gland on coronal slice with the level of FSH dan LH. There is also a correlation between pituitary SIR T2 value with the level of LH. There are no correlation between relaxometry values of T2 on coronal sagittal slices with the level of FSH and LH, furthermore there are no correlation between pituitary SIR T2 with FSH level.

Conclusion: Relaxometry value of pituitary T2 on coronal slice and pituitary SIR T2 value may be use as reference for iron deposit on pituitary gland as well as to monitor chelating therapy in major thalassemia patients."

"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.

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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."

"Latar belakang: Thalassemia merupakan kelainan genetik yang paling banyak ditemukan di seluruh dunia. Penyakit ini dapat menimbulkan berbagai masalah dan kelainan berbagai organ tubuh, termasuk pada rongga mulut. Tujuan: memperoleh gambaran mengenai kelainan yang terjadi pada rongga mulut pasien thalassemia mayor di Pusat Thalassemia RSCM. Metode: Penelitian cross-sectional terhadap 76 pasien thalassemia mayor yang berusia diatas 12 tahun. Data didapat dengan melakukan pemeriksaan klinis dan wawancara terstruktur menggunakan panduan kuesioner. Hasil: Keluhan subyektif dalam rongga mulut yang sering dialami adalah: serostomia, diikuti dengan sariawan berulang, bibir mengelupas dan pecah-pecah, serta gusi berdarah. Prevalensi kelainan klinis yang ditemukan meliputi: inkompetensi bibir (25,0%); malokusi: klas I (40,79%), klas II (51,32%) dan klas III (3,95%); higiene oral buruk (67,11%), dan gingivitis (82,89%). Nilai rata-rata DMF-T adalah 4,97. Kondisi dan lesi patologik mukosa mulut yang paling banyak ditemukan adalah pigmentasi mukosa (69,74%), diikuti dengan depapilasi lidah (56,58%), mukosa ikterik (52,63%), cheilosis/cheilitis (50,0%), mukosa pucat (44,74%), erosi/deskuamasi mukosa (44,74%), stomatitis aftosa rekuren (15,79%), glositis defisiensi (14,47%) dan perdarahan gingiva (11,84%). Kesimpulan: Maloklusi, higiene oral buruk, gingivitis, serostomia, pigmentasi mukosa, depapilasi lidah, mukosa ikterik, dan cheilosis/cheilitis, merupakan masalah yang paling umum ditemukan pada pasien thalassemia mayor dalam penelitian ini, namun indeks karies gigi terlihat rendah.

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Background: Thalassemia is the most common genetic disorders worldwide. The disease can cause various problems and disorders of various organs of the body, including in the oral cavity.

Objective: to describe the oral cavity disorders in patients with major thalassemia in Thalassemia Centre at Cipto Mangunkusumo Hospital.

Methods: cross-sectional study involved 76 patients with major thalassemia over 12 years of age. Data obtained by clinical examination and structured interviews using guidance from quistionnare.

Results: Oral subjective symptom which is often experienced is xerostomia, followed by recurrent aphthous stomatitis, cheilosis/cheilitis, and gingival bleeding. Prevalence of clinical findings consist of: incompetence of lips (25%); malocclusion: class I (40,79%), class II (51,32%) and class III (3,94%); poor oral hygiene (67,11), gingivitis (82,89%). DMF-T score was 4,97. Conditions and pathologic lesions more frequently seen are pigmentation of mucosa (69,74%), followed by depapillation of tongue (56,58%), icterus of mucosa (52,63%), cheilosis/cheilitis (50%), pallor of mucosa (44,74%), erosion/desquamation of mucosa (44,74%), recurrent aphthous stomatitis (15,79%), glossitis deficiency (14,47%), and gingival bleeding (11,84%).

Conclusion: Malocclusion, poor oral hygiene, gingivitis, xerostomia, pigmentation of mucosa, depapillation of tongue, icterus of mucosa, and cheilosis/cheilitis, were most prevalent problems in patients with major thalassemia in this study; nevertheless, dental caries show low index."

