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"[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.;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.;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., 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.]"

"Latar belakang: Pemeriksaan cardiac siderosis dengan MRI T2 1,5 Tesla merupakan baku emas tetapi belum ada yang menggunakan MRI 3 Tesla. Provinsi Aceh merupakan daerah yang mempunyai banyak pasien thalassemia mayor di Indonesia, sampai saat ini belum ada data mengenai cardiac siderosis dan gangguan fungsi diastolik yang diperiksa dengan ekokardiografi tissue Doppler. Pemeriksaan cardiac siderosis yang tersedia di Aceh adalah MRI 3 Tesla.Tujuan: Menilai korelasi antara gangguan fungsi diastolik dengan cardiac siderosis ekokardiografi tissue Doppler dan feritin serum pada pasien anak thalassemia major di Aceh.Metode: Studi observasional dilakukan di Rumah Sakit Umum Dr. Zainoel Abidin (RSUZA) Banda Aceh pada bulan Juli hingga September 2018. Dilakukan pencatatan data karakteristik, Hb pre-transfusi, feritin serum, data ekokardiografi dan nilai MRI T2 3 Tesla jantung. Korelasi antara MRI T2 jantung dengan ekokardiografi dan feritin serum dinilai dengan uji Pearson.Hasil: Penelitian ini mengikutsertakan 34 subyek usia 8-17,5 tahun. Cardiac siderosis didapat pada 8 (23,5%) subyek. Gangguan diastolik didapati pada 10 (29,5%) subyek. Tidak dijumpai korelasi antara MRI T2 jantung dengan fungsi diastolik ETD (r= 0,086; p= 0,62). Terdapat korelasi signifikan antara MRI T2 dengan feritin serum (r= -0,537; p < 0,0001).Simpulan: Terdapat korelasi kuat antara MRI T2 jantung dengan kadar feritin serum, tetapi tidak terdapat korelasi antara fungsi diastolik dengan MRI T2 3 Tesla jantung

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Backgrounds: Cardiac T2 MRI at 1,5 T remains gold standard for cardiac siderosis. However in some centres only MRI 3 T is available. Aceh Province is the largest region with thalassemia careers in Indonesia, there are no data about cardiac siderosis and diastolic dysfunction in children with thalassemia major in Aceh. Thalassemia center in Aceh has only MRI 3 Tesla

Objectives: To study correlation diastolic function cardiac siderosis with cardiac T2 MRI 3 Tesla among Acehnesse children thalassemia.

Methods: Observational studies were conducted at Dr. General Hospital Zainoel Abidin (RSUZA) Banda Aceh from July to September 2018. Data on characteristics, pre-transfusion hemoglobin, serum ferritin, echocardiography and cardiac T2 MRI were recorded. Correlation between heart T2 MRI is carried out by the Pearson test as well as serum ferritin.

Results: Thirty-four subjects participated in the study aged 8-17.5 years. Eight subjects (23.5%) experienced cardiac siderosis which was examined by cardiac T2 MRI T2 3 Tesla. Diastolic dysfunction examination by tissue Doppler echocadiography were found in 10 (29.5%) subjects. There was no correlation between MRI of heart T2 with diastolic function tissue Doppler echocardiography (r = 0.086; p = 0.62). There was a significant correlation between MRI T2 and serum ferritin (r = -0.537; p <0.0001).

Conclusion: There was no correlation between cardiac T2 MRI 3 Tesla and diastolic function ETD. There was a strong and significant correlation between MRI T2 and serum ferritin. Tissue Doppler can detect early diastolic dysfunction in thalassemia patients better than conventional."

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

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

"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"

"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."

