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"ABSTRACTLatar belakang: Kardiomiopati merupakan penyebab kematian tertinggi pada pasien thalassemia mayor, dengan mayoritas kasus merupakan gagal jantung kiri. Nilai referensi ventrikel kiri yang spesifik untuk pasien thalassemia mayor anak belum tersedia. Tujuan penelitian: Menentukan normogram rentang volume dan fungsi ventrikel kiri jantung sesuai BSA pada pasien thalassemia mayor anak dengan cardiac iron load normal. Metode: Sampel studi ini mencakup 60 pasien thalassemia mayor 30 lelaki, 30 perempuan berusia < 18 tahun yang memiliki cardiac iron load normal berdasarkan waktu T2 jantung > 20 ms pada 1.5T , tidak memiliki keluhan kardiovaskular secara klinis, maupun ko-morbiditas yang signifikan. Volume dan fungsi ventrikel kiri diukur dari gambar MRI sekuens cine-SSFP potongan short axis, pada fase end-diastolic dan end-systolic. Hasil: Analisis regresi menunjukkan korelasi signifikan antara BSA dengan hasil pengukuran ventrikel kiri dengan model non-linear vol = a BSAb , kecuali untuk LVEF. Tidak terdapat perbedaan LVEF yang signifikan antara subjek thalassemia mayor anak lelaki mean 62,5 , SD 3,8 dan perempuan mean 61,7 , SD 4,3 . Cardiac output lebih tinggi pada subjek lelaki dibandingkan perempuan pada rentang BSA 0,6 hingga 1,7 m2. Kesimpulan: Penelitian ini menghasilkan nilai referensi dalam bentuk normogram untuk parameter volume dan fungsi ventrikel kiri yang dapat dipergunakan secara spesifik untuk pasien thalassemia mayor anak.

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ABSTRACT"Background Cardiomyopathy represents the leading cause of mortality in thalassemia major patients, with left sided heart failure predominating. Normalized LV parameters for adult thalassemia major population has been established, yet specific reference values for pediatric thalassemia major population are still lacking. Objective To determine gender specific reference values of LV measurements for pediatric thalasemia major patients based on BSA. Methods The study included 60 pediatric thalassemia major patients 30 males, 30 females who had normal cardiac iron load based on T2 MRI time above 20 ms at 1.5T , without cardiovascular symptoms or significant co morbidities. Left ventricular volumes and function were measured on SSFP cine CMR end diastolic and end systolic images, acquired in short axis plane. Results Regression analyses demonstrated good, significant correlations p 0.05 between BSA and cardiac measurements with non linear growth model vol a BSAb , except for LVEF which remained constant throughout the BSA range. LVEF in males mean 62.5 , SD 3.8 did not differ significantly to females mean 61.7 , SD 4.3 . Cardiac output was projected to be constantly higher in males from BSA 0.6 to 1.7 m2. Conclusion This study has established normograms of left ventricular volumes and function parameters to be used specifically for pediatric thalassemia major patients. "

"Latar 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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Background: 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: Hiperfosfatemia pada penyakit ginjal kronik (PGK) terjadi akibat kegagalan ginjal dalam mengekskresi fosfat, tingginya asupan fosfat atau peningkatan pelepasan fosfat dari ruang intraselular. Hipertrofi ventrikel kiri (left ventricle hypertrophy/LVH) adalah perubahan jantung yang umum terjadi dan menjadi tanda awal penyakit kardiovaskular pada anak dengan PGK. Penelitian ini bertujuan untuk menilai hubungan antara kadar fosfat darah dengan fungsi sistolik serta penebalan ventrikel kiri jantung pada pada pasien anak penyakit ginjal kronik tahap akhir. Metode: Penelitian ini merupakan studi potong lintang di RSUPN dr. Cipto Mangunkusumo Jakarta terhadap anak PGTA tanpa ada kelainan jantung bawaan dari april-mei 2024 dengan dilakukan pemeriksaan fosfat darah dan ekokardiografi. Hasil: Terdapat 56 subyek dengan titik potong kadar fosfat darah 7,35 mg/dL. Didapatkan penurunan fungsi fraksi ejeksi dengan rasio prevalens pada pasien dengan hiperfosfatemia adalah 3,895 dengan IK 95% antara 2,552-9,773 (p = 0,002) serta kecenderungan hubungan kadar fosfat dengan penebalan LVMI (p = 0,680) dan disfungsi diastolik jantung kiri (p = 0,145). Kesimpulan: Terdapat hubungan antara kadar hiperfosfemia darah dengan fungsi sistolik pada pasien anak penyakit ginjal kronik tahap akhir. Tetapi tidak terdapat hubungan dengan peningkatan massa ventrikel kiri jantung dan diastolik jantung.

