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"Latar belakang: Transfusi rutin merupakan terapi utama bagi pasien thalassemia mayor, namun transfusi berulang diikuti masalah baru yaitu beban kelebihan besi yang terakumulasi dalam jaringan. Pemberian terapi kelasi besi adalah satu-satunya cara untuk mempertahankan keseimbangan besi dalam tubuh.Tujuan: Studi ini bertujuan untuka mengetahui hubungan efektivitas terapi, efek samping obat dan biaya antara kelasi besi regimen kombinasi (DFO+DFP dan DFP+DFX) dengan monoterapi DFP dosis ≥ 90 mg/kgbb/hari. Metode: Penelitian ini merupakan penelitian retrospektif observasional dengan desain potong lintang, untuk menganalisis hubungan efektivitas terapi, efek samping obat dan biaya antara kelasi besi regimen kombinasi (DFO+DFP dan DFP+DFX) dengan monoterapi DFP dosis ≥ 90 mg/kgbb/hari. Luaran efektivitas dinilai dengan penurunan serum feritin ≥ 500 ng/mL.Hasil: Setelah 6 atau 12 bulan terjadi penurunan serum feritin pada 16 (34,7%) subyek kelompok kombinasi, dan 22 (27,5%) subyek kelompok monoterapi (p = 0,391). Sembilan (19,5%) subyek kombinasi mengalami efek samping obat, dan 17 (21,2%) subjek pada kelompok monoterapi (p = 0,822). Analisis minimalisisasi biaya menunjukkan bahwa rerata biaya per pasien thalassemia-β mayor anak yang menggunakan rejimen monoterapi selama 6 dan 12 bulan lebih murah Rp 13.556.592,64 (30,46%) dan Rp 20.162.836,10 (25,56%) dari rejimen kombinasi.Kesimpulan: Rejimen kombinasi sama efektifnya dengan rejimen monoterapi dalam menurunkan serum feritin. Tidak ada perbedaan efek samping obat yang bermakna diantara keduanya.

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Background: Blood transfusion is the main therapy for thalassemia major patients, but repeated transfusions are followed by new problems namely the excess iron load accumulated in the body tissue. Iron chelation therapy is the only way to maintain iron balance in the body.

Aim: This study aimed to determine the efficacy, safety , and cost analysis of of combination iron chelation regimen with mono-therapy.

Method:This study was designed as a retrospective observational study with a cross-sectional design, to analyze the relationship between therapeutic effectiveness, drug side effects and the cost of combination iron chelation regimen (DFO+DFP and DFP+DFX) and DFP mono-therapy dose ≥ 90 mg/kg/day. Outcome effectiveness was assessed by decreasing serum ferritin ≥ 500 ng/mL.

Result: After 6 or 12 months there was serum ferritin decreased in 16 (34,7%) subjects in combination group and 22 (27,5%) subjects in mono-therapy group (p = 0,391). Nine (19,5%) subjects in combination group experienced adverse effect, and 17 (21,2%) subjects in the mono-therapy group (p = 0,822). Analysis cost of minimization shows that the average cost per major thalassemia-β patient for children using a mono-therapy regimen for 6 and 12 months is cheaper Rp 13.556.592,64 (30,46%) and Rp 20.162.836,10 (25,56%) compared to combination regimen.

Conclusion: Combination regimens are as effective as a mono therapy regimens in decreasing serum ferritin. There were no significant differences in adverse effect between the two."

