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ABSTRAK
Latar belakang: Kardiomiopati akibat kelebihan besi masih merupakan penyebab kematian utama pada pasien thalassemia b mayor. Kardiomipati akibat kelebihan besi merupakan penyakit yang reversibel dengan pemberian kelasi besi yang ditandai dengan gejala awal gangguan diastolik. Amino-terminal pro-brain natriuretic peptide NT-proBNP merupakan biomarker yang sensitif dalam mendeteksi disfungsi diastolik.Tujuan: Mengevaluasi nilai diagnostik NT-proBNP dalam mendeteksi hemosiderosis jantung yang dinilai dengan MRI T2 jantung.Metode: Enam puluh delapan pasien dengan thalassemia b mayor usia 10-18 tahun tanpa gejala gagal jantung menjalani pemeriksaan NT-proBNP saat sebelum transfusi darah. Semua subyek diperiksa MRI T2 Jantung dalam kurun waktu maksimal 3 bulan median 19 hari . Pasien kemudian dibagi menjadi kelompok hemosiderosis jantung MRI T2 jantung 20 ms .Hasil: Dari 68 pasien, didapatkan rasio lelaki : perempuan sebesar 1: 1,1 dengan usia median 14,1 tahun rentang : 10-17,8 tahun . Kadar NT-proBNP tidak berbeda bermakna antara kelompok hemosiderosis jantung dan tidak hemosiderosis jantung p-0,233 . Uji diagnosis NT-proBNP dengan nilai titik potong 160 pg/mL menghasilkan nilai sensitivitas, spesifisitas, nilai duga postif, dan nilai duga negatif secara berurutan sebesar 38,46 , 58,1 , 17,8 , dan 50 .Simpulan: Pengukuran NT-proBNP tidak dapat digunakan untuk mendeteksi hemosiderosis jantung pada anak. ABSTRACT
Background Iron induced cardiomyopathy remains the leading cause of mortality in patients with thalassemia b major. Iron deposition related cardiomiopathy, which may be reversible through iron chelation, is characterized by early diastolic dysnfunction. Amino terminal pro brain natriuretic peptide NT proBNP is a sensitive biomarker of diastolic dysfunction.Aim To evaluate the diagnostic value of NT proBNP as a surrogate marker of iron overload examined with MRI T2 .Methods sixty eight b thalassemia major patients 10 18 years with no signs of heart failure underwent NT proBNP measurement before routine transfusion. All subjects were prospectively performed cardiac MRI T2 examination within three months median 19 days . Patients were divided as cardiac hemosiderosis cardiac MRI T2 20 ms Result Of 68 patients, the male to female ratio was 1 1,1 and the median age was 14.1 years range 10 17.8 years . NT proBNP levels were not different between hemosiderosis and non hemosiderosis p 0,233 . Diagnosis test using cut off value of 160 pg mL resulted in sensitivity of 38.46 , specificity of 58.1 , positive predictive value of 17.8 , and negative predictive value of 50 .Conclusion NT proBNP cannot be used to detect cardiac hemosiderosis in adolescent.