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Background: Hyperphosphatemia in chronic kidney disease (CKD) occurs due to renal failure to excrete phosphate, high phosphate intake or increased phosphate release from the intracellular space. Left ventricle hypertrophy (LVH) is a common heart change and an early sign of cardiovascular disease in children with CKD. This study aimed to assess the relationship between blood phosphate levels to decreased systolic and diastolic function and thickening of the left ventricle in pediatric patients with end-stage chronic kidney disease. Method: This was a cross-sectional observational study at RSUPN dr. Cipto Mangunkusumo Jakarta with PGTA children without congenital heart defects. Paremeters for function and LVM were assessed by Doppler echocardiography and blood phosphate examination. Results: There were 56 subjects with a cut point for blood phosphate levels of 7.35 mg/dL. It was found that a decrease in ejection fraction function with a prevalence ratio in patients with hyperphosphatemia was 3.895 with a 95% CI between 2.552-9.773 (p = 0.002) as well as a trend in the relationship between phosphate levels and LVMI thickening (p = 0.680) and left heart diastolic dysfunction (p = 0.145) Conclusion: There is association between blood levels of phosphemia and systolic function in pediatric patients with end-stage chronic kidney disease. However, there is no association with increased left ventricular mass index and dyastolic function."

"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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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."

"Latar belakang. Pasien thalassemia mengalami anemia kronis akibat tidak terbentuk atau berkurangnya produksi rantai globin-α atau -β yang diturunkan secara genetik, menyebabkan terjadinya eritropoiesis inefektif dan hemolisis. Kondisi tersebut menyebabkan pasien thalassemia memerlukan transfusi rutin seumur hidupnya. Transfusi rutin akan menyebabkan stimulasi antigen berulang dan iron overload. Stimulasi antigen berulang dan stres oksidatif akibat iron overload akan menyebabkan terjadinya gangguan imunitas dan membuat mereka lebih rentan terhadap infeksi, termasuk infeksi tuberkulosis. Tujuan. Mengetahui prevalensi infeksi tuberkulosis laten pasien thalassemia mayor anak, dan mengetahui hubungan antara lama sakit dan kadar feritin serum dengan kejadian infeksi tuberkulosis laten pada anak dengan thalassemia mayor. Metode. Desain penelitian ini adalah studi potong lintang. Subjek merupakan pasien thalassemia mayor anak usia 1 hingga 18 tahun yang kontrol rutin di RS Anna Medika Kota Bekasi. Uji interferon gamma release assay (IGRA) dilakukan pada semua subjek untuk mendeteksi adanya infeksi tuberkulosis. Hasil. Penelitian dilakukan pada 127 subjek thalassemia mayor anak, usia 1-18 tahun, sesuai kriteria inklusi dan eksklusi. Prevalensi infeksi TB laten pada populasi pasien thalassemia mayor anak adalah sebesar 8,7%. Tidak ada hubungan antara variabel lama sakit dan kadar feritin serum dengan terjadinya infeksi TB laten pada pasien thalassemia mayor anak. Kesimpulan. Prevalensi infeksi TB laten pada populasi thalassemia mayor anak lebih tinggi dibanding populasi anak nonthalassemia.

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Background. Thalassemia patient suffers chronic anemia due to no or lack of production of the α- or β-globin chain that causes ineffective erythropoiesis and hemolysis. This typical condition causes thalassemia patients to need routine blood transfusions for the rest of their life. Routine transfusion causes repeated antigen stimulation and iron overload. Repeated antigen stimulation and oxidative stress due to iron overload can cause immunity problems that make thalassemia major patients more prone to infection, including tuberculosis (TB) infection. Objectives. To identify the prevalence of latent tuberculosis infection in pediatric patients with thalassemia major and to evaluate the correlation between the length of illness and serum ferritin level with the occurrence of tuberculosis infection latent in pediatric patients with thalassemia major. Methods. This research is a cross-sectional study. The subjects were pediatric patients aged 1 to 18 years old who were diagnosed with thalassemia major, who routinely visited and were treated in Anna Medika Hospital in Bekasi. IGRA test had been done to all of the subjects to detect the existence of tuberculosis infection. Results. The research is done to 127 thalassemia major pediatric patients,range ages 1to 18 years old, that are suitable with inclusive and exclusive criteria. The prevalence of latent TB infection in thalassemia major pediatric patients population is 8,7%. There is no correlation variable between the length of illness and serum ferritin level with the occurrence of latent TB infection in thalassemia major pediatric patients. Conclusions. The prevalence of latent TB infection in thalassemia major children population is higher than that in the population of children without thalassemia."