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

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

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

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

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

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

"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. "

"The most characteristic in clinical finding of thalassemia are anemia, facies Cooley, and enlargement of the spleen and liver. Osseous alterations occur in response to marrow overstimulation due to ineffective erythropoiesis these alterations result in changes of the skeletal architecture which is most typically reflected in the maxillofacial appearance of the patients. Uncontrolled maxillary overgrowth and procumebency of the anterior teeth make facial disfigurement (Asbell,1969).The therapy of thalassemia is focussed on treating the anemia by giving blood transfusions. It is believed that blood transfusion could prevent bone deformities (Karagiorga-Lagana 1988). Even though dentoskeletal deformity was found in many thalassemia children, not much attention has been given yet to the maxillofacial aspect. The effect but also in the alteration of mastication function. The condition of thalassemia children usually undernourish and anorexia caused by anemia. The dysfunction of mastication made the condition became worse. Therefore, it is necessary to prevent the deformity of dentoskeletal. The purpose of this study was to evaluate growth in thalassemia children.The result of this study revealed that dentoskeletal of thalassemia children in general had smaller size than normal, but not well proportion vertically caused by lower face. The skeletal profile was more convex than normal and indicated class II skeletal due to retruded mandible. The factor of age influenced all linear skeletal and dentoskeletal component, and only one angular skeletal components (SNB). the mean level of hemoglobin pretransfusion influenced the posterior cranial base, skelet of palatal, maxilla and posterior face of thalassemia children."

"Thalassemia merupakan salah satu kelainan genetik paling umum di seluruh dunia. Tanpa managemen yang adekuat, komplikasi akan terjadi pada berbagai organ, termasuk sistem saraf. Tujuan penelitian ini adalah untuk menentukan proporsi abnormalitas Brain Auditory Evoked Potentials (BAEP), elektroensefalografi (EEG), dan elektroneurografi (ENG) pada anak thalassemia mayor dan hubungannya dengan faktor risiko terkait. Metode: Penelitian ini merupakan studi potong lintang deskriptif analitik yang dilakukan di Rumah Sakit Cipto Mangunkusumo (RSCM) Jakarta dan Rumah Sakit M Djamil (RSMDJ) Padang. Kriteria inklusi adalah anak thalassemia mayor berusia 12-18 tahun yang kontrol teratur minimal dalam 1 tahun terakhir. Pasien dengan epilepsi, palsi serebral, gangguan pendengaran, dan gangguan neurodevelopmental dikeluarkan dari penelitian. Pemeriksaan BAEP, EEG, dan ENG dilakukan pada semua subyek dan diinterpretasikan oleh konsultan neuropediatri. Dilakukan pencatatan usia onset, durasi transfusi, rerata hemoglobin (Hb) pra-transfusi, kadar feritin serum, saturasi feritin, dan kepatuhan konsumsi obat kelasi besi. Hubungan antar variabel dinilai menggunakan analisis bivariat dengan nilai p < 0,05 dikatakan bermakna. Hasil: Sebanyak 64 anak dengan rerata usia 15,1 tahun memenuhi kriteria penelitian, terdiri atas 29 anak laki-laki dan 35 anak perempuan. Rerata Hb pra transfusi, kadar feritin serum, dan saturasi transferin berturut-turut adalah 8,36 g/dL, 4495,3 ng/mL, dan 87,3%. Abnormalitas EEG ditemukan pada 28 (43,8%) subyek dan berhubungan bermakna dengan rerata Hb pra-transfusi < 9 g/dL (p=0,011, rasio prevalensi 3,014, interval kepercayaan 1,04-8,71). Abnormalitas BAEP ditemukan pada 4 (4,6%) subyek dan berhubungan bermakna dengan kadar feritin serum yang lebih tinggi (p=0,007). Hasil ENG abnormal hanya ditemukan pada 1 orang subyek. Tidak terdapat hubungan antara faktor risisko lainnya dengan masing-masing pemeriksaan neurofisiologi. Kesimpulan: Abnormalitas EEG ditemukan pada 43,8% anak thalassemia mayor dan berhubungan dengan rerata Hb pra-transfusi <9 g/dL, sedangkan abnormalitas BAEP ditemukan pada 4% subyek dan berhubungan dengan kadar feritin serum yang lebih tinggi.

