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

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

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ABSTRAK
Latar Belakang: Muatan besi berlebih akibat transfusi darah dan peningkatan serapan besi di saluran cerna, masih merupakan isu penting pada Thalasemia Intermedia TI , dan dikaitkan dengan berbagai komplikasi yang meningkatkan morbiditas dan mortalitas. Pemeriksaan MRI T2 hati sebagai pemeriksaaan yang tervalidasi dalam menilai Liver Iron Concentration LIC , merupakan pemeriksaan yang mahal dan tidak tersedia secara merata di Indonesia.Tujuan: Mengetahui gambaran muatan besi berlebih darah dan hati pada pasien thalasemia intermedia dewasa dan korelasi antara saturasi transferin, feritin serum, MRI T2 hati, dan LIC yang dinilai dengan pemeriksaan MRI T2 hati dengan nilai elastografi hati.Metode: Penelitian potong lintang pada pasien thalasemia intermedia dewasa dengan transfusi darah dan dengan atau tanpa kelasi besi di RSUPNCM dr. Cipto Mangunkusumo mulai dari bulan Agustus sampai dengan Oktober 2016, dengan total subyek penelitian sebanyak 45 orang. Dilakukan pemeriksaan saturasi transferin, feritin serum, elastografi hati, dan MRI T2 hati. Analisis statistik menggunakan uji korelasi Pearson dan Spearman.Hasil: Sebanyak 64,4 subyek penelitian adalah Thalasemia ?-Hb E, dengan median RIK umur 33 22 tahun. Sebanyak 84,4 subyek penelitian mendapatkan transfusi darah secara reguler. Seluruh subyek penelitian mengalami komplikasi hemosiderosis hati melalui pemeriksaan MRI T2 hati. Sebanyak 48,9 mengalami hemosiderosis hati berat, dengan nilai median MRI T2 hati 1,6 ms. rerata feritin serum adalah 2831 1828 ng/mL, dengan nilai median saturasi transferin 66 . Nilai rerata LIC melalui pemeriksaan MRI T2 adalah 15,36 7,37 mg besi/gr berat kering hati dan nilai rerata elastografi hati adalah 7,7 3,8 Kpa. Uji korelasi didaptakan terdapat korelasi nilai elastografi hati dan rerata feritin serum r = 0,651; p = 0,000 , nilai elastografi hati dan LIC r = 0,433; p = 0,003 dan korelasi negatif nilai elastografi hati dan MRI T2 hati r = -0,357; p = 0,016 .Simpulan: Terdapat korelasi antara muatan besi berlebih feritin serum dan LIC dengan nilai elastografi hati. Terdapat korelasi negatif nilai elastografi hati dengan MRI T2 hati pada pasien thalasemia intermedia dewasa.

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ABSTRACT
Background Iron overload is a common feature of thalassemia intermedia due to regular blood transfusion and increase gastrointestinal iron absorption. Early detection and adequate iron chelator can significantly decrease related morbidities and mortality due to complication from iron overload. Liver Iron Concentration LIC is the best way to measure body iron stores. MRI T2 as a validated test to identify LIC, is expensive and currently not available in all medical services in Indonesia.Objective To identify liver iron overload and correlation of transferrin saturation, serum ferritin, liver MRI T2 , and LIC with transient liver elastography in adult thalassemia intermedia patient.Methods We conducted a cross sectional study enrolling 45 patients with thalassemia intermedia with blood transfusion and with and without iron chelator therapy. The study was conducted at Cipto Mangunkusumo Hospital from August 2016 through October 2016. We performed measurements of transferrin saturation, serum ferritin level, transient liver elastography and liver MRI T2 . The Pearson and Spearman correlation test were used to evaluate the correlation transient liver elastography with transferrin saturation, serum ferritin, Liver MRI T2 , and LIC.Results As much as 64,4 of study subject are Hb E Thalasemia Intermedia with median IQR age is 33 22 years old. As much as 84,4 of study subject have regular blood transfusion. On the basis of liver MRI T2 , all studi subject suffered from liver iron overload, with 48,9 had severe liver iron overload. The median value of Liver MRI T2 was 1,6 ms. The mean serum ferritin was 2831 1828 ng mL, with median value of transferrin saturation was 66 . The mean of LIC corresponding to Liver MRI T2 and mean liver stiffness measurement was 15,36 7,37 mg Fe gr dry weight and 7,7 3,8 Kpa respectively. Liver Stiffness correlated with serum ferritin r 0,651 p 0,000 , Liver MRI T2 r 0,357 p 0,016 , and LIC r 0,433 p 0,003 . No correlation was found between liver elastography and transferrin saturation r 0,204 p 0,178 .Conclusions Serum ferritin, Liver MRI T2 , and LIC correlated with liver elastography. No correlation was found between transferin saturation and liver elastography.

