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"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: Transfusi darah dan kelasi besi merupakan terapi utama thalasemia khususnya pada thalassemia beta mayor yang bergantung transfusi. Harapan hidup dan prognosis pada pasien pasien tersebut telah bertambah baik dalam beberapa dekade terakhir. Namun demikian gangguan pada jantung masih merupakan penyebab mortalitas utama pada pasien thalassemia beta mayor bergantung transfusi. Toksisitas besi pada jantung merupakan penyebab utama kelainan jantung pada pasien-pasien tersebut. Modalitas utama untuk deteksi toksisitas besi pada jantung adalah dengan MRIT2 yang kesediaannya terbatas.Tujuan: Penelitian ini bertujuan mendapatkan profil toksisitas besi pada jantung penderita thalassemia beta bergantung transfusi. Penelitian ini juga bertujuan melihat korelasi antara toksisitas besi dengan fungsi sistolik jantung, fungsi diastolik jantung dan kadar NTproBNP. Selain itu penelitian ini bertujuan untuk melihat korelasi muatan besi dengan toksisitas besi pada jantung, fungsi sistolik jantung, fungsi diastolik jantung dan kadar NTproBNP.Metode: Penelitian potong lintang pada penderita dewasa dengan thalassemia beta mayor yang bergantung transfusi di Poliklinik Thalasemia Dewasa RSCM Jakarta pada bulan Desember 2017. Data sekunder diperoleh dari rekam medik pasien berupa riwayat medis, hasil lab, hasil pemerikssan MRI T2 dan ekokardiografi. Dilakukan pemeriksaan fisik dan pengambilan darah untuk pemeriksaan NTproBNP. Analisis data berupa data deskriptif dan uji korelasi dengan uji Pearson dan Spearman.Hasil: Sebanyak 62 orang pasien dilibatkan dalam studi. Median untuk T2 jantung adalah 24,96 ms. Terdapat 27,4 pasien dengan hemosiderosis jantung yang ringan-sedang dan 11,3 dengan hemosiderosis jantung yang berat. Tidak terdapat korelasi antara toksisitas besi pada jantung dengan fraksi ejeksi, ratio E/A maupun kadar NTproBNP. Terdapat korelasi lemah antara ferritin serum dan toksisitas besi pada jantung r=-0,312, p=0,007. Terdapat korelasi lemah antara ferritin serum dan fraksi ejeksi r=-0,281, p=0,013.Simpulan: Toksisitas besi pada jantung ditemukan pada 38,7 penderita dewasa dengan thalassemia beta bergantung transfusi. Tidak terdapat korelasi antara toksisitas besi pada jantung dengan fungsi sistolik, fungsi diastolik mauapun kadar NTproBNP pada penderita dewasa dengan thalssemia beta bergantung transfusi. Terdapat korelasi lemah antara ferritin serum dengan toksisitas besi pada jantung dan fungsi sitolik jantung. Tidak terdapat korelasi antara muatan besi dengan NTproBNP.

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Background Regular blood transfusions and chelation are the mainstay of treatment in TDT patients. This has improved the survival and prognosis of the patients. Cardiac complications still remain to be the main cause of mortality. Cardiac iron toxicity is the main cause of complications leading to cardiac failure. MRI T2 is the gold standard for diagnosing cardiac iron toxicity, however this facility is limited in Indonesia.Objective.This study aimed to get a profile of the cardiac iron toxicity in adult TDT beta major patients to obtain a correlation of cardiac iron toxicity with cardiac function and NTproBNP levels and to evaluate if there is any correlation between iron overload with cardiac iron toxicity, cardiac function and NTproBNP levels.Methods Cross sectional study was done which included thalassemia beta major transfusion dependent patients from the adult thalassemia policlinic of RSCM hospital during desember 2017. Data was obtained from the medical records including history, laboratory results and results of MRIT2 and echocardiography. Physical examination was done and blood drawn for NTproBNP estimation. Data was analysed for descriptive data and correlation tests using pearsons or spearman rsquo s test.Results 62 patients were included in the study. Median cardiac T2 was 24.96 ms. 27.4 patients had mild moderate cardiac hemosiderosis and 11.3 had severe cardiac siderosis. There was no correlation between cardiac iron toxicity and ejection fraction, E A ratio and NTproBNP levels. There was a weak correlation between ferritin levels and cardiac toxicity r 0,312, p 0,007 as well as with ejection fraction r 0,281, p 0,013. There was no correlation between ferritin levels and E A or NTproBNP. There was no correlation between transferrin saturation levels with cardiac iron toxicity, ejection fraction, E A ratio as well as NTproBNPConclusion Cardiac iron toxicity was seen in 38,7 of TDT patients in this study. There was no correlation between cardiac toxicity and cardiac functions as well as NTproBNP levels. There was a weak correaltion between ferritin levels with cardiac toxicity and systolic function but no correlation with diastolic function. There was no correlation between transferrin saturation levels with cardiac toxicity, systolic function, diatolic function as well NtproBNP levels."