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Thalassemia is among the most common genetic disorders worldwide. Without adequate management, complications occur in various organs as well as neurology system. The aim of the study was to determine the proportion of abnormal electroencephalography, brain auditory evoked potentials (BAEP), and nerve conduction study (NCS) in children with thalassemia major and its association with related risk factors. Methods: This was a descriptive-analytic cross sectional study conducted in Cipto Mangunkusumo Hospital and M Djamil Hospital in January to March 2019 All children with thalassemia major aged 12 to 18 years were eligible for the study. Children with epilepsy, palsy cerebral, hearing disorder, or neurodevelopmental problems were excluded. Electroencephalography, BAEP, and NCS were performed in all subjects. Age of onset, transfusion duration, mean pre-transfusion hemoglobin, serum ferritin, transferrin saturation, and compliance to chelating agents therapy were recorded. Bivariate analysis was performed to determine the relationsip between variables with p < 0,05 was considered significant. Results: As many as 64 children with mean age 15,1 years fulfilled the study criteria during the study period, consisting of 29 boys and 35 girls. Mean pre-transfusion hemoglobin, serum ferritin, and transferrin saturation was 8,36 g/dL, 4495,3 ng/mL, and 87,3% respectively. Abnormal EEG was found in 28 (43,8%) subjects and significantly associated with mean Hb below 9 g/dL (p = 0,011; prevalence ratio 3,014; confidence interval 1,04 - 8,71). Abnormal BAEP was found in 4 (4,6%) subjects and significantly associated with higher serum ferritin (p=0,007). Only 1 subject showed abnormal NCS. No association was found between other risk factors with each neurophysiology study. Conclusion: Abnormal EEG was found in 43,8% thalassemia major children and significantly associated with lower pre-transfusion hemoglobin level. Abnormal BAEP was found in 4% subjects and significantly associated with higher serum ferritin."

"Latar belakang: Pandemi COVID-19 menyebabkan anak tetap tinggal di rumah untuk menjalani pembelajaran jarak jauh (PJJ). Hal ini dapat berdampak kepada peningkatan paparan screen time anak yang melebihi anjuran. Lebih lanjut hal ini dapat berpotensi terjadinya gangguan tidur. Pada anak dengan thalassemia, yang memiliki beberapa penyulit, dapat semakin meningkatkan risiko gangguan tidur tersebut sehingga akan berdampak terhadap tumbuh kembang anak. Metode: Penelitian ini menggunakan desain potong lintang dengan menggunakan instrumen Sleep Disturbance Scale for Children (SDSC) versi Bahasa Indonesia. Pengambilan sampel dilakukan secara consecutive sampling pada orang tua pasien thalassemia yang berada di Poli Hematologi dan Ruang Transfusi RSCM Kiara. Hasil: Dari 93 data yang diperoleh, sebanyak 85 data yang memenuhi kriteria inklusi dan eksklusi yang kemudian dianalisis. Subjek didominasi oleh kelompok usia sekolah dengan rentang usia 6-15 tahun (51,2%) dan berjenis kelamin laki-laki (57,6%). Sebanyak 57 dari 85 subjek memiliki tingkat screen time yang tinggi. Dengan menggunakan kuesioner SDSC didapatkan juga 50 dari 85 subjek mengalami gangguan tidur dengan hiperhidrosis saat tidur menjadi faktor gangguan tidur terbanyak (26%). Terdapat hubungan bermakna antara screen time dan gangguan tidur (p=0,01). Pasien anak thalassemia dengan screen time tinggi (lebih dari 120 menit) memiliki peluang untuk mengalami gangguan tidur 3,35 kali lebih tinggi dibandingkan dengan pasien thalassemia yang tidak memiliki screen time tinggi (OR = 3,35 dan CI 95% = 1,31-8,59). Kesimpulan: Terdapat hubungan antara screen time dan gangguan tidur pada pasien thalassemia sehingga perlu dilakukan edukasi dan pembatasan screen time.