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Penelitian ini bertujuan untuk mengetahui keluhan subjektif mata kering dan gangguan komponen air mata (lipid, akuos, mucin) pada pasien thalassemia mayor dengan riwayat transfusi darah jangka panjang, serta menganalisis hubungan antara kadar feritin serum, durasi, dan frekuensi transfusi darah dengan masing-masing parameter penilaian komponen lapisan air mata. Penelitian ini merupakan studi potong lintang (cross sectional) pada pasien thalassemia mayor yang sudah berusia dan mengalami transfusi darah selama minimal 10 tahun. Penilaian mata kering terdiri dari pengisian kuesioner OSDI untuk menilai keluhan subjektif, pemeriksaan biomikroskopi lampu celah dan nilai tear break up time (TBUT) untuk menilai tingkat keparahan mata kering, pemeriksaan Schirmer basal, Ferning, dan sitologi impressi konjungtiva untuk menghitung jumlah sel goblet. Data perhitungan tingkat keparahan mata kering, nilai uji Schirmer basal, TBUT, dan jumlah sel goblet dianalisis dan dicari hubungannya dengan kadar feritin serum, durasi dan frekuensi transfusi. Pada 77 subyek, mata kering terjadi sebanyak 14.3%, penurunan nilai TBUT (39%), nilai Schirmer basal (37.7%), nilai Ferning (24.7%), dan jumlah sel goblet (45.5%). Tidak terdapat perbedaan bermakna antara tingkat keparahan mata kering, nilai TBUT, nilai Schirmer basal, nilai Ferning, dan jumlah sel goblet dengan kadar feritin serum, durasi, dan frekuensi transfusi. Namun, terdapat hubungan yang bermakna antara tingkat keparahan mata kering dan usia (p = 0.014), serta nilai TBUT (p = 0.012) dan Schirmer (p = 0.014) dengan jenis kelamin. Penelitian ini memperlihatkan 14.3% subyek thalassemia mayor mengalami mata kering berdasarkan kriteria DEWS 2007. Kejadian mata kering pada thalassemia mayor tidak dipengaruhi oleh faktor transfusi dan kadar feritin serum, melainkan dipengaruhi oleh usia dan jenis kelamin. ......This study is aimed to understand subjective complaints for dry eyes and disruption of component of tear fluid (lipid, aqueous, mucin) in patients with major thalassemia with a history of long-term blood transfusions and to analyse the correlation between serum ferritin level, duration and frequency of blood transfusion. This study is a cross-sectional study. The subject of this study is patients with major thalassemia age minimum of 10 years old and have had blood transfusion for at least 10 years. OSDI questionnaire, slit-lamp biomicroscopy examination, tear break up time (TBUT), and basal Schirmer test was used to assess dry eyes severity. Ferning and conjunctiva impression cytology examination was used to assess mucin quality and count the amount of goblet cells. The correlation analysis between the result of these assessments and serum ferritin level and duration and frequency of blood transfusion was done. In 77 subjects, the prevalence of dry eyes is 14.3%. There is a decrease in TBUT (39%), basal Schirmer (37.7%), Ferning (24.7%), and goblet cells (45.5%). There is no significant correlation between dry eyes severity and TBUT, basal Schirmer, Ferning, and the amount of goblet cells with serum ferritin level, duration, and frequency of blood transfusion. There is a significant correlation between dry eyes severity and patient s age (p = 0.014); TBUT (p = 0.012), as well as, Schirmer (p = 0.014) with sex. This study showed that 14.3% of patients with major thalassemia suffer from dry eyes with severity level grade 2 according to DEWS 2007. The incidence of dry eyes is not influenced by transfusion and serum ferritin level but is influenced by age and sex.

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ABSTRAK
Indonesia tergolong dalam negara sabuk thalassemia yang memiliki prevalensi thalassemia yang tinggi. Thalassemia β mayor dan β-HbE merupakan dua jenis thalassemia yang paling banyak ditemukan di Indonesia. Studi-studi sebelumnya menemukan bahwa thalassemia menyebabkan gangguan pertumbuhan, di antaranya berupa perawakan pendek dan abnormalitas proporsi tubuh. Belum ada penelitian di Indonesia yang mencari perbedaan kejadian perawakan pendek dan proporsi tubuh antara anak thalassemia β mayor, β-HbE, dan normal usia prepubertal. Penelitian ini dilakukan dengan desain potong-lintang dengan menyertakan sampel berusia 5-12 tahun sebanyak 130 orang. Prevalensi perawakan pendek pada anak thalassemia β mayor ditemukan sebesar 34,0, pada anak thalassemia β-HbE sebesar 24,3, dan pada anak normal sebesar 9,4. Tidak terdapat perbedaan rasio segmen atas terhadap segmen bawah pada anak thalassemia β mayor, β-HbE, dan kontrol. Rerata rasio rentang lengan terhadap tinggi badan lebih pendek pada anak thalassemia β mayor dan β-HbE dibanding pada anak normal. Jenis kelasi, usia diagnosis, durasi sakit, frekuensi transfusi, rerata Hb pretransfusi dalam 6 bulan terakhir, dan rerata ferritin dalam 6 bulan terakhir tidak berbeda antara anak thalassemia yang pendek dan yang tidak pendek.