"Latar Belakang: Hipertrofi ventrikel kiri VKi merupakan adaptasi kardiak pada hipertensi dan meningkatkan risiko gagal jantung diastolik. Hipertrofi VKi sering ditemui pada gagal jantung diastolik, namun hubungan hipertrofi VKi dengan kapasitas fungsional dan parameter disfungsi diastolik masih kontroversi. Tujuan: Menilai korelasi IMVKi dengan kapasitas fungsional, perubahan parameter diastolik, dan global longitudinal strain GLS pada pasien hipertensi laki-laki asimptomatik dengan hipertrofi VKi. Metode: Pasien hipertensi laki-laki asimptomatik dengan IMVKi>115 gr/m2 tanpa masalah koroner, aritmia, penyakit jantung bawaan, dan penyakit jantung katup masuk kriteria studi. Uji latih ergocycle menggunakan protokol ramp. Akuisisi IMVKi pada awal uji dan pengukuran parameter diastolik E/A, E/e rsquo;, IVRT dan GLS pre dan puncak uji. Hasil: Terdapat 41 subjek dengan usia 55 32-64 tahun. Median nilai IMVKi subjek 129 116-319 gr/m2, dengan rerata kapasitas fungsional 5,7 1 METs. parameter diastolik pre dan puncak uji latih beban tidak berbeda bermakna. Rerata GLS pre uji rendah namun berbeda bermakna pada puncak uji latih pre vs puncak: -15,4 vs 18,5 ; p

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Backgrounds: Left Ventricular Hypertrophy LVH is an adaptation on hypertension and increases diastolic heart failure risk. LVH are common in diastolic heart failure. Prior studies showed various results on correlation Left ventricular mass index LVMI, with functional capacity and diastolic parameters.

Objectives: To assess correlations of LVMI with functional capacity, diastolic parameters changes, and global longitudinal strain GLS in male asymptomatic hypertensive patients with LVH.

Methods: Male asymptomatic hypertensive patients with LVMI 115 gr m2 without history of CAD, arrhythmia, congenital, and valvular heart disease are recruited. Stress test use ramp protocol. Initial LVMI is acquired, and diastolic parameters E A, E e, IVRT and GLS are acquired at pre and peak stress test.

Results: Forty one patients were recruited aged 55 32 64 years old. The median of LVMI was 129 gr m2 and mean functional capacity was 5,7 METs. Pre and peak stress test diastolic parameter values were insignificant. Pre stress test GLS mean was low but increased at peak pre vs peak 15,4 vs 18,5 p"

"Tujuan penelitian ini adalah untuk menilai perubahan area katup mitral (AKM) dan respon hemodinamik dengan Doppler ekokardiografi (DE) pada stenosis katup mitral (SKM) yang dilakukan uji latih baring. Menilai perubahan gradien tekanan (MVPG) dan aliran katup mitral (MVF) dalam penerapannya terhadap rumus Gorlin. Perekaman dilakukan pada istirahat dan akhir uji latih. Penelitian dilakukan pada 20 penderita SKM (18 SKM murni dan 2 SKM+Insufisiensi katup mitral). Parameter hemodinamik yang dinilai adalah AKM, dimensi atrium kiri, denyut jantung (DJ), curah jantung (CJ), isi sekuncup (IS); MVPG, MVF dan rasio ∆ MVPG/∆ MVF. Berdasarkan derajat stenosis penderita dibagi atas SKM ringan (AKM >1,5 cm2), SKM sedang (AKM 1-1,5 cm2) dan berat (AKM <1,0 cm2). Membuat korelasi AKM Doppler dengan kateterisasi, menilai perubahan AKM dengan uji latih dan menilai berbagai respon hemodinamik dengan AKM. Ada 8 penderita yang mempunyai data kateterisasi. Penilaian AKM dari Doppler dengan kateterisasi mempunyai korelasi yang balk (r=0,7365,p=O,04). Hanya 12 penderita yang dapat dinilai AKM dengan uji latih. Tidak didapatkan perubahan AKM dengan uji latih (p >0,05). Terdapat korelasi antara AKM dengan delta CJ (r=0,7552,p=0,0001) dan dengan delta IS (r=0,52,p=0,02), tetapi tidak mempunyai korelasi dengan delta DJ (selisih DJ puncak uji latih dengan istirahat) dengan r=0,09 maupun dengan delta DJ yang diperoleh dari selisih DJ pada saat rekaman Doppler pada akhir uji latih dengan DJ istirahat (r=-0,05). Nilai DJ pada puncak uji latih (dari EKG) tidak sama dengan DJ pada saat rekaman Doppler pada akhir uji latih (136 ± 13 dan 108 T 19). Terdapat keterbatasan DE untuk mendapatkan rekaman pola pada puncak uji latih, disamping penentuan "slope" dari pola mempunyai pengaruh terhadap perhitungan AKM. Perubahan gradien tekanan rata-rata {delta mMVPG) tidak mempunyai korelasi dengan AKM (r=0,01). Terdapat korelasi antara MVF dengan AKM (r=0,6692,p=0,001) begitu jugs pada rasio ∆MVPG/∆ MVF mempunyai korelasi terbalik dengan AKM (r=- 0,8247, p=0,00001). Perubahan hemodinamik ini mengikuti rumus Gorlin. Penelitian ini menyimpulkan, bahwa pemeriksaan Doppler ekokardiografi dapat dipakai untuk menilai perubahan hemodinamik pada penderita SKM yang dilakukan uji latih. "