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Introduction: The COVID-19 pandemic has caused children to stay at home and undergo distance learning. This situation can have an impact on increasing exposure to child screen time exceeding the recommendation. Furthermore, it can potentially lead to sleep disturbances. Especially for children with thalassemia, having complications, be able to increase the risk of these sleep disturbances that will increasingly impact the child's development. Method: This study used a cross-sectional design using instruments Sleep Disturbance Scale for Children (SDSC) Indonesian version. Subject selection is done by consecutive sampling in parents of thalassemia patients in the Hematology Poly and Transfusion Room of RSCM Kiara. Result: Of the 93 data obtained, 85 data met the inclusion and exclusion criteria which were then analyzed. Subjects were dominated by the school age group with an age range of 6-15 years (51.2%) and were male (57.6%). As many as 57 out of 85 subjects have level screen time tall one. Using the SDSC questionnaire, it was also found that 50 out of 85 subjects experienced sleep disturbances with hyperhidrosis during sleep being the most common sleep disturbance factor (26%). There was a significant relationship between screen time and sleep disturbance (p=0.01). Thalassemia pediatric patients with high screen time (more than 120 minutes) had a 3.35 times higher chance of experiencing sleep disturbances compared to thalassemia patients who did not have high screen time (OR = 3.35 and 95% CI = 1.31- 8,59). Conclusion: In conclusion, this study recommends education and screen time restriction be needed for children with thalassemia to reduce the chance of sleep disturbances."

"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. Penelitian ini bertujuan menilai gambaran struktur dan fungsi retina serta menilai hubungan antara durasi terapi kelasi besi dan kadar feritin serum dengan abnormalitas struktur retina pada penyandang thalasemia-β mayor yang memperoleh terapi kelasi besi di RSCM. Metode. Penelitian potong lintang ini dilakukan pada penyandang thalasemia-β mayor berusia di atas 10 tahun yang memperoleh terapi kelasi besi dan menjalani kontrol di Pusat Thalasemia RSCM. Subjek dilakukan pemeriksaan oftalmologis, foto fundus, dan fundus autofluorescence. Selanjutnya dilakukan pengambilan subsampel dari subjek awal berdasarkan hasil fundus autofluorescence dan dilakukan pemeriksaan elektroretinografi multifokal dan elektrookulografi. Hasil. Abnormalitas struktur retina didapatkan pada 46,2% subjek sedangkan abnormalitas pemeriksaan fundus autofluorescence didapatkan pada 41,9% subjek. Sebagian besar subjek memiliki tajam penglihatan dan sensitivitas kontras yang normal. Nilai tengah seluruh parameter elektroretinografi multifokal dan rasio amplitudo light peak terhadap dark trough elektrookulografi kedua kelompok subjek berada dalam rentang normal. Didapatkan penurunan sensitivitas kontras yang signifikan pada subjek dengan abnormalitas struktur retina dan makula, namun tidak untuk tajam penglihatan. Kadar feritin serum yang lebih tinggi berhubungan dengan abnormalitas struktur retina. Kesimpulan. Rerata kadar feritin serum dalam periode satu tahun dengan titik potong ≥6.000 ng/ml dapat digunakan sebagai panduan untuk memulai pemeriksaan struktur dan fungsi retina.

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Introduction. This study aims to evaluate retinal structure and function and association between iron chelation treatment duration and serum ferritin level with retinal structure abnormality in β-thalassemia major patients treated with iron-chelating agent in Cipto Mangunkusumo Hospital. Methods. This cross-sectional study was performed on β-thalassemia major patients aged more than 10 years old in Thalassemia Center, Cipto Mangunkusumo Hospital, who received iron-chelating agent for at least one year. Patients underwent ophthalmologic examination, fundus photography, and fundus autofluorescence imaging. Afterwards subsample was chosen based on fundus autofluorescence imaging result, and underwent multifocal electroretinography and electrooculography examination. Results. Retinal structure abnormality was found in 46.2% patients and fundus autofluorescence abnormality in 41.9% patients. The majority of patients had normal visual acuity and contrast sensitivity. Each multifocal electroretinography parameters and light peak to dark trough amplitude ratio in electrooculography had normal median values. Significant contrast sensitivity reduction was found on patients with retinal and macular structure abnormality, but not for visual acuity. Significant association between higher ferritin serum level and retinal structure abnormality was found. Conclusion. Mean ferritin serum level within one year with cutoff point of ≥6.000 ng/ml can be used as a guide to start retinal structure and function evaluation."