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ABSTRACT
Indonesia belongs to thalassemia belt countries which has high prevalence of thalassemia. major and HbE thalassemia are the two most common types of thalassemia found in Indonesia. Previous studies have found that thalassemia causes growth disorders, including short stature and abnormalities of body proportions. There have been no studies in Indonesia that looked for differences in the incidence of short stature and body proportion between β major and β-HbE thalassemia and normal children in prepubertal age. This research was conducted as cross sectional study towards 130 children with age of 5 12. The prevalence of short stature was found to be 34.0 in major thalassemia, 24.3 in HbE thalassemia, and 9.4 in normal children. There was no difference in upper segment to lower segment ratio between major and HbE thalassemia and control. Arm range to stature ratio is shorter in β major and β-HbE thalassemia than control. Types of chelating agent, age of diagnosis, duration of sickness, frequency of transfusion, mean pretransfusion Hb in the last 6 months, and mean ferritin in the last 6 months did not differ between short and not short thalassemia children.

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Transfusi darah berulang pada subjek thalassemia mayor berpotensi menyebabkan transmisi virus hepatitis B dan / atau C. Infeksi dapat menyebabkan perubahan kadar feritin serum. Penelitian ini bertujuan untuk mengetahui prevalensi infeksi varian virus hepatitis dan hubungannya dengan kadar feritin serum. Penelitian potong-lintang dilakukan dengan membandingkan kadar feritin serum antar kelompok subjek terinfeksi varian virus hepatitis pada subjek thalassemia mayor di RS Cipto Mangunkusumo Kiara Jakarta antara tahun 2006-2015. Hasil penelitian menyebutkan bahwa prevalensi infeksi hepatitis keseluruhan sebesar 10,06 subjek dan didapatkan nilai p < 0,050 dari uji komparasi antara kadar feritin serum pada kelompok subjek hepatitis B dengan hepatitis C, hepatitis B dengan hepatitis B dan C, hepatitis C dengan non-hepatitis serta hepatitis B dan C dengan non-hepatitis. Pevalensi infeksi hepatitis keseluruhan pada penelitian ini lebih rendah dibandingkan dengan penelitian lain dan terdapat hubungan yang bermakna antara kadar feritin serum pada kelompok subjek hepatitis B dengan hepatitis C, hepatitis B dengan hepatitis B dan C, hepatitis C dengan non-hepatitis serta hepatitis B dan C dengan non-hepatitis. ...... Regular blood transfusion in major thalassemia subjects potentially mediates infection of hepatitis B and or C virus. Infection can change serum ferritin level. This research intends to know the prevalence of hepatitis virus variant infection and its association with serum ferritin level. This research used cross sectional method to compare serum ferritin level within each hepatitis virus variant infection subject's groups on major thalassemia subjects in RS Cipto Mangunkusumo Kiara Jakarta within 2006 2015. Results showed that prevalence of hepatitis in total was 10.06 subjects and p value from comparison test of serum ferritin level in subject's group of hepatitis B with hepatitis C, hepatitis B with hepatitis B and C, hepatitis C with non hepatitis, and hepatitis B and C with non hepatitis is p 0,050. Prevalence of hepatitis in total was lower than prevalence value in the other studies and there were significant association of serum ferritin level in subject's group of hepatitis B with hepatitis C, hepatitis B with hepatitis B and C, hepatitis C with non hepatitis, and hepatitis B and C with non hepatitis.