"ABSTRAKLatar Belakang: Muatan besi berlebih adalah kondisi yang akan terjadi pada penderita thalassemia yang bergantung transfusi. Muatan besi berlebih yang terjadi progresif akan menimbulkan kerusakan organ akibat toksisitas besi. Banyak ditemukan kelainan tulang pada penderita thalassemia seperti perawakan pendek, facies cooley atau fraktur spontan, tetapi sampai saat ini hanya sedikit penelitian yang secara khusus mencari efek toksisitas besi di tulang pada penderita thalassemia dewasa.Tujuan: Mengetahui peran toksisitas besi pada penurunan densitas tulang penderita thalassemia dewasa yang bergantung transfusiMetode: Studi potong lintang dilakukan terhadap penderita thalassemia mayor dan intermedia dewasa yang mendapat transfusi rutin di RSUPNCM Jakarta dari Agustus sampai Oktober 2016. Dilakukan pemeriksaan kadar besi yaitu saturasi transferrin ST dan ferritin serum, pemeriksaan Dual X-ray Absorbtiometry DXA untuk menilai densitas masa tulang BMD dan rontgen pelvis untuk menilai indeks femoral Singh. Analisis dilakukan untuk mengetahui korelasi antara ST atau ferritin dengan nilai BMD, korelasi antara indeks femoral Singh dengan BMD dan pencarian titik potong ST atau ferritin untuk membedakan densitas tulang rendah dan normal pada penderita thalassemia mayor dan intermedia dewasa dengan menggunakan receiver operating curve ROC .Hasil: Sebanyak 60 penderita usia 18-68 tahun, 32 adalah penderita thalassemia mayor dan 68 adalah penderita intermedia dewasa yang mendapat transfusi minimal sekali tiap bulan dengan rerata Hb pre-transfusi sebesar 8.08mg/dL. Sebanyak 68 penderita memiliki densitas tulang rendah. Didapatkan nilai median ST 86 20-112 , median dari rerata nilai ferritin setahun yaitu 3881 ng/mL 645-15437ng/mL , median nilai BMD terendah -1.1 -5.7- -2.6 . Didapatkan korelasi negatif secara bermakna antara ST dengan nilai BMD r=-0.329, nilai p=0.01 , namun tidak didapatkan korelasi yang bermakna antara ferritin dengan nilai BMD r=-0.088, nilai p=0.504 serta tidak ditemukan korelasi yang bermakna antara indeks femoral Singh dengan BMD r=0.273, nilai p= 0.038 . Kurva ROC, nilai ST didapatkan area dibawah kurva AUC 0.727 dengan titik potong ST 89.5 untuk membedakan densitas tulang rendah dan normal Kesimpulan: Kejadian densitas tulang rendah pada penderita thalassemia adalah sebesar 68 . Terdapat korelasi terbalik yang signifikan antara ST dan nilai BMD dengan nilai titik potong ST 89.5 untuk membedakan densitas tulang rendah dan normal pada thalassemia dewasa"