"Latar belakang. Gagal jantung dan aritmia merupakan penyebab kematian tersering pada penderita thalassemia R. Gangguan fungsi jantung, khususnya disfungsi diastolik merupakan komplikasi dini pada jantung akibat muatan besi berlebih (iron overload). Kadar feritin serum sampai saat ini masih secara luas digunakan sebagai parameter muatan besi berlebih (iron overload).Tujuan. Mengetahui perbedaan kadar feritin serum antara penderita thalassemia j3 dewasa yang mengalami dan tidak mengalami disfungsi diastolik ventrikel kiri, dan mengetahui besar proporsi disfungsi diastolik pada penderita thalassemia 13 dewasa.Metodologi. Penelitian ini merupakan studi potong lintang untuk melihat perbedaan kadar feritin serum (sebagai parameter iron overload) pada penderita thalassemia 13 dewasa yang mengalami disfungsi diastolik dibandingkan dengan yang tanpa disfungsi diastolik, serta untuk mendapatkan proporsi disfungsi diastolik pada penderita thalassemia 3 dewasa. Analisis terhadap variabel-variabel yang diteliti menggunakan uji-1 independen untuk mendapatkan perbedaan rerata kadar feritin serum antara kedua kelompok.Hasil. Dari penelitian ini 30 orang penderita thalassemia 13 dewasa, laki-laki 13 orang, perempuan 17 orang, didapatkan rerata usia 25,9 tahun dengan rentang usia antara 18-38 tahun. Rerata Hb sebesar (7,5g%, SB I,4g%) dengan rentang kadar Hb antara 5,2 - 9,9 g%. Rerata kadar feritin serum sebesar (5590ng1m1, SB 4614,7 nglml) dengan rentang kadar, feritin antara 296,4 - 15900 nglml. Tidak terdapat perbedaan rerata kadar feritin antara penderita yang mengalami disfungsi diastolik dibandingkan dengan yang tidak mengalami disfungsi diastolik. Proporsi disfungsi diastolik pada thalassemia 13 dewasa pada penelitian ini sebesar 70%.Kesimpulan. Tidak terdapat perbedaan rerata kadar feritin antara penderita yang mengalami disfungsi diastolik dibandingkan dengan yang tidak mengalami disfungsi diastolik. Proporsi disfungsi diastolik pada thalassemia 13 dewasa pada penelitian ini sebesar 70%.

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Background. Heart failure and aritmia is the major cause of death in 3 thalassemia major. Heart dysfunction, especially diastolic dysfunction in ji thalassemia seems to be an early involvement of the heart due to iron overload. Serum ferritin level as a parameter of iron overload still widely use for evaluation in 13 thalassemia.

Objectives. To know the mean difference of serum ferritin level between adult 13 thalassemia patients who have left ventricular diastolic dysfunction and who do not have Ieft ventricular diastolic dysfunction, and to obtain the proportion of diastolic dysfunction in adult 13 thalassemia patients.

Methods. This cross-sectional study was conducted to see the mean difference of Serum ferritin. IeVel'(as a parameter of iron overload) in adult P'thalassemia who have left ventricular diastolic dysfunction and who do not have left ventricular diastolic dysfunction and to know the proportion of diastolic dysfunction among adult 13 thalassemia. The independent t-test was used to analyze the variables to obtain the mean difference of serum ferritin level between the two groups.