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Pendahuluan: Beta thalassemia mayor merupakan salah satu penyakit genetik yang mengharuskan penderitanya mendapatkan transfusi rutin seumur hidup untuk menunjang fungsi darah yang hilang. Transfusi yang dilakukan memiliki efek samping berupa peningkatan kadar besi dalam tubuh pasien (hemokromatosis) yang dapat menimbulkan berbagai komplikasi, salah satunya penurunan produksi hormon gonadotropin yang berujung pada keterlambatan pubertas. Terapi kelasi dapat dilakukan untuk menurunkan kadar besi dalam tubuh pasien dalam rangka mencegah terjadinya komplikasi, akan tetapi masalah kepatuhan dapat menghalangi keberhasilan terapi. Penelitian ini bertujuan untuk mengetahui hubungan antara kepatuhan terapi kelasi besi pada pasien beta thalassemia mayor dengan kadar hormon LH. Metode: Penelitian ini menggunakan desain potong lintang dengan data kepatuhan terapi kelasi diukur menggunakan Morisky Medication Adherence Scale 8 (MMAS-8) dan data laboratorium (kadar LH) diperoleh dari rekam medis. Subjek penelitian merupakan pasien dengan beta thalassemia mayor di Pusat Kesehatan Ibu dan Anak Rumah Sakit Dr. Cipto Mangunkusumo Kiara PKIA RSCM Kiara yang memiliki data laboratorium berupa kadar LH. Pengolahan data dilakukan dengan menggunakan uji Mann-Whitney, Kruskal Wallis, dan Spearman. Hasil: Dari 39 sampel yang valid, 84,6% subjek memiliki tingkat kepatuhan rendah. Sebanyak 28,2% subjek memiliki kadar LH di bawah batar normal. Tidak ditemukan hubungan signifikan anatara tingkat kepatuhan terapi kelasi dengan kadar LH pada subjek (p = 0,151, n = 39). Kesimpulan: Tidak ada hubungan signifikan antara kepatuhan terapi kelasi besi dengan penurunan kadar LH.

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Latar belakang dan tujuan : Hemosiderosis jantung dan pankreas merupakan komplikasi transfusi pada pasien thalassemia mayor. Evaluasi hemosiderosis pankreas dan jantung dilakukan dengan pemeriksaan MRI sekuens T2*. Kedua organ tersebut mempunyai kesamaan dalam penyimpanan besi dan tehnik pemeriksaan T2* pankreas lebih mudah dan cepat dibandingkan tehnik pemeriksaan jantung, sehingga diharapkan evaluasi hemosiderosis jantung dipermudah dengan menghitung nilai T2* pankreas. Metode : Uji korelatif dengan pendekatan potong lintang pada nilai T2* pankreas dan nilai T2* jantung dihitung menggunakan perangkat lunak CMRtools™ pada 30 subjek thalassemia mayor. Hasil : Tidak terdapat korelasi antara nilai T2* pankreas dengan nilai T2* jantung (R = 0,05, p = 0,798). Kesimpulan : Tidak terdapat korelasi antara nilai T2* pankreas dengan nilai T2* jantung pada pasien thalassemia mayor. ......Background and objective : Cardiac and pancreatic hemosiderosis are transfusion complication in major thalassemia patients. Evaluation of cardiac and pancreatic hemosiderosis assessed by MRI T2* examination. Both organs have same iron deposition, pancreatic T2* examination easier and faster than cardiac. Pancreatic T2* score can be used to evaluate cardiac hemosiderosis. Method : A cross sectional correlation study between pancreatic and cadiac T2*score calculated with CMRtools™ software conducted in 30 major thalassemia patients. Result : There is no correlation between pancreatic and cardiac T2* score (R = 0,05, p = 0, 79) Conclusion : There is no correlation between pancreatic and cardiac T2* score in major thalassemia patients.