"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."

"ABSTRAKLatar belakang: Talasemia merupakan penyakit kronis yang dapat menganggu kualitas hidup anak, baik karena keparahan penyakitnya maupun pengobatannya yang bersifat jangka panjang. Transfusi darah dan terapi kelasi besi yang diberikan seumur hidup pada anak dengan talasemia memberikan harapan hidup yang sama dengan anak sehat. Kualitas hidup menjadi hal yang penting dengan bertambahnya angka harapan hidup pasien talasemia. Tujuan: Mengetahui kualitas hidup anak dengan talasemia di Pusat Thalassemia departemen ilmu kesehatan anak FKUI-RSCM, Rumah Sakit Umum Daerah Tangerang, dan Rumah Sakit Ibu dan Anak Harapan Kita serta faktor-faktor yang berhubungan. Metode: Penelitian menggunakan rancangan deskriptif dengan analisis potong lintang. Pengambilan sampel dilakukan secara konsekutif pada bulan Januari ndash; Maret 2017. Subyek penelitian adalah anak berusia 2-18 tahun yang datang selama periode penelitian. Penilaian kualitas hidup menggunakan kuesioner baku PedsQL trade; modul generik yang diisi oleh orangtua pasien. Analisis data dengan metode univariat dengan tingkat kemaknaan ?=0,05. Hasil: Terdapat 387 subyek yang mengikuti penelitian, sebaran subyek merata. Nilai rerata total kualitas hidup anak dengan talasemia adalah 76,88 12,92 dengan nilai total pada masing-masing rumah sakit berturut-turut adalah 77,54 RSCM, 81,3 RS Harapan Kita, dan 75,18 RSUD Tangerang. Faktor sosiodemografis dan faktor medis tidak memiliki hubungan bermakna dengan kualitas hidup. Tetapi, seiring bertambahnya usia, kualitas hidup anak semakin menurun, terutama pada domain fungsi sekolah. Simpulan: Lebih dari separuh anak dengan talasemia 72,8 memiliki kualitas hidup yang baik, namun pada domain fungsi sekolah masih memiliki nilai yang rendah.

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ABSTRACTBackground Thalassemia is a chronic condition and affect patient's health related quality of life HRQoL , because of the disease and the effect of treatment. Blood transfusion and chelating agent given for thalassemia children in a lifetime could give a good quality of life compared to healthy children. Assesment is needed to determine and to improve the quality of life in thalassemic patient. Aim To assess the life quality of children with thalassemia in Cipto Mangunkusumo Hospital, Harapan Kita Hospital, and Tangerang Public Hospital as well as affecting factors contributing on it. Method We performed a cross sectional study from January ndash March 2017 in Cipto Mangunkusumo Hospital, Harapan Kita Hospital, and Tangerang Public Hospital. Thalassemia children aged 2 18 years were involved, parents were asked to fill out the PedsQL trade generic score scale version 4.0 questionnaire parent proxy report to assess their quality of life. Result Of the 387 thallasemia patients approached, the distribution of the subject was evenly distributed. The overall mean total score for quality of life in children with thalassemia were 76,88 12,92 each hospital was 77,54 Cipto Mangunkusumo Hospital, 81,3 Harapan Kita Hospital, and 76,88 Tangerang Public Hospital. While The sosiodemographic and medical factors were not significantly affect the HRQoL of the patients. The study reveal that thalassaemia has different impact at different ages especially in school function. Conclusion Thalassemic children have a good quality of life in general 72,8 but the school function still have a low score.Key words thalassemia, children, quality of life, PedsQL."