"ABSTRAK Latar belakang. Kelasi besi diduga berperan terhadap penurunan fungsi ginjal pada pasien thalassemia mayor. Data fungsi ginjal pasien thalassemia mayor yang menggunakan kelasi besi oral di Jakarta masih terbatas. Tujuan. Mengetahui penurunan fungsi ginjal pasien thalassemia mayor yang mendapat kelasi besi oral dan faktor yang memengaruhinya. Metode penelitian. Penelitian dilakukan bulan Maret ndash; Juli 2017 pada pasien thalassemia mayor yang mendapat kelasi besi oral tunggal selama minimal 1 tahun. Fungsi ginjal dinilai dengan laju filtrasi glomerulus berdasarkan formula Schwartz revisi Fungsi tubulus ginjal dinilai dengan peningkatan rasio kalsium kreatinin urin hiperkalsiuria . Hasil penelitian. Total subjek sebanyak 54 orang 28 deferipron, 26 deferasiroks . Proporsi LFG menurun pada kelompok deferipron lebih tinggi dibandingkan deferasiroks 53,6 vs 46,2 . Hiperkalsiuria lebih banyak ditemukan pada kelompok deferasiroks dibandingkan deferipron 12,9 vs 3,6 . Penurunan LFG bermakna pada kelompok deferipron tetapi tidak bermakna pada kelompok deferasiroks. Tidak terdapat perbedaan bermakna LFG dan rasio kalsium kreatinin urin antara kelompok deferipron vs deferasiroks p=0,427; p=0,109 . Usia, hemoglobin, rerata hemoglobin, feritin, dosis kelasi besi dan saturasi transferin hanya memengaruhi fungsi tubular ginjal. Simpulan. Terdapat penurunan fungsi ginjal pada pasien thalassemia mayor yang mendapatkan kelasi besi oral. Fungsi ginjal pada thalassemia perlu dinilai berkala meski penurunannya tidak bermakna secara klinis.Kata kunci: Thalassemia, fungsi ginjal, kelasi besi oralABSTRACT Background. Iron chelator can cause renal dysfunction in thalassemia major patients. Data of renal function in thalassemia major patients who receive oral iron chelator are limited. Objective. To determine kidney dysfunction in thalassemia major patients receiving oral iron chelator and its correlating factors. Methods. The study was conducted in March ndash July 2017 on thalassemia major patients treated with single oral iron chelator for at least 1 year. Renal function determined by glomerular filtration rate measured with revised Schwartz formula. Tubular function determined by increased urine calcium creatinine ratio hypercalciuria . Results. Total subjects were 54 28 deferiprone, 26 deferasirox . Proportion of decreased GFR in deferipron group was higher than deferasirox 53,6 vs 46,2 . Hypercalciuria was higher in deferasirox group than deferiprone 12,9 vs 3.6 . Declining of GFR was significant in deferiprone group but not significant in deferasirox group. There was no significant difference of GFR and urinary creatinine calcium ratio in deferiprone vs deferasirox group p 0.427 p 0.109 . Age, hemoglobin level, mean hemoglobin, ferritin, iron chelator dose and transferrin saturation only affecting kidney tubular function. Conclusions. Renal dysfunction was found in thalassemia major patients receiving oral iron chelator. Kidney function in thalassemia major patients should be monitored periodically eventhough the decline was not significant. Keywords Thalassemia, renal function, oral iron chelator"