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""ABSTRACT"Background Iron overload is a complication experienced by transfusion dependent thalassemia TDT patients. Progressive iron accumulation results in tissue damage referred as iron toxicity. Bone deformities complication such as short stature, cooley rsquo s face and fracture are also commonly found among TDT patients but only few studies has been conducted to evaluate the effect of direct iron toxicity in bone among such population.Objective To determine the role of iron toxicity in low bone mass density among transfusion dependent thalassemia patients.Methods Cross sectional study conducted among major and intermedia thalassemia patients whom regularly received blood transfusion in CiptoMangunkusumo Central Hospital Jakarta between August to October 2016. Level of transferrin saturation TS and ferritin were measured as indicator of body iron level while dual x ray absorptiometry were measured to evaluate bone mass density BMD and pelvic X ray to evaluate Singh femoral index. Statistical analysis were conducted to evaluate correlation between TS or ferritin to BMD, correlation between Singh index and BMD and to determine the best cutoff value of TS or ferritin to differentiate between normal to low bone mass density among TDT patients using receiver operating curve ROC Results Total of 60 patients between 18 68 years old, 32 were thalassemia major patients, 68 were transfusion dependent thalassemia intermedia patients. Mean pre transfusion HB were 8.08mg dL, and as much as 68 subjects had low bone density. Median value of TS was 86 20 112 , median value of ferritin was 3881ng mL 645 15437ng mL , median value of the lowest BMD score was 1.1 5.7 2.6 . Significant reverse correlation between BMD score and TS was found r 0.329 p value 0.01 but no correlation with ferritin r 0.088, p value 0.504 nor correlation to Singh femoral index r 0.273, p value 0.038 . ROC curve analysis showed with area under the curve AUC 0,727, the best cutoff TS to differentiate normal to low bone density was 89.5 Conclusion Low bone mass density is a common complication of thalassemia major and transfusion dependent thalassemia intermedia. Reverse correlation between BMD score and TS with cutoff value of TS 89.5 to to differentiate normal to low bone density"

"Latar Belakang: Thalassemia adalah penyakit herediter, dan anemia berat adalah salah satu fenotip utama pada thalassemia mayor, sehingga transfusi sel darah merah adalah modalitas utama tatalaksana. Transfusi sel darah merah diberikan sebanyak 1-2 kali setiap bulan akan meningkatkan kesintasan, tetapi dapat meningkatkan risiko infeksi dan menyebabkan muatan besi berlebih, terutama pada penderita thalassemia bergantung transfusi/transfusion dependent thalassemia TDT. Infeksi adalah penyebab kematian kedua pada TDT, setelah gagal jantung. Risiko infeksi meningkat pada transfusi berulang, hal ini terjadi karena adanya infeksi akibat transfusi, dan perubahan respon imun. Perubahan respon imun terjadi karena adanya aloimunisasi dan muatan besi berlebih.Perubahan respon imun dalam TDT dapat terjadi baik dalam respon imun inat maupun imun spesifik. Dalam studi sebelumnya terdapat korelasi ferritin serum dengan jumlah CD4, tetapi hal ini belum diteliti di Indonesia.Tujuan: Mendapatkan korelasi antara muatan besi berlebih ferritin serum dan saturasi transferin dengan imuitas selular CD4 pada penderita dewasa thalassemia beta bergantung transfusi.Metode: Penelitian ini adalah studi potong lintang. Pengambilan sampel secara konsekutif pada TDT dewasa. Jumlah subjek adalah 64 orang. Subjek melakukan ronsen toraks dan pemeriksaan laboratorium darah. Pemeriksaan HBsAg, anti HCV, anti HIV diperiksa dengan menggunakan metode Electroimmunoassay ELISA . Serum feritin, dan saturasi transferin diperiksa menggunakan metode Electrocheminulescentimmunoassay ECLIA . Limfosit subset diperiksa menggunakan flowcytometer. Uji korelasi dengan menggunakan korelasi Spearman`s.Hasil: Pada penelitian ini mendapatkan proporsi Hepatitis B sebanyak 4,7 , Hepatitis C positif sebanyak 10,9 , tidak ditemukan anti HIV dan ditemukan 4 dari 41 subjek yang mengalami TB paru. Hasil uji Spearman menunjukkan korelasi negatif lemah dan tidak bermakna antara ferritin serum dengan CD4 p= 0,75, r= -0,04 , dan korelasi positif lemah dan tidak bermakna antara saturasi transferin dengan CD4 p= 0,133, r= 0,19 .Simpulan: Tidak terdapat korelasi yang bermakna antara muatan besi ferritin serum dan saturasi transferin dengan imunitas seluler CD4.