Abstrak :
Talasemia merupakan penyakit autosomal resesif yang menyebabkan tubuh tidak mampu memproduksi hemoglobin (Hb) secara normal, sehingga penderitanya membutuhkan transfusi darah seumur hidup. Skrining genetik bagi pasangan yang akan menikah merupakan langkah awal untuk menekan angka bayi lahir dengan gen talasemia. Namun, perhatian masyarakat masih rendah karena skrining ini tidak termasuk ke dalam prosedur pra-nikah yang dapat ditanggung oleh Jaminan Kesehatan Nasional (JKN), serta harganya cukup mahal. Penelitian ini memanfaatkan machine learning untuk memprediksi carrier dan mengklasifikasikan jenis talasemia berdasarkan hasil tes hematologi lengkap/Complete Blood Count (CBC) yang memiliki harga lebih terjangkau dari skrining genetik. Pada penelitian, digunakan beberapa algoritma pembelajaran mesin bersifat supervised classification seperti Logistic Regression, Random Forest, Support Vector Machine, Gradient Boosting, XGBoost, dan AdaBoost. Hasil menunjukkan penggunaan Support Vector Machine dengan oversampling menggunakan synthetic minority oversampling technique edited nearest neighbors (SMOTE-ENN), normalisasi dengan RobustScaler, hyperparameter tuning, dan 10-fold cross-validation berhasil mencapai nilai akurasi 98.84% dalam mengklasifikasikan carrier talasemia alfa berdasarkan hasil CBC. ......Thalassemia is an autosomal recessive disease that unable the body to produce hemoglobin (Hb) normally, requiring lifelong blood transfusions. Genetic screening for future married couples is the first step to reduce the number of babies born with the thalassemia gene. However, public attention is still low because the screening is not included in the pre-marital procedures that can be covered by the Jaminan Kesehatan Nasional (JKN), despite the price is quite expensive. This study utilizes machine learning to predict the carrier and classify the type of alpha-thalassemia based on the results of the Complete Blood Count (CBC) test, which is more affordable than genetic screening. In the study, several supervised classification machine learning algorithms were utilized such as Logistic Regression, Random Forest, Support Vector Machine, Gradient Boosting, XGBoost, and AdaBoost. The results show the use of Support Vector Machine with oversampling with synthetic minority oversampling technique edited nearest neighbors (SMOTE-ENN), normalization with RobustScaler, hyperparameter tuning, and 10-fold cross-validation successfully achieved 98.84% accuracy in classifying alpha thalassemia carriers based on CBC results.