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[ABSTRAK
Latar Belakang: Infeksi merupakan penyebab kematian yang penting pada thalassemia. Peningkatan risiko infeksi disebabkan oleh banyak faktor antara lain karena kelebihan besi dan splenektomi. Penelitian ini bertujuan mengetahui perbedaan fungsi fagositosis monosit pada pasien thalassemia mayor pasca splenektomi dan non splenektomi serta mengetahui hubungan fungsi fagositosis monosit dengan kadar feritin serum. Metode: Penelitian dilakukan di Departemen Patologi Klinik RSCM, Jakarta pada September 2013 ? Februari 2014. Desain penelitian potong lintang, dengan subjek penelitian pasien thalassemia mayor, terdiri dari 58 subjek pasca splenektomi dan 58 subjek non splenektomi yang telah dilakukan macthing umur dan jenis kelamin. Dilakukan pemeriksaan fagositosis monosit menggunakan E.coli yang telah diopsonisasi dan dilabel FITC sebagai target, (PhagotestTM) dan diperiksa dengan flow cytometry BD FACSCalibur. Kadar feritin serum diperiksa dengan Cobas e 601. Hasil: Median fagositosis monosit pada 58 subjek pasca splenektomi 5,03 (0,17 ? 22,79) %, dan pada 58 subjek non splenektomi 7,09 (0,11 ? 27,24) %, dan nilai p > 0.05. Kadar feritin serum pada subjek pasca splenektomi 6.724 (644,60 ? 21.835) ng/mL dan subjek non splenektomi 4.702,50 (1.381 ? 14.554) ng/mL, dan nilai p < 0.05. Hasil uji korelasi fungsi fagositosis monosit dengan kadar feritin didapatkan r = 0.13 (nilai p = 1.00). Kesimpulan: Tidak terdapat perbedaan bermakna antara fungsi fagositosis monosit pada pasien thalassemia mayor pasca splenektomi dan non splenektomi. Kadar feritin serum pada pasien thalassemia mayor pasca splenektomi lebih tinggi secara bermakna dibandingkan non splenektomi. Tidak didapatkan hubungan antara fagositosis monosit dengan kadar feritin serum.

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ABSTRACT
Background : Infection is an important cause of death in thalassemia. Increase of risk of infection could be due to iron overload and post-splenectomy. The study aimed to determine the difference of phagocytosis function of monocyte between post-splenectomized and non- splenectomized patients with thalassemia major, and the correlation of phagocytosis function of monocyte and serum ferritin level. Methods : The study was conducted in Department of Clinical Pathology Cipto Mangunkusumo hospital, Jakarta, in September 2013 ? Februari 2014. It was a cross sectional study. The study subjects consisted of 58 post-splenectomized patients and 58 non-splenectomized patients with age and sex matching. Phagocytosis function of monocyte was determined using E.coli opsonized and labelled with FITC as target, (Phagotest TM) and was measured by flow cytometry BD FACSCalibur. Serum ferritin level was measured using Cobas e 601. Result : Median phagocytosis of monocyte was 5,03 (0,17 ? 22,79) %, in 58 post- splenectomized subjects and 7,09 (0,11 ? 27,24) % in non-splenectomized subjects; p value > 0.05. Serum ferritin level was 6.274 (644,60 ? 21.835) ng/mL in post-splenectomized subjects and 4.702,50 (1.381 - 14.554) ng/mL in non-splenectomy subjects; p value < 0.05. The correlation between phagocytosis function of monocyte and serum ferritin level was r = 0.13 ( p value = 1.00). Conclusion : There was no statistical difference of phagocytosis function of monocyte between post-splenectomized subjects and non-splenectomized subjects. Serum ferritin level in post- splenectomized was higher than non-splenectomized subjects. There was no correlation between phagocytosis function of monocyte and serum ferritin level.;Background : Infection is an important cause of death in thalassemia. Increase of risk of infection could be due to iron overload and post-splenectomy. The study aimed to determine the difference of phagocytosis function of monocyte between post-splenectomized and non- splenectomized patients with thalassemia major, and the correlation of phagocytosis function of