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Background Thalassemia is a hereditary disease and severe anemia is main phenotype in major thalassemia, therefore red cell transfusion is main modality in major thalassemia management. Transfusion which are given 1 2 times every month will improve prognosis and survival, but both higher risk infections and iron overload are found in thalassemia, especially in transfusion dependent thalassemia TDT. Infections are second cause of death in adult TDT, after heart failure. Higher risk infections are caused by multiple transfusions, which can cause alter in immune response due to alloimunization, transfusion related infections and iron overload. Iron overload in TDT can altered immune response, both innate immune and specific immune. Some studies showed correlation between ferritin and CD4, but these were not yet studied in IndonesiaObjective. Objectives in this study were to determine correlations between iron overload serum ferritin and transferrin saturation and immune cellular specific CD4 Methods This were cross sectional study. Subjects were examined consecutively with chest x ray and serum blood collections. Total subjects were 64 subjects. HBsAg, anti HCV, anti HIV, were tested using ELISA. Serum Ferritin, and transferin saturation were tested using ECLISA. lymphocyte subsets were analyzed using flowcytometer. Correlations tests used Spearman rsquo s test.Results We found proportion HBsAg 4,9 positive, Anti HCV positive 10,7 , no subjects with positive for anti HIV, and there was 4 41 subjects with lung tuberculosa from chest X ray. There were weak negative correlation and not significant between serum ferritin with CD4 p 0,75, r 0,04 , and weak positive correlation and not significant between transferrin saturation with CD4 p 0,133, r 0,19 .Conclusions There were no correlations between iron overload ferritin and cellular immunity CD4 in adult transfusion dependent thalassemia."

"Pendahuluan : Muatan besi berlebih merupakan masalah utama pada pasien thalassemia beta bergantung transfusi karena menyebabkan toksisitas pada jaringan atau organ. Laporan mengenai korelasi antara muatan besi berlebih dengan fungsi endokrin pada pasien dewasa TDT beta yang mengalami retardasi pertumbuhan di Indonesia belum pernah dilaporkan.Tujuan: Mendapatkan profil muatan besi dengan fungsi endokrin pada pasien dewasa TDT beta yang mengalami retardasi pertumbuhan.Metode: Dilakukan studi potong lintang pada pasien thalassemia beta mayor homozigot dan beta HbE usia dewasa yang mendapat transfusi darah di Poliklinik Thalassemia RSCM Jakarta pada Desember 2017. Muatan besi berlebih diwakili oleh feritin serum FS dan saturasi transferin ST, fungsi endokrin yang diperiksa adalah TSHs, fT4, dan IGF-1. FS, fT4 dan TSHs diperiksa dengan metode ELISA. IGF-1 diperiksa berdasarkan metode Solid-Phase ECLIA.Hasil: Proporsi hipotiroid subklinis sebesar 32,7 , kadar IGF-1 rendah pada 79,3 subjek penelitian. Terdapat korelasi negatif lemah FS dengan fT4 r = -0,361; p=0,003 , dan IGF-1 r=-0,313; p=0,008 , tidak terdapat korelasi FS dengan kadar TSHs r=0,074; p=0,29 . Tidak terdapat korelasi ST dengan TSHs r =0,003; p=0,492 , fT4 r=0,018; p=0,448 , dan IGF-1 r=-0,142; p=0,143.Simpulan: Terdapat korelasi negatif antara muatan besi berlebih yang dinilai dari feritin serum dengan fungsi endokrin yang dinilai dengan fT4 dan IGF-1.

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Introduction. Iron overload is a major problem in patients with transfusion dependent beta thalassemia, because it causes toxicity to tissues or organs. The correlation between iron overload and endocrine function in adult TDT beta patients in Indonesia have not been reported.This study aims to obtain a profile of iron load and endocrine function of adult TDT beta patients with growth retardation.Methods Cross sectional study was performed on beta homozygous beta and adult HbE beta patients receiving blood transfusions at the Thalassemia Kiara RSCM Jakarta Clinic, December 2017. Iron overload was represented by serum ferritin FS and transferrin saturation ST, and the endocrine functions are TSHs, fT4 by ELISA method and IGF 1 by the Solid Phase ECLIA method.Results Subclinical hypothyroid proportion was 32,7 and low IGF 1 level was found in 79.3 of subjects. There is a weak negative correlation between FS and fT4 r 0.361 p 0.003, and IGF 1 r 0.313 p 0.008 . No correlation was found between ST with TSHs r 0,003 p 0,492, fT4 r 0,018 p 0,448, and IGF 1 r 0,142 p 0,143.Conclusion There was negative correlation between iron overload based on serum ferritin with endocrine function based on fT4 and IGF 1."