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ABSTRAK
Pendahuluan: Terapi transfusi darah rutin pada pasien thalassemia berisko menyebabkan hemokromatosis sekunder pada tubuh. Hemokromtasosis sekunder, yang diukur dengan kadar ferritin serum, dapat menyebabkan gangguan fungsi hati yang berdampak pada keterlambatan pertumbuhan atau stunting. Tujuan penelitian ini adalah untuk mengetahui presentase pasien thalassemia anak di RSCM Kiara yang mengalami stunting, melihat hubungan antara kadar ferritin serum dengan stunting, kadar hemoglobin dengan stunting, dan nilai hematokrit dengan stunting. Metode: Dari 285 data pasien thalassemia di RSCM Kiara, diambil 109 data pasien yang memenuhi kriteria untuk penelitian ini. Data tersebut terdiri dari 55 anak laki-laki dan 54 anak perempuan dengan rentang usia 0-18 tahun. Hasil: Dari 109 pasien, terdapat 48,7 53/109 pasien yang memiliki kondisi stunting. Kelompok stunting tersebut terdiri dari 40 22/55 anak laki laki dan 54,7 31/54 anak perempuan. Bila digunakan kadar ferritin serum ng/dL dengan cut off 1000, 2000, 3000, 4000 sebagai batas cut off untuk menyatakan adanya hemokromatosis, tidak ditemukan adanya hubungan p>0,05 . Namun, terdapat perbedaan signifikan p.

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ABSTRACT
Introduction Routine blood transfusion in thalassemia patient is commonly reported on causing secondary hemochromatosis. Untreated secondary hemochromatosis, which is measure with serum ferritin level, can lead to liver hemosiderosis which directly responsible for delay growth or stunting. The aim of this study is to know the percentage of stunting condition in RSCM Kiara thalassemia patient and if there any correlation between secondary hemochromatosis, hemoglobin, and hematocrit with stunting. Methode From 285 thalassemia patient data in RSCM Kiara, 109 were taken as a subject in this research. It is consisted of 55 boys and 54 girls with age range from 0 18 years. Result From 109 patients, there are around 48.7 53 109 patient who shown stunting condition. It is consisted 40 22 55 boys and 57.4 31 54 girls. If serum ferrtin level ng dL cut off point 1000, 2000, 3000, 4000 is used to determined condition of hemochromatosis, it was found to have no significant relation p 0.05 with stunting, However, there are a significant different p

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[ABSTRAK
Anemia defisiensi besi dan thalassemia β trait merupakan penyebab tersering anemia mikrositik hipokrom di Indonesia. Kedua penyakit tersebut sulit dibedakan hanya dengan pemeriksaan hematologi, oleh karena itu diperlukan pemeriksaan tambahan seperti feritin dan analisis hemoglobin. Namun tidak semua laboratorium dapat melakukan pemeriksaan ini. Banyak penelitian yang membedakan kedua penyakit tersebut dengan indeks eritrosit. Namun indeks eritrosit memiliki nilai diagnostik yang berbeda di setiap negara dan belum ada data di Indonesia. Penelitian ini melakukan uji diagnostik Indeks Mentzer, RDW, Green- King, Sirdah, dan mencari nilai cut-off baru yang memberikan nilai diagnostik lebih baik. Penelitian terdiri dari 98 subyek definitif anemia defisiensi besi dan 80 subyek thalassemia β trait. Nilai diagnostik Indeks Mentzer untuk anemia defisiensi besi adalah sensitivitas 83.6%, spesifisitas 66.2%, NPP 75.2%, NPN 76.8%, RKP 2.4, RKN 0.2. Nilai diagnostik Indeks Mentzer untuk thalassemia β trait adalah sensitivitas 66.2%, spesifisitas 83.6%, NPP 76.8%, NPN 75.2%, RKP 4.0, RKN 0.4. Nilai diagnostik Indeks RDW untuk anemia defisiensi besi adalah sensitivitas 91.8%, spesifisitas 75%, NPP 81.8%, NPN 88.2%, RKP 3.6, RKN 0.1. Nilai diagnostik Indeks RDW untuk thalassemia β trait adalah sensitivitas 75%, spesifisitas 91.8%, NPP 88.2%, NPN 81.8%, RKP 9.1, RKN 0.2. Nilai diagnostik Indeks Green-King untuk anemia defisiensi besi adalah sensitivitas 96.9%, spesifisitas 67.5%, NPP 78.5%, NPN 94.7%, RKP 2.9, RKN 0.04. Nilai diagnostik Indeks Green-King untuk thalassemia β trait adalah sensitivitas 67.5%, spesifisitas 96.9%, NPP 94.7%, NPN 78.5%, RKP 22.0, RKN 0.3. Nilai diagnostik Indeks Sirdah untuk anemia defisiensi besi adalah sensitivitas 92.8%, spesifisitas 58.7%, NPP 73.3%, NPN 87.0%, RKP 2.2, RKN 0.1.Nilai diagnostik Indeks Sirdah untuk thalassemia β trait adalah sensitivitas 58.7%, spesifisitas 92.8%, NPP 87.0%, NPN 73.3%, RKP 8.2, RKN 0.4. Nilai cut-off baru Indeks Mentzer adalah 13.44, RDWI 233.4, Green-King 75.06, dan Sirdah 32.52. Keempat indeks eritrosit dapat diaplikasikan untuk orang Indonesia dengan Indeks Green-King sebagai indeks yang terbaik.

