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Abstrak :
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. ......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.

Abstrak :
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.

Abstrak :
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 Indonesia Objective. 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.

Abstrak :
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. ......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.

Abstrak :
ABSTRACT
Latar 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.

Abstrak :
Praktik Kerja Profesi Apoteker di Rumah Sakit Umum Pusat (RSUP) Fatmawati dan Apotek Roxy Klender Periode Bulan Agustus – November Tahun 2020 bertujuan untuk mengetahui tugas dan tanggung jawab apoteker, menambah wawasan, pengetahuan dan keterampilan, serta pengalaman praktis untuk melakukan praktik kefarmasian di pelayanan kefarmasian seperti rumah sakit dan apotek. Selain itu, peserta praktik kerja profesi juga mendapat gambaran nyata tentang permasalahan praktik kefarmasian, serta dapat mempelajari strategi dan kegiatankegiatan yang dapat dilakukan dalam rangka pengembangan praktik. Tujuan pengerjaan tugas khusus ini adalah untuk melakukan Pemantauan Terapi Obat (PTO) pada pasien thalassemia anak dan menganalisis pola penyakit berdasarkan resep yang masuk di Apotek Roxy Klender. Secara umum, penggunaan obat pada pasien thalassemia anak di RSUP Fatmawati kurang efektif dan rasional karena ditemukannya drug related problems (DRPs) yaitu adanya indikasi tanpa terapi (kadar Hb pra-transfusi < 9 gr/dL) dan berdasarkan resep yang masuk diketahui pola penyakit yang sering terjadi di Apotek Roxy Klender per tanggal 1-10 Oktober 2020 yaitu infeksi bakteri (16,54%), nyeri + inflamasi (13,85%), gangguan saluran cerna (10,77%), penyakit kulit (8,85%), hipertensi (6,92%), alergi (5,77%), kardiovaskular (5%), diabetes (5%), hiperlipidemia (5%), dan syaraf (4,62%) Penulis telah mendapatkan untuk memahami peran, tugas, wawasan dan tanggung jawab apoteker baik dalam kegiatan manajemen sediaan farmasi dan kegiatan farmasi klinis yang terdapat di rumah sakit dan apotek. ......Internship at Rumah Sakit Umum Pusat Fatmawati, and Apotek Roxy Klender Period August - November 2020 aims to find out the duties and responsibilities of pharmacists, gain insight, knowledge and skills, as well as practical experience to carry out pharmaceutical practices in pharmaceutical services such as hospitals and pharmacies. In addition, participants in professional work practices also get a real picture of the problems of pharmaceutical practice, and can learn strategies and activities that can be carried out in the context of practical development. The purpose of this particular task is to carry out Drug Therapy Monitoring (PTO) in pediatric thalassemia patients and to analyze disease patterns based on the prescription that was received at the Apotek Roxy Klender. In general, the use of drugs in pediatric thalassemia patients at Rumah Sakit Umum Pusat Fatmawati is less effective and rational because of the discovery of drug related problems (DRPs), namely the indication without therapy (pre-transfusion Hb level <9 g / dL) and based on the incoming prescription, the disease pattern was found. often occurs at the Apotek Roxy Klender as of 1-10 October 2020, namely bacterial infections (16.54%), pain + inflammation (13.85%), gastrointestinal disorders (10.77%), skin diseases (8.85%), hypertension (6.92%), allergies (5.77%), cardiovascular (5%), diabetes (5%), hyperlipidemia (5%), and neurological (4.62%). , duties, insights and responsibilities of pharmacists both in the management of pharmaceutical preparations and clinical pharmacy activities in hospitals and pharmacies.