Results. Thirty adult P thalassemia patients, 13 were male and 17 were female had been enrolled into this study. The age of the patients ranged from 18 to 38 years old, and the average-age was 25,9 years. The Hb level ranged from 5,2 to 9,9 g% and the mean was (7,5g%, SD 1,4g°/o). The serum ferritin level ranged from 296,4 to 15900 nglml, and the mean was (5590ng/ml, SD 4614,7 nglml). There was no significance mean difference serum ferritin level in patients who had diastolic dysfunction and those who do not have diastolic dysfunction. The proportion of diastolic dysfunction in adult 13 thalassemia patients in this study was 70%.

Conclusions. There was no significannce mean difference serum ferritin level in patients who had. diastolic dysfunction and those. who. did, not have diastolic dysfunction . The proportion of diastolic dysfunction in adult thalassemia 3 patients in this study was 70%."

"Aliran darah koroner terjadi terutama pada fase diastolik. Stenosis arteri koronaria menimbulkan iskemia miokard. Iskemia miokard dapat menimbulkan gangguan diastolik. Gangguan diastolik mengakibatkan penurunan aliran darah koroner pada sepertiga awal diastolik, baik pada waktu istirahat maupun selama takikardia. Angina pektoris merupakan gejala iskemia miokard. Tujuan penelitian ini untuk membuktikan bahwa ditemukan gangguan diastolik ventrikel kiri, dan angina pektoris akan lebih berat bila disertai dengan gangguan diastolik ventrikel kiri pada pasien stenosis arteri koronaria. Pada penelitian ini dilakukan pemeriksaan variabel diastolik ventrikel kiri, dengan ali ran mitral gelombang pulsa doppler ekokardiografi saat istirahat, pada pasien yang terbukti mengalami stenosis arteri koronaria dari pemeriksaan angiografi koroner. Pada penelitian ini, yang memenuhi kelima variabel diastolik dan diklasifikasikan sebagai fungsi diastolik normal adalah 3,3%, relaksasi abnormal 10%, sementara 86,7% menunjukkan perubahan beberapa variabel diastolik.

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Coronary blood flow occurs mainly in the diastolic phase. Arterial stenosis The coronary artery gives rise to myocardial ischemia. Myocardial ischemia can cause diastolic disorders. Diastolic disorders result in decreased blood flow coronary in the first third of diastolic, both at rest and during tachicardia. Angina pectoris is a symptom of myocardial ischemia. The purpose of this study to prove that diastolic disorders of the left ventricle, and angina were found Pectoris will be more severe when accompanied by diastolic disorders of the left ventricle in patients with coronary artery stenosis.

In this study, a variabel examination was carried out diastolic left ventricle, with ali ran mitral pulse wave Doppler echocardiography at rest, in patients who have been shown to have coronary artery stenosis of coronary angiography examination. In this study, a variabeI examination was carried out diastolic left ventricle, with ali ran mitral pulse wave Doppler echocardiography at rest, in patients who have been shown to have coronary artery stenosis of coronary angiography examination. In this study, which meets all five variables diastolic and classified as normal diastolic function is 3.3%, relaxation abnormal 10%, while 86.7% showed changes in several diastolic variables."

"Introduction: The major cause of mortality and morbidity post-MI is the complications following the infarction. One of the most common MACE is malignant arrhythmia, this includes VF, VT and non-sustained VT. Malignant arrhythmia is caused due to the culmination of biochemical, electrophysiological, autonomic and genetic changes after an event of ischemia which results in myocardial damage and scarring, as well as left ventricular systolic dysfunction. Early risk stratification is important in AMI and cTnI and LVEF has been two accessible markers that has been studied in various aspects of AMI as prognostic markers, however there has been little studies of its role and correlation in post-AMI malignant arrhythmia. This research will therefore explore the correlation between myocardial damage (cTnI) and left ventricular systolic function (LVEF) with malignant arrhythmia in AMI patients. Methods: A retrospective cohort study was conducted on AMI patients who are admitted to the ICCU of Cipto Mangunkusumo General Hospital, Jakarta from November 2018 to May 2019. Patients who experienced severe infection and who has malignant arrhythmia when admitted were excluded. The association between cTnI and malignant arrhythmia was tested using Mann-Whitney test, while the association between LVEF and malignant arrhythmia was tested using Independent T-Test. Pearson’s Chi-Square test was done to test the relationship between systolic function status with malignant arrhythmia, All data analysis was performed on IBM SPSS Statistics. Results: Total of 110 patients were included in this study. 13.6% of total subjects experience malignant arrhythmia during hospitalisation. There is no significant correlation between cTnI and post-AMI malignant arrhythmia (p = 0.053, RR 1.2, 95%CI 1.1-1.2) but significant correlation between LVEF and post-AMI malignant arrhythmia was found, on both metric (t(108)=3.450, p = 0.001) and categorical (c2(1) = 6.132, p = 0.013, RR 4.8, 95%CI 1.15-20.4) assessment. There were major differences in the mean value of cTnI and LVEF between the two groups. Conclusion: This study has found statistically significant correlation between left ventricular systolic function (LVEF) with malignant arrhythmia in AMI patients, but no significant correlation between cTnI and malignant arrhythmia in AMI patients. Higher cTnI levels are more frequent in malignant arrhythmia group. Malignant arrhythmia is more common in AMI patients with lower LVEF.