"Latar Belakang: Talasemia merupakan penyakit kelainan hemoglobin (Hb) dengan prevalensi tinggi di Indonesia maupun dunia. Komplikasi pada talasemia dapat terjadi akibat kadar Hb pre-transfusi yang rendah dan penumpukan feritin serum. Pengetahuan, sikap, dan perilaku yang baik terhadap suatu penyakit dibutuhkan untuk mencapai kesehatan yang diinginkan dan mencegah komplikasi. Studi ini bertujuan untuk mencari hubungan antara pengetahuan, sikap, dan perilaku terhadap talasemia, Hb pre-transfusi dan kadar feritin serum pada pasien remaja talasemia karena mereka memiliki prevalensi tertinggi. Metode: Kuesioner pengetahuan, sikap, dan perilaku (PSP) melalui google form disebarkan untuk mendapatkan data dari pasien talasemia remaja yang memenuhi kriteria studi. Pengetahuan akan dibagi menjadi adekuat atau tidak adekuat, sikap dibagi menjadi positif atau negatif, perilaku dibagi menjadi baik atau buruk berdasarkan hasil skor kuesioner. Kadar Hb pre-transfusi dan feritin serum diambil dari rekam medik pasien, dan dikelompokkan menjadi Hb dan serum ferritin yang tinggi atau rendah. Hasil Penelitian: Dari 85 subjek, terdapat 49.4% pasien dengan pengetahuan adekuat, 91.8% pasien dengan sikap positif, dan 72.9% pasien dengan perilaku baik. Pasien masih kurang memahami fasilitas skrining dan pentingnya suplementasi vitamin. Pasien perlu meningkatkan sikap positif terhadap skrining thalassemia dan perilaku baik terhadap kepatuhan obat. Terdapat hubungan yang tidak bermakna secara statisik antara pengetahuan, sikap dan perilaku terhadap kadar Hb pre-transfusi dan kadar ferritin (p >0.05) pada remaja dengan talasemia. Kesimpulan: Remaja talasemia di Rumah Sakit Cipto Mangunkusumo memiliki pengetahuan yang tidak adekuat, namun dengan sikap dan perilaku yang baik. Perlu adanya edukasi berkala untuk meningkatkan pengetahuan.

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Introduction: Thalassemia is a hemoglobin (Hb) disorder that has a high prevalence in Indonesia and the world. Complications in thalassemia can occur due to low pre-transfusion Hb and accumulation of serum ferritin. A good knowledge, attitude, and practice towards a disease are needed to achieve desired health outcomes and prevent complications. This study aims to find the relationship between knowledge, attitude, and practice towards thalassemia, pre-transfusion Hb, and serum ferritin levels in thalassemic adolescents as they have the highest prevalence. Methods: Knowledge, attitude, and practice (KAP) questionnaire through google form were distributed to adolescent thalassemic patients who met the criteria. Knowledge will be divided into adequate or inadequate; attitudes are divided into positive or negative; practice is divided into good or bad based on the questionnaire results. Level of Pre-transfusion Hb and serum ferritin were taken from the patient's medical record and grouped into high or low Hb and ferritin. Result: Out of 85 subjects, there were 49.4% patients with adequate knowledge, 91.8% patients with positive attitude, and 72.9% patients with good practice. Patients still lack understanding of screening facilities and the importance of vitamin supplementation. Patients need to increase positive attitude towards thalassemia screening and good behavior towards treatment adherence. There was a statistically insignificant relationship between knowledge, attitude, and practice on thalassemia with pre-transfusion Hb and serum ferritin (p > 0.05) in thalassemic adolescents. Conclusion: Thalassemic adolescents at Cipto Mangunkusumo Hospital have inadequate knowledge, but with good attitudes and behavior. Periodic education is needed to increase knowledge."