Abstrak :
Talasemia merupakan penyakit genetik dimana terjadi kelainan pada sel darah merah akibat kesalahan produksi hemoglobin. Perkawinan antara sesama pembawa gen talasemia dapat menyebabkan munculnya keturunan yang menderita talasemia mayor. Talasemia mayor merupakan jenis talasemia yang dapat menyebabkan kematian. Oleh karena itu, pendeteksian dini terhadap penyakit talasemia merupakan hal yang penting. Salah satu cara untuk melakukan pendeteksian talasemia adalah dengan menggunakan machine learning. Pada skripsi ini, algoritma KC-Means (KCM) clustering digunakan untuk memprediksi talasemia. Kemudian performa algoritma tersebut dibandingkan dengan algoritma Kernel KC-Means (KKCM) clustering, dimana fungsi kernel yang digunakan adalah kernel Gaussian Radial Basis Function (RBF) dan polinomial. Pengujian kedua algoritma tersebut dilakukan dengan menggunakan hold-out evaluation dan 5-fold cross validation. Data yang digunakan adalah data talasemia yang berasal dari Rumah Sakit Harapan Kita, Jakarta, Indonesia. Data talasemia tersebut terdiri dari 150 sampel dengan komposisi 82 sampel talasemia dan 68 sampel non-talasemia, yang tiap sampelnya memiliki 11 fitur. Berdasarkan percobaan yang dilakukan, algoritma KCM dan KKCM menghasilkan prediksi dengan akurasi, sensitivitas, presisi, spesifisitas, dan F1-Score yang sama, yaitu di atas 96 persen. Namun, waktu komputasi yang dibutuhkan oleh KKCM dapat mencapai hingga 10.25 kali lebih cepat dari waktu komputasi KCM....... Thalassemia is a genetic disease in which there are abnormalities in red blood cells due to an error in the production of hemoglobin. Marriage between thalassemia carriers can cause the birth of a child suffering from thalassemia major. Thalassemia major is a type of thalassemia that can cause death. Therefore, early detection of thalassemia is essential. One of the techniques to detect thalassemia is to use machine learning. In this thesis, the KC-Means (KCM) clustering algorithm is used to predict thalassemia. Then the performance of the algorithm is compared with the KC-Means Kernel (KKCM) clustering algorithm, where the kernel functions used are the Gaussian Radial Base Function (RBF) and polynomial kernel. Evaluation of those algorithms is carried out using hold-out evaluation and 5-fold cross-validation. The data used is thalassemia data from Harapan Kita Hospital, Jakarta, Indonesia. The data consists of 150 samples with a composition of 82 thalassemia samples and 68 non-thalassemia samples; each has 11 features. Based on the experiments, the KCM and KKCM algorithms make predictions with the same accuracy, sensitivity, precision, specificity, and F1-Score, which is above 96 percent. However, the computing time needed by KKCM can reach up to 10.25 times faster than the KCM computing time.

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

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

Abstrak :
Ansietas merupakan masalah psikososial yang dialami oleh anak dengan thalasemia, dan berdampak terhadap fungsi emosional anak. 56,52% anak usia sekolah dengan thalasemia di Rumah Sakit Sumedang mengalami masalah fungsi emosional. Penelitian ini bertujuan untuk mengetahui pengaruh Cognitive Behavior Play Therapy terhadap ansietas dan kemampuan mengatasi ansietas. Desain penelitian menggunakan Quasi experimental pre-post test non equivalent control group, jumlah sampel 42 orang kelompok intervensi dan 43 orang kelompok kontrol dengan consecutive sampling. Analisis data menggunakan uji t test. Hasil penelitian menunjukkan bahwa ansietas klien yang mendapat cognitive behavior play therapy menurun lebih besar secara bermakna dari ansietas berat menjadi tidak ansietas jika dibandingkan dengan kelompok yang tidak mendapatkan cognitive behavior play therapy. Kemampuan mengatasi ansietas klien yang mendapat cognitive behavior play therapy meningkat lebih tinggi secara bermakna dari kemampuan cukup menjadi kemampuan baik jika dibandingkan dengan kelompok yang tidak mendapatkan cognitive behavior play therapy. Faktor yang berkontribusi terhadap kemampuan mengatasi ansietas adalah usia dan lama sakit. Cognitive Behavior Play Therapy direkomendasikan untuk diberikan pada anak dengan masalah psikososial akibat penyakit fisik sebagai terapi spesialis. ......Anxiety is a psychosocial problems experienced by children with thalassemia. Anxiety affects child’s emotional function and 56,52% of school-age children with thalasemia who is admitted in Sumedang General Hospital experienced poor emotional function. The aims of this study is to determine the effect of Cognitive Behavior Play Therapy (CBPT) for reducing anxiety and uncreasing ability to cope with anxiety. This study used Quasi-experimental research design with prepost- test non-equivalent control group. Number of sample was 85 school-age children, taken by consecutive sampling technique and devided into two groups (42 children in intervention group and 43 children in control group). Data analysis used t test. The results showed that anxiety level in patient who received CBPT were significantly decreased from severe to less anxiety compared to those who did not receive CBPT. Ability to cope with anxiety in patient who received CBPT was signficantly increase better than the group who did not receive CBPT. Factors that contribute to the ability to cope with anxiety are age and period of illness. This research suggests for implementing CBPT in children with psychosocial problems due to physical illness as a specialist therapy.