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ABSTRACT
Iron deficiency anemia and β trait thalassemia are the most common causes of microcytic hypochromic anemia in Indonesia. Differentiation between them is difficult when solely based on a hematology examination, so additional laboratory tests are required such as ferritin and hemoglobin analysis. However, not all laboratories can perform these tests. Many erythrocytes indices have been proposed to determine whether a blood sample is more suggestive for iron deficiency anemia or β trait thalassemia. Unfortunately these indices have different diagnostic value in many countries and there is no data about diagnostic value in Indonesia. This study performs diagnostic test Mentzer, RDW, Green-King, and Sirdah Index and develops a new cut-off point that could make a better diagnostic value. This study consists of 98 subjects of iron deficiency anemia and 80 subjects of β trait thalassemia. Diagnostic values of Mentzer Index for iron deficiency anemia were sensitivity 83.6%, specificity 66.2%, PPV 75.2%, NPV 76.8%, LR+ 2.4, LR- 0.2. Diagnostic values of Mentzer Index for β trait thalassemia were sensitivity 66.2%, specificity 83.6%, PPV 76.8%, NPV 75.2%, LR+ 4.0, LR- 0.4. Diagnostic values of RDW Index for iron deficiency anemia were sensitivity 91.8%, specificity 75%, PPV 81.8%, NPV 88.2%, LR+ 3.6, LR- 0.1. Diagnostic values of RDW Index for β trait thalassemia were sensitivity 75%, specificity 91.8%, PPV 88.2%, NPV 81.8%, LR+ 9.1, LR- 0.2. Diagnostic values of Green- King Index for iron deficiency anemia were sensitivity 96.9%, specificity 67.5%, PPV 78.5%, NPV 94.7%, LR+ 2.9, LR- 0.04. Diagnostic values of Green-King Index for β trait thalassemia were sensitivity 67.5%, specificity 96.9%, PPV 94.7%, NPV 78.5%, LR+ 22.0, LR- 0.3. Diagnostic values of Sirdah Index for iron deficiency anemia were sensitivity 92.8%, specificity 58.7%, PPV 73.3%, NPV 87.0%, LR+ 2.2, LR- 0.1. Diagnostic values Sirdah Index for β trait thalassemia were sensitivity 58.7%, specificity 92.8%, PPV 87.0%, NPV 73.3%, LR+ 8.2, LR- 0.4. The new cut-off point of Mentzer, RDW, Green-King, and Sirdah Index was 13.44, 233.4, 75.06, and 32.52 respectively. All indices can be applied for Indonesian people, among which Green-King Index had the best diagnostic value, Iron deficiency anemia and β trait thalassemia are the most common causes of microcytic hypochromic anemia in Indonesia. Differentiation between them is difficult when solely based on a hematology examination, so additional laboratory tests are required such as ferritin and hemoglobin analysis. However, not all laboratories can perform these tests. Many erythrocytes indices have been proposed to determine whether a blood sample is more suggestive for iron deficiency anemia or β trait thalassemia. Unfortunately these indices have different diagnostic value in many countries and there is no data about diagnostic value in Indonesia. This study performs diagnostic test Mentzer, RDW, Green-King, and Sirdah Index and develops a new cut-off point that could make a better diagnostic value. This study consists of 98 subjects of iron deficiency anemia and 80 subjects of β trait thalassemia. Diagnostic values of Mentzer Index for iron deficiency anemia were sensitivity 83.6%, specificity 66.2%, PPV 75.2%, NPV 76.8%, LR+ 2.4, LR- 0.2. Diagnostic values of Mentzer Index for β trait thalassemia were sensitivity 66.2%, specificity 83.6%, PPV 76.8%, NPV 75.2%, LR+ 4.0, LR- 0.4. Diagnostic values of RDW Index for iron deficiency anemia were sensitivity 91.8%, specificity 75%, PPV 81.8%, NPV 88.2%, LR+ 3.6, LR- 0.1. Diagnostic values of RDW Index for β trait thalassemia were sensitivity 75%, specificity 91.8%, PPV 88.2%, NPV 81.8%, LR+ 9.1, LR- 0.2. Diagnostic values of Green- King Index for iron deficiency anemia were sensitivity 96.9%, specificity 67.5%, PPV 78.5%, NPV 94.7%, LR+ 2.9, LR- 0.04. Diagnostic values of Green-King Index for β trait thalassemia were sensitivity 67.5%, specificity 96.9%, PPV 94.7%, NPV 78.5%, LR+ 22.0, LR- 0.3. Diagnostic values of Sirdah Index for iron deficiency anemia were sensitivity 92.8%, specificity 58.7%, PPV 73.3%, NPV 87.0%, LR+ 2.2, LR- 0.1. Diagnostic values Sirdah Index for β trait thalassemia were sensitivity 58.7%, specificity 92.8%, PPV 87.0%, NPV 73.3%, LR+ 8.2, LR- 0.4. The new cut-off point of Mentzer, RDW, Green-King, and Sirdah Index was 13.44, 233.4, 75.06, and 32.52 respectively. All indices can be applied for Indonesian people, among which Green-King Index had the best diagnostic value]