monocyte and serum ferritin level. Methods : The study was conducted in Department of Clinical Pathology Cipto Mangunkusumo hospital, Jakarta, in September 2013 – Februari 2014. It was a cross sectional study. The study subjects consisted of 58 post-splenectomized patients and 58 non-splenectomized patients with age and sex matching. Phagocytosis function of monocyte was determined using E.coli opsonized and labelled with FITC as target, (Phagotest TM) and was measured by flow cytometry BD FACSCalibur. Serum ferritin level was measured using Cobas e 601. Result : Median phagocytosis of monocyte was 5,03 (0,17 – 22,79) %, in 58 post- splenectomized subjects and 7,09 (0,11 – 27,24) % in non-splenectomized subjects; p value > 0.05. Serum ferritin level was 6.274 (644,60 – 21.835) ng/mL in post-splenectomized subjects and 4.702,50 (1.381 - 14.554) ng/mL in non-splenectomy subjects; p value < 0.05. The correlation between phagocytosis function of monocyte and serum ferritin level was r = 0.13 ( p value = 1.00). Conclusion : There was no statistical difference of phagocytosis function of monocyte between post-splenectomized subjects and non-splenectomized subjects. Serum ferritin level in post- splenectomized was higher than non-splenectomized subjects. There was no correlation between phagocytosis function of monocyte and serum ferritin level., Background : Infection is an important cause of death in thalassemia. Increase of risk of infection could be due to iron overload and post-splenectomy. The study aimed to determine the difference of phagocytosis function of monocyte between post-splenectomized and non- splenectomized patients with thalassemia major, and the correlation of phagocytosis function of monocyte and serum ferritin level. Methods : The study was conducted in Department of Clinical Pathology Cipto Mangunkusumo hospital, Jakarta, in September 2013 – Februari 2014. It was a cross sectional study. The study subjects consisted of 58 post-splenectomized patients and 58 non-splenectomized patients with age and sex matching. Phagocytosis function of monocyte was determined using E.coli opsonized and labelled with FITC as target, (Phagotest TM) and was measured by flow cytometry BD FACSCalibur. Serum ferritin level was measured using Cobas e 601. Result : Median phagocytosis of monocyte was 5,03 (0,17 – 22,79) %, in 58 post- splenectomized subjects and 7,09 (0,11 – 27,24) % in non-splenectomized subjects; p value > 0.05. Serum ferritin level was 6.274 (644,60 – 21.835) ng/mL in post-splenectomized subjects and 4.702,50 (1.381 - 14.554) ng/mL in non-splenectomy subjects; p value < 0.05. The correlation between phagocytosis function of monocyte and serum ferritin level was r = 0.13 ( p value = 1.00). Conclusion : There was no statistical difference of phagocytosis function of monocyte between post-splenectomized subjects and non-splenectomized subjects. Serum ferritin level in post- splenectomized was higher than non-splenectomized subjects. There was no correlation between phagocytosis function of monocyte and serum ferritin level.]

Abstrak :
ABSTRAK
Latar Belakang: Thalassemia merupakan hemoglobinopati herediter yang menyebabkan anemia kronis, sehingga pasien membutuhkan transfusi darah secara rutin yang dapat menyebabkan kelebihan besi. Kelebihan besi dapat memicu beberapa komplikasi, salah satunya adalah gangguan pertumbuhan. Tujuan: Mengetahui hubungan antara kadar hemoglobin dan profil besi dengan gangguan pertumbuhan pada pasien thalassemia. Metode: Studi cross-sectional pada 102 pasien thalassemia di Pusat Thalassemia RSCM Jakarta. Hasil: Empat puluh lima 44,1 subjek adalah perempuan dan 57 55,9 subjek adalah lelaki dengan rentang usia 9-14 tahun. Tiga puluh sembilan 38,2 subjek memiliki perawakan pendek dan 63 61,8 subjek memiliki perawakan normal. Nilai median kadar feritin serum pada pasien perawakan pendek adalah 2062 318-8963 ng/mL dan pada pasien perawakan normal adalah 3315 422,9-12269 ng/mL p.