"Seratus lima belas penderita thalassemia beta mayor yang mendapatkan tranfusi rutin di Pusat Thalassemia Bagian Ilmu Kesehatan Anak, Fakultas Kedokteran, Universitas Indonesia ? Rumah Sakit Umum Pusat Dr. Cipto Mangunkusumo dilakukan pemeriksaan hematologi. Didapatkan korelasi yang negatif antara besarnya limpa dengan parameter hematologi darah tepi. Hasil pemeriksaan parameter darah tepi cenderung menurun dengan membesarnya limpa dan kondisi tersebut akan membaik setelah splenektomi. Pada penelitian kami hipersplenisme dimulai pada limpa S (V ? VI). Pemeriksaan elektroforesis hemoglobin didapatkan penebalan fraksi HbF dan 90 penderita tersebut menunjukkan pola yg normal. Oleh karena itu, untuk melakukan konfirmasi elektroforesis hemoglobin perlu dilakukan dengan pemeriksaan elektroforesis hemoglobin pada kedua orang tua atau pemeriksaan analisis genetik dengan teknik biomolekuler. (Med J Indones 2004; 13: 8-16)

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One hundred and fifteen beta thalassemia major outpatients attending the Thalassemia Center Department of Child Health, Medical School University of Indonesia Dr. Cipto Mangunkusumo General Hospital for routine blood transfusion and hematology examination, participated in this study. There was a negative correlation between the size of the spleen and the peripheral blood parameters. All peripheral blood parameters tend to decrease with the enlargement of the spleen, and the condition is reversed after splenectomy. We observed that hypersplenism starts when the spleen is as big as S (V ? VI). The hemoglobin electrophoresis pattern from beta thalassemia major patients receiving repeated blood transfusion did not show a dense HbF fraction, 90 patients showed a normal hemoglobin electrophoresis pattern. A hemoglobin analysis of both parents could be useful to confirm the diagnosis of beta thalassemia major for patients receiving repeated blood transfusion. In order to get a definite diagnosis, a genetic analysis by bio molecular technique is needed. (Med J Indones 2004; 13: 8-16)"

"Latar belakang. Kadar hemoglobin pre-transfusi dan feritin serum mempengaruhi pertumbuhan anak dengan thalassemia B-mayor. Penelitian tentang thalassemia sudah dilakukan di Indonesia, namun penelitian tentang hubungan thalassemia dengan pertumbuhan fisik masih terbatas. Tujuan. Mengetahui pengaruh kadar Hb pre-transfusi dan feritin serum berpengaruh terhadap pertumbuhan fisik pasien thalassemia ?-mayor. Metode. Dilakukan bulan Agustus-Oktober 2017 pada pasien anak dengan thalassemia B-mayor yang berobat ke Thalassemia-Centre RSUD Pekanbaru. Penelitian berupa analitik observasional potong lintang, menganalisis pengaruh kadar Hb pre-transfusi dan feritin serum terhadap parameter perawakan pendek dan sangat pendek, gizi kurang dan buruk, usia tulang yang terlambat. Hasil. Subjek 41 orang, rentang usia 18-204 bulan. Jenis kelamin laki-laki lebih banyak daripada perempuan 53,7 vs 46,3. 40 subjek mengalami retardasi pertumbuhan. Terdapat korelasi bermakna antara kadar Hb pre-transfusi dengan Z-score TB/U r=0,507, p=0,001 dan LILA/U r=0,467, p=0,02. Hb pre-transfusi berpengaruh terhadap interpretasi duduk/umur p=0,007, IK95 -1,5 - -0,3, subischial leg length/umur p=0,002, namun tidak pada interpretasi rasio segmen atas/bawah dan usia tulang. Hasil berbeda pada kadar feritin yang tidak memiliki korelasi terhadap semua variabel. Simpulan. Terdapat pengaruh yang bermakna secara statistik antara kadar Hb pre-transfusi dengan parameter penelitian serta tidak terdapat pengaruh yang bermakna secara statistik antara kadar feritin serum dengan parameter tersebut.

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Background. The level of pre transfusion hemoglobin and ferritin serum affect physical growth on patient with thalassemic mayor. Study about thalassemia is mainly reported but its relationship with physical growth is limited.

Objective. The main objective of the present study was to evaluate the relationship of pre transfusion Hb and serum ferritin level in patient with thalassemic mayor.

Material and method. In this analytical cross sectional study, the growth parameters weight, standing height, sitting height, subischial leg length, nutritional status, bone age were measured in 41 patients attending Thalassemia Centre at RSUD in Pekanbaru from August October 2017.