Abstrak :
Model pemberian asuhan keperawatan merupakan metode/pendekatan yang sangat penting dalam memberikan asuhan keperawatan berkualitas. Tujuan penelitian ini adalah memperoleh model asuhan penyakit kronis pada anak usia sekolah yang mengidap thalassemia f3 mayor untuk meningkatkan kualitas hidupnya. Penelitian ini menggunakan desain riset operasional melalui tiga tahapan penelitian. Tahap I: Identifikasi kebutuhan melalui penelitian kualitatif Tahap II: pengembangaan Modified Chronic Care Model hasil integrasi antara penelitian tahap I, studi literatur, dan konsultasi pakar. Tahap III: uji coba model dengan rancangan quasi experiment with control group. Metode sampling menggunakan simple random sampling dengan jumlah sampel sebanyak 148. Hasil penelitian menunjukkan : 1) Tahap I: diperoleh 15 tema; 2) Tahap II: dihasilkan modifikasi chronic care model dengan 1 buku asuhan keperawatan, 2 buku saku untuk pasien dan keluarga, 1 buku saku untuk perawat; 3) Tahap III: sikap keluarga, kualitas hidup anak, pengelolaan diri dan status fungsional anak yang mendapat intervensi Modified Chronic Care Model lebih tinggi dibandingkan dengan anak yang tidak mendapat intervensi. Kesimpulan, Modified Chronic Care Model efektif meningkatkan kualitas hidup anak, meningkatkan kemampuan pengelolaan diri anak, meningkatkan status fungsional anak dan meningkatkan sikap keluarga dalam merawat anak. Rekomendasi : 1) Replikasi model di rumah sakit pemerintah lain; 2) Pelatihan berkelanjutan bagi perawat di ruang rawat thalassemia; 3) Penelitian lanjut yaitu penghitungan ratio tenaga perawat dengan pasien.