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Pendahuluan: Penyebab utama mortalitas dan morbiditas infark miokard akut (IMA) adalah komplikasi pasca infark. Salah satu MACE paling umum ditemukan adalah aritmia maligna, yang meliputi VF, VT dan VT sesaat. Aritmia maligna disebabkan oleh kombinasi perubahan biokimia, elektrofisiologi, otonomi, serta genetik setelah kejadian iskemik yang kemudian menyebabkan kerusakan dan fibrosis pada miokard. Stratifikasi risiko awal sangat penting dalam kasus IMA. cTnI serta LVEF merupakan dua marka yang mudah diakses dan telah dipelajari dalam berbagai aspek IMA. Akan tetapi, studi mengenai peran dua marka tersebut dalam aritmia maligna pasca-IMA masih sedikit. Studi ini akan mempelajari korelasi antara kerusakan pada miokard (cTnI) dan fungsi sistolik ventrikel kiri (LVEF) dengan aritmia maligna pada pasien IMA. Metode: Sebuah studi kohort retrospektif dilakukan pada pasien IMA yang dirawat di ICCU Rumah Sakit Cipto Mangunkusumo, Jakarta dalam periode November 2018 hingga Mei 2019. Pasien yang mengalami infeksi parah dan pasien yang mengalami aritmia maligna saat admisi tidak diikutsertakan dalam penelitiaan ini. Hubungan cTnI dengan aritmia maligna dianalisis melalui uji Mann-Whitney dan hubungan LVEF dengan aritmia maligna dianalisis oleh uji Independent T-Test dan pada hubungan status fungsi sistolik dengan aritmia maligna dianalisis menggunakan uji Pearson Chi-Square. Analisis data dilakukan dengan software IBM SPSS Statistics. Hasil: Total 110 pasien dilibatkan dalam penelitian ini. 13.6% dari total pasien mengalami aritmia maligna selama masa hospitalisasi. Tidak ditemukan adanya hubungan signifikan antara cTnI dengan aritmia maligna pada pasien IMA (p = 0.053, RR 1.2, 95%CI 1.1-1.2), namun ditemukan adanya hubungan signifikan antara LVEF dengan aritmia maligna pada pasien IMA, baik pada data metrik (t(108)=3.450, p = 0.001) maupun data kategorik (c2(1) = 6.132, p = 0.013, RR 4.8, 95%CI 1.15-20.4). Terdapat perbedaan besar antara nilai rata-rata cTnI and LVEF pada kedua kelompok pasien. Kesimpulan: Studi ini menemukan korelasi yang signifikan secara statistikal antara fungsi sistolik ventrikel kiri dengan aritmia maligna pada pasien IMA, namun tidak ditemukan adanya korelasi signifikan antara cTnI dengan aritmia maligna pada pasien IMA. Nilai cTnI yang tinggi lebih umum ditemukan pada kelompok pasien dengan aritmia maligna. Kejadian aritmia maligna lebih umum pada pasien yang memiliki LVEF yang lebih rendah."