"Penelition ini bertuiuan untuk menilai derajat Hipertensi Pulmonal (HP) secora kwantitatif dengan pemeriksaan "Pulsed Doppler Echocardiography" (PDE). Penelition dilakukan terhadap 60 penderita HP, semua menialani kateterisasi jantung. Kelompok kelola terdiri dari 15 orang normal.Parameter PDE dilakukan pengukuran "right ventricel pre ejection period" (RPEP), "acceleration time" (ACT), "right Ventricel ejection time" (RVEP) serta rosio RPEP/ACT, RPEP/RVET don AcT/RVET. BerdosarKan panjang fase AcT.penderita dibagi dalom 2 kelompok, yaitu kelompok ACT < 80 ms (kelompok A) dan ACT = 80-120 ms (kelompok B). Berdasarkan "mean pulmonary artery pressure" (MPA) dari kateterisasi jantung, penderita dikelompokkan menjadi kelompok 20-40 mmHg (HP-1), 41-60 mmHg (HP-2) don >60 mmHg C HP-3 ), berturut-turut sesuai dengan deraiat ringan, sedang dan berat. Berdasarkan "pulmonary arterial resintance" ( PAR ) penderita HP karena pirau intrakardial dibagi 2, yaitu kelompok < 5HRU (PAR-1) dan > 5 HRU (PAR-2). Nilai Parameter PDE dari tiap kelompok dibandingkan dengan nilai MPA dan PAR dari hasil pemeriksaan kateterisasi jantung. penderita dibagi 2 golongan, yaitu HP hiperkinetiK don HP pasif, selonjutnya parameter PDE kedua golongon tersebut dibandingkan.Didapatkon korelasi kuat antara AmPA masing-masing dengan RPEP ( MPA = 5.14 + 0.44 RPEP, r = 0.76, SEE = 9.34, X0.01), ACT ( MPA = 84.69 + 0.55 ACT, r =-0.78, SEE=8.99, p<0.01), dan RPEP/ACT (MPA= 18.93 + 15.90 RPEP/ACT, r=0.87, SEE= 7.07, P<0.01). Juga didapatkan korelasi kuat antara PAR dengan RPEP (PAR = -7.93 + 0.12 RPEP, r = 0.82, SEE = 2-055, P<0-01), ACT ( PAR = 17.44 - 0.15 ACT, r = -0.84, SEE = 1.89, P<0.01) dan RPEP/ACT (PAR= -1.16 + 4-24 RPEP/Ac75 r = 11.90, SEE=1.56, P<0-01). Rasio RPEP/ACT dapat membedakon kelcmpoK HP-1, HP-2 don HP-3 secara bermakna (HP-13 1.03-+0.27, dibanding HP-2, 2-02 (0.36, P<0.05 ; HP-2 dibanding HP-3, 2.82±0.423 p<0.05). Rasio RPEP/ACT 1.61 atau kurang sesuai dengan HP ringan, rasio 2.22 atau lebih sesuai dengan HP berat, rasio antara 1.61 - 2.22 sesuai dengan HP sedang. Parameter tersebut juga dapat menentukan tingginya PAR. Parameter PDE golongan HP hiperkinetik tidak berbeda bermakna dibanding HP pasif.Penelitian ini menyimpulkan bahwa RPEP, ACT don RPEP/ACT merupakan Parameter PDE yang dapat dipokai untuk menilai secora kwanitatif don kwanitatif dero,iat HP. Rasio RPEP/ACT merupakan satu-satunya parameter sensitif yang dapat digunakan untuk menentukan HP ringan, sedang dan berat. Golongan HP hiperkinetik dan HP pasif mempunyai parameter PDE soma."

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

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

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

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

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