"Di Indonesia, thalassemia mayor merupakan salah satu masalah kesehatan karena morbiditas dan mortalitasnya yang tinggi. Thalassemia mayor ditandai dengan anemia berat sejak usia anak-anak dan memerlukan transfusi teratur untuk mempertahankan kadar hemoglobin. Untuk mengurangi kebutuhan akan transfusi darah, dilakukan splenektomi. Trombosis merupakan salah satu komplikasi thalassemia yang banyak dilaporkan di berbagai negara, tetapi di Indonesia sampai saat ini belum ada laporan. Trombosit dan sistem koagulasi memegang peranan dalam patogenesis trombosis. Tujuan penelitian ini adalah untuk mendapatkan gambaran mengenai kelainan trombosit serta aktivasi koagulasi pada penderita thalassemia mayor yang sudah maupun yang belum di-splenektomi di Indonesia.Desain penelitian ini potong lintang. Subyek penelitian terdiri dari 31 orang penderita thalassemia mayor yang sudah displenektomi (kelompok splenektomi) dan 35 orang penderita thalassemia mayor yang belum mengalami splenektomi (kelompok nonsplenektomi). Untuk menilai fungsi trombosit, dilakukan pemeriksaan agregasi trombosit terhadap adenosin difosfat (ADP), aktivasi trombosit dinilai dengan mengukur kadar β-tromboglobulin (β-TG), sedangkan aktivasi koagulasi dinilai dengan pemeriksaan D-dimer.Hasil penelitian ini menunjukkan bahwa jumlah trombosit pada kelompok splenektomi Iebih tinggi secara bermakna dibandingkan kelompok non-splenektomi (549.260+251.662/μI vs 156.000/μl (kisaran 34.000-046.000/μl); p<0,001). Demikian pula agregasi trombosit terhadap ADP 1 pM maupun 10 pM Iebih tinggi secara bermakna pada kelompok splenektomi dibandingkan dengan kelompok non-splenektomi (1 pM: 17,3% (kisaran 1,9-104,0%) vs 5,2% (kisaran 0,5-18,2%); p <0,001 dan 10 pM: 91,2% (kisaran 27,3-136,8%) vs 55,93 + 17,27%; p<0,001). Kadar β-TG Iebih tinggi secara bermakna pada kelompok splenektomi dibandingkan kelompok non-splenektomi (178,81 + 86,3 IU/ml vs 100,11 + 40,0 IU/ml; p<0,001). Kadar D-dimer juga Iebih tinggi secara bermakna pada kelompok splenektomi dibandingkan non-splenektomi walaupun keduanya masih dalam rentang normal (0,2 μg/ml (kisaran 0,1-0,7 g/ml) vs 0,1 μg/ml (kisaran 0,1-0,8 μg/mI).Dari hasil penelitian ini, disimpulkan bahwa pada penderita thalassemia mayor di Indonesia terdapat jumlah trombosit dan fungsi agregasi yang bervariasi, sedangkan aktivasi trombosit meningkat, tetapi belum dapat dibuktikan adanya aktivasi koagulasi. Pada penderita thalassemia mayor yang sudah displenektomi didapatkan trombositosis, serta agregasi trombosit terhadap ADP dan aktivasi trombosit yang Iebih tinggi dibandingkan dengan penderita yang belum di-splenektomi.

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Thalassemia major is one of the health problem in Indonesia due to its high morbidity and mortality. Thalassemia major is characterized by severe anemia presenting in the first years of life and requires regular transfusions to maintain hemoglobin level. Splenectomy is performed to decrease the need for transfusion. Thrombosis is one of the complications widely reported in patients with thalassemia in many parts of the world, but until now, there had been no report on this complication in Indonesia. Platelet and the coagulation system play a role in the pathogenesis of thrombosis. The aim of this study was to obtain the pattern of changes in platelet count, function and activation level, and activation of coagulation in patients with thalassemia major patients in Indonesia.The design of this study was cross-sectional. The subjects were 31 splenectomized and 35 non-splenectomized patients with thalassemia major. Platelet aggregation to adenosine diphosphate (ADP) was performed to assess platelet function; β-thromboglobulin level was used as marker of platelet activation, and D-dimer for activation of coagulation.The result of this study revealed a significantly higher platelet count in splenectomized compared to non-splenectomized patients (549.260 + 251.86210 vs-156.000/μl (34.000- 46.000/μl); p<0.001). Platelet aggregation to ADP were significantly higher in splenectomized patients than non-splenectomized group, both to 1 pM (17.3% (range 1.9-104M%) vs 5.2% (range 0.5-118.2%); p<0.001) and 10 μM ADP (91.2% (range 27.3-136.8%) vs 55.93 + 17.27%; p<0.001). β-thromboglobulin level was significantly higher in splenectomized patients compared to non-splenectomized patients (178.81 + 86.3 IU/rnl vs 100.11 + 40.0 IU/ml; p<0.001). D-dimer level was also significantly higher in the splenectomized group compared to non-splenectomized group although both had values within normal range (0.2 pglml (range 0.1-0.7 μg/mI) vs 0.1 pg1ml (range 0.1-0.8 μg/ml).We concluded that the platelet count and function were varied, while platelet activation level was increased in patients with thalassemia major in Indonesia, but activation of coagulation was not established. We also concluded that in splenectomized patients there were thrombocytosis and increased platelet aggregation to ADP and platelet activation level compared to non-splenectomized patients."