"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: Gagal jantung adalah penyebab utama kematian pada thalassemia akibat penumpukan besi dari transfusi darah. Ekokardiografi sering digunakan untuk evaluasi fungsi jantung, namun interpretasi hasilnya sangat bergantung dari operator. Uji berjalan 6 menit adalah metode sederhana yang terbukti mempunyai reliabilitas baik untuk menilai kapasitas fungsional kardiorespirasi sehingga dapat menjadi alternatif penilaian fungsi jantung anak thalassemia. Tujuan: Mendapatkan uji berjalan 6 menit sebagai metode sederhana untuk mengukur fungsi jantung anak thalassemia. Metode: Penelitian kasus kontrol pada subjek thalassemia dan kontrol berusia 11-18 tahun yang dipilih secara consecutive sampling. Subjek thalassemia mempunyai rerata feritin serum >2500 ng/mL dalam 6 bulan terakhir. Subjek kontrol dalam kondisi sehat dan tidak pernah menjalani transfusi darah. Uji berjalan 6 menit dilakukan pada kedua subjek, sedangkan ekokardiografi konvensional (EK) dan tissue Doppler (ETD) hanya dilakukan pada subjek thalassemia oleh seorang konsultan kardiologi anak. Data sekunder lain pada subjek thalassemia diambil dari rekam medis yaitu rerata hemoglobin pra-transfusi dalam 1 tahun terakhir, feritin serum dan saturasi transferin dalam 6 bulan terakhir. Hasil: Sebanyak 40 subjek thalassemia dan 109 kontrol berpartisipasi dalam penelitian ini. Median usia subjek thalassemia 13,4 (11-17,9) tahun dan kontrol 14,2 (11,3-17,9) tahun. Rerata hemoglobin pra-transfusi 7,6±0,6 g/dL. Median feritin serum 4246,5 (2506-10749,7) ng/mL dan saturasi transferin 100 (50-100) %. Setelah dilakukan matching usia dan jenis kelamin, jarak tempuh uji berjalan 6 menit pada subjek thalassemia lebih pendek daripada kontrol (465,1±74,2 vs 671±94,2, p<0,001). Parameter fungsi sistolik dan diastolik jantung dari EK dalam batas normal, tetapi ETD menunjukkan 45% subjek thalassemia mengalami gangguan fungsi diastolik (rasio E/E’ >8). Tidak ada faktor yang berkorelasi dengan jarak tempuh pada subjek thalassemia, sedangkan tinggi badan berkorelasi dengan jarak tempuh pada kontrol berdasarkan analisis bivariat. Kesimpulan: Jarak tempuh antara subjek thalassemia lebih rendah daripada kontrol. Peran ETD lebih baik daripada EK dalam mengevaluasi fungsi jantung. Uji berjalan 6 menit dapat digunakan sebagai skrining fungsi jantung pada anak thalassemia.

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Background: Heart failure is leading cause of mortality in thalassemia due to transfusion-induced iron overload. Evaluation of cardiac function is routinely performed with echocardiography. However, its interpretation depends on operator. The six minute walk test is a simple and reliable method to assess cardiorespiratory performance, therefore, it is suggested to be an alternative in evaluating cardiac function in thalassemia.

Aim: To obtain six minute walk test as a simple method in order to evaluating cardiac function in thalassemia.

Methods: This case control study was performed in thalassemia subjects (cases) and controls aged 11-18 year old which were selected with consecutive sampling. Cases should have mean serum ferritin level >2500 ng/mL in last 6 months. Controls must be in healthy condition and have never had blood transfusion. Both cases and controls performed six minute walk test, while echocardiography (conventional and tissue Doppler) was only done in cases by a pediatric cardiologist. Other secondary data collected from medical records in cases were mean of pre-transfusion hemoglobin in last 1 year, serum ferritin and transferin saturation in last 6 months.

Results: There were 40 cases and 109 controls involved in this study with median age were 13.4 (11-17.9) and 14.2 (11.3-17.9), respectively. The mean of pre-transfusion hemoglobin was 7,6±0,6 g/dL. The median serum ferritin was 4246.5 (2506-10749.7) ng/mL and transferin saturation 100 (50-100) %. After sex and age matching, the six minute walk distance was lower in cases than controls (465.1±74.2 vs 671±94.2, p<0.001). Conventional echocardiography did not find any systolic and diastolic dysfunction in cases. However, tissue Doppler echocardiography found 18 (45%) subjects with E/E’ ratio >8, which were categorized as diastolic dysfunction. There were no factors correlated to six minute walk distance in cases, while body height was correlated to six minute walk distance in controls based on bivariat analysis.

Conclusion: The distance of six minute walk test in thalassemia subjects was shorter than controls. Tissue Dopper echocardiography is better than conventional in order to evaluating cardiac function. The six minute walk test can be used for screening cardiac function in thalassemia."