Results. 41 patients with mean age 18 204 months. The results are boys dominated girls in sex criteria 53,7 vs 46,3. As much as 40 subjects have growth retardation. There rsquo s correlation in pre transfusion hemoglobin with Z score height for age r 0,507, p 0,001 and subischial length r 0,467, p 0,02. This study shows relationship in pre transfusion hemoglobin with sitting height p 0,007, IK95 1,5 0,3, subischial leg length p 0,002, but not in segment length and bone age. Serum ferritin level has no correlation to one of those parameters.

Conclusion. There is a significant relationship in physical growth based on parameters mentioned above with pre transfusion Hemoglobin, but not with serum ferritin level. "

" ABSTRAKLatar 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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ABSTRACTBackground 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."

"ABSTRAKLatar Belakang: Muatan besi berlebih yang diduga dapat menyebabkan peningkatan stres oksidatif malondialdehyde merupakan masalah utama pada pasien thalasemia beta mayor dan intermedia, baik TDT maupun NTDT. Transfusi darah dan kelasi besi merupakan terapi utama thalasemia. Kadar malondialdehyde MDA plasma belum diteliti mendalam di Indonesia, terutamapada pemberian transfusi serta korelasinya dengan muatan besi berlebih.Tujuan: mendapatkan profil kadar MDA pada pasien thalasemia dewasa; membandingkan kadarnya antara sebelum dan setelah transfusi antara pasien TDT dan NTDT serta mendapatkan korelasinya dengan feritin serum FS dan saturasi transferin ST .Metode: Penelitian potong lintang serta pre dan post study pada penderita dewasa thalasemia beta yang mendapatkan transfusi darah serta dengan/atau tanpa kelasi besi. Sampel darah diambil sesaat sebelum transfusi dan satu hari setelah transfusi. Kadar MDA plasma diperiksa berdasarkan metode Wills.Hasil: Sebanyak 63 orang pasien dilibatkan dalam studi, terdiri dari 51 TDT dan 12 NTDT. Median kadar MDA adalah 0,49 0,21-1,33 ?mol/L. Kadar tersebut tidak berbeda bermakna antara sebelum dan setelah transfusi, antara pasien TDT dan NTDT. Didapatkan korelasi lemah antara FS dengankadar MDA sebelum transfusi sedangkan tidak ada korelasi antara FS dengan kadar MDA setelah transfusi dan antara ST dengan kadar MDA.Simpulan: Median kadar MDA plasma pada pasien dewasa dengan thalasemia beta mayor dan intermedia sebelum transfusi 0,49?mol/L.Tidak ada perbedaan bermakna antara kadar MDA sebelum dan setelah transfusi dan antara pasien TDT dan NTDT. Terdapat korelasi lemah antara FS dengan MDA sebelum transfusi dan tidak terdapat korelasi FS dengan MDA setelah transfusidan ST dengan kadar MDA.

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ABSTRACTAbstract Background Iron overload is a major problem in thalassemic patients, either in TDT or NTDT. Iron overload may increase oxidative stress malondialdehyde MDA . Blood transfusion and chelating iron are the main therapy for beta thalassemia major TDT. However, plasma MDA levels have not been well studied in Indonesia, especially its correlation with iron overload.Objective This study aimed to profile MDA levels in adult thalassemic patients to compare its level between before and after transfusion and between TDT and NTDT patients and to obtains its correlation with serum ferritin SF and transferrin saturation TS .Method A cross sectional as well as pre and post study in adult patients with thalassemia major and intermedia who received blood transfusion with or without chelating iron.Blood samples were withdrawn immediately before transfusion and one day after transfusion. Plasma MDA levels were assayed according to Wills method. Results Total of 63 patients were enrolled, consisting 51 TDT and 12 NTDT patients. Median MDA level was 0.49 0,21 1,33 mol L. The level was not significantly different between before and after transfusion, between TDT and NTDT patients. Weak correlation was observed between SF and MDA levels before transfusion and there is no correlation was observed between SF and MDA levels before transfusion and also between TS and MDA levels.Conclusion Median plasma MDA levels in adult patients with beta thalassemia major and intermedia before transfusion 0,49 mol L. No significant different is found between MDA before and after transfusion and between TDT and NTDT patients as well. Plasma MDA levels have weak correlation with serum feritin levels before blood transfusion."