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ABSTRACT
Background Thalassemia is a hereditary hemoglobinopathy which causes chronic anemia, thus the patients need regular blood transfusion which can cause iron overload. It leads to some complications, one of them is growth retardation. Aim To determine the association between hemoglobin level and iron profile with growth retardation on thalassemia patients. Methods cross sectional study on 102 patients in Thalassemia Center of RSCM Jakarta. Results Forty five 44.1 subjects are girls and 57 55.9 subjects are boys. Their age range was 9 14 years old. Thirty nine 38.2 subjects had short stature and 63 61.8 subjects had normal stature. Median of serum ferritin level in the short stature patients was 2062 318 8963 ng mL and normal stature was 3315 422.9 12269 ng mL p 0.001 . Median of transferrin saturation in the short stature patients was 88 19 100 and normal stature was 83 35 100 p 0.94 . Mean of pra transfusion hemoglobin level in the short stature patients was 8.14 SD 0.93 g dL and normal stature was 8.07 SD 0.86 g dL p 0.68 . Conclusion there is a significant association between serum ferritin level and growth retardation, but there is no significant association between transferrin saturation and pra transfusion hemoglobin level with growth retardation.

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ABSTRAK
Latar belakang: Thalassemia merupakan suatu penyakit gen tunggal yang disebabkan oleh kerusakan pada gen dalam mengontrol produksi protein sehingga sel darah merah akan mudah pecah dan pengikatan oksigen terganggu. Hal ini akan memicu terjadinya anemia dan membutuhkan transfusi darah secara rutin dan seumur hidup. Transfusi darah rutin menyebabkan terjadinya akumulasi besi yang memicu beberapa komplikasi, salah satunya adalah gangguan pada fungsi pankreas. Tujuan: Untuk mengetahui hubungan antara profil besi dengan gangguan fungsi pankreas berupa diabetes mellitus dan gangguan toleransi glukosa pada subjek thalassemia mayor. Metode: Desain potong-lintang pada 79 subjek thalassemia mayor di Pusat Thalassemia RS Cipto Mangunkusumo Jakarta. Hasil: Dua 2,53 subjek mengalami gangguan toleransi glukosa dan 77 97,47 subjek dengan nilai toleransi glukosa normal. Nilai median feritin serum pada kelompok gangguan toleransi glukosa yakni 5595,5 2062,0-9199,0 ng/mL sedangkan yang tidak mengalami gangguan toleransi glukosa yakni 3309,0 487,0-11247,0 ng/mL p= 0,574 . Nilai median saturasi transferin pada subjek gangguan toleransi glukosa yakni 76 52-100 sedangkan yang tidak mengalami gangguan toleransi glukosa yakni 89 11-100 p= 0,827 . Kesimpulan: Tidak didapatkan adanya hubungan yang signifikan antara kadar feritin serum dan saturasi transferin terhadap gangguan fungsi pankreas.

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ABSTRACT
Background Thalassemia is a single gene disease that is caused by defect on gene which controls the protein production that eventually leads to red blood cell lysis and defect on oxygen binding capacity. Therefore, the patient needs regular blood transfusion during his lifetime. Regular blood transfusion causes iron accumulation that leads to complications such as defect on pancreas function. Aim To know the association between iron profile and defect on pancreas function such as diabetes mellitus and glucose intolerance in major thallassemia subjects. Methods Cross sectional design on 79 major thalassemia subjects in Thalassemia Center of RS Cipto Mangunkusumo, Jakarta. Results Two 2.53 subjects were glucose intolerant and 77 97,47 subject has a normal blood glucose. Median value of serum ferritin level in glucose intolerant subjects was 5595.5 2062,0 9199,0 ng mL meanwhile the median value of serum ferritin level in normal glucose level subjects was 3309.0 487,0 11247,0 ng mL p 0.574 . The median value of transferrin saturation in glucose intolerant patients is 76 52 100 meanwhile the median value of tranferrin saturation level in normal glucose level subjects is 89 11 100 p 0,827 . Conclusion There is no significant association between serum ferritin level and transferrin saturation and defect of pancreas function.

Abstrak :
ABSTRAK
Transfusi darah dibutuhkan dalam meningkatkan kualitas hidup pasien thalassemia mayor, namun dapat menyebabkan kelebihan zat besi, sehingga diperlukan terapi kelasi besi, seperti deferipron dan deferasirox. Tujuan penelitian ini adalah menganalisis obat yang lebih cost-effective dengan metode Analisis Efektivitas-Biaya AEB karena masing-masing obat memiliki perbedaan efektivitas dan biaya obat yang signifikan. Data diambil secara retrospektif dan pengambilan sampel dilakukan secara total sampling berdasarkan catatan rekam medik dan sistem informasi rumah sakit. Pasien yang diikutsertakan merupakan pasien anak-anak pengguna deferipron n=33 dan deferasirox n=27 yang rutin melakukan transfusi darah pada tahun 2016. Efektivitas pengobatan diukur berdasarkan perubahan kadar serum ferritin. Biaya didapatkan dari median total biaya pengobatan, meliputi biaya obat, alat kesehatan, tindakan, administrasi dan jasa dokter, laboratorium serta kantong darah. Berdasarkan hasil penelitian, deferasirox 1.164 ng/mL lebih efektif dari deferipron 692 ng/mL dan median total biaya pengobatan deferasirox lebih mahal. Hasil akhir menunjukkan bahwa rasio efektivitas-biaya deferasirox Rp 65.816,68 lebih rendah dari deferipron Rp 74.956,60 , namun keduanya tidak ada yang mendominasi sehingga tidak dapat ditentukan terapi yang lebih cost-effective. Bila pengobatan deferipron dipilih, perlu dikeluarkan biaya tambahan sebesar Rp 52.416,64 untuk peningkatan satu unit efektivitas dan pengambil kebijakan di pelayanan kesehatan harus mempertimbangkan apakah biaya lebih tersebut sebanding dengan peningkatan efektivitasnya.

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ABSTRAK
Blood transfusions are needed in improving the quality of life of major thalassemia patients, but it can lead to excess iron, so it requires iron chelation therapy, such as deferiprone and deferasirox. This study is aimed to analyse whether deferipron or deferasirox is more cost effective with Cost Effectiveness Analysis CEA method because each drug has a significant difference in effectiveness and drug costs. Data were taken retrospectively and sampling was done using total sampling based on medical records and hospital information systems. Patients which included are pediatric patients with deferiprone n 33 and deferasirox n 27 who regulary perform blood transfusion in 2016. The effectiveness is measured by changes in serum ferritin levels and the cost is median of the total cost, summed from the cost of drugs, medical devices, hospitalization, administration, physician, laboratories and blood bags. Based on the results, the effectiveness of deferasirox 1,164 ng mL is greater than deferiprone 692 ng mL and median total cost of deferasirox is more expensive. The final result showed that cost effective ratio of deferasirox Rp 65.816,68 is lower than deferiprone Rp 74.956,60 , but none of both medications is dominant and therefore we could not determine which medication is more cost effective. If deferiprone is selected, it requires extra cost Rp 52.416,64 to increase the effectivity. Policy maker in healthcare facility need to consider if incremental cost of medication is equal to its increased effectiveness.