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Nursing care model is the important thing for better quality of nursing care. The aim of this study is to develop nursing care model for thalassemic school age children. This study used operational research design through three stages namely Stage I: Identifying problems and needs. Stage II: Developing the Modified Chronic care Model resulted from integration of the results of stage 1 studies, literature studies, and expert consultation; Stage III: Testing the model with the quasi experiments with control group design. Sampling strategy used simple random sampling method with 148 samples. Results ofresearch were obtained: 1) Stage I: 15 themes were obtained; 2) Stage II: Modification of Chronic Care Model with 1 book o f nursing care, 2 pocket books for patients and their family, 1 pocket book for nurses; 3) Stage III: Children's quality of life, functional status and self-management who received Modified Chronic Care Model intervention were higher than children who didn't receive. Family attitudes who received Modified Chronic Care Model intervention were higher than family who didn't receive. In conclusion, Modified Chronic Care Model effectively increases thalassemic school age children's quality of life, functional status, self- management, and family attitude. Recommendations: 1) Replicate similar models in other government hospitals; 2) Keep continuing training for nurses in thalassemic unit; 3) study about patient-nurse ratio.

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ABSTRAK
Latar Belakang dan tujuan: Pemeriksaan MRI T2 pankreas merupakan pemeriksaan yang lebih akurat dalam menilai hemosiderosis pankreas, namun memiliki keterbatasan karena hanya tersedia di kota-kota besar. Penelitian ini bertujuan untuk mengetahui korelasi nilai T2 pankreas dengan kadar glukosa darah, karakteristik T2 pankreas dan kadar glukosa darah subyek penelitian, serta pengaruh letak ROI terhadap nilai T2 pankreas. Metode: Penelitian menggunakan desain potong lintang pada 52 subyek anak dan 12 subyek dewasa antara September 2015-Juni 2016. Hasil: Nilai T2 pankreas anak antara 2,71-48,77 ms dan dewasa 6,49-41,82 ms. Kadar glukosa darah puasa anak rerata 87,31 11,01 mg/dL dan dewasa 88,00 10,27 mg/dL. Korelasi nilai T2 pankreas terhadap kadar glukosa darah puasa anak r=0,198 p=0,160 dan dewasa r=0,004 p=0,991 . Hubungan nilai T2 pankreas dengan letak ROI didapatkan p=0,105. Kesimpulan: Tidak terdapat korelasi nilai T2 pankreas dengan kadar glukosa darah puasa pada anak dan dewasa. Tidak terdapat perbedaan signifikan nilai T2 pankreas menurut letak ROI.

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ABSTRACT
Background and Objective T2 MRI examination is more accurate in assessing pancreatic hemosiderosis but lack of availability to be used routinely in daily practice. This study aimed to determine the correlation value of T2 pancreas with blood glucose levels in thalassemic children and adults. Methods Cross sectional study that held in September 2015 June 2016, involving 52 children and 12 adults. Results Pancreatic T2 values of children are 2.71 to 48.77 ms and values of adults are from 6.49 to 41.82 ms. Mean of fasting blood glucose levels in children are 87.31 11.01 mg dL and mean of adults are 88.00 10.27 mg dL. Correlation r between value of pancreatic T2 against children rsquo s fasting blood glucose levels was 0.198 p 0.160 and adults was 0.004 p 0.991 . Significancy value p of relationship between pancreatic T2 and ROI of pancreas was 0.105. Conclusion There was no significant correlation between pancreatic T2 values with fasting blood glucose levels in children and adults, nor significant difference in T2 values according to ROI of pancreas.

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[ABSTRAK
Latar Belakang: Infeksi merupakan penyebab kematian yang penting pada thalassemia. Peningkatan risiko infeksi disebabkan oleh banyak faktor antara lain karena kelebihan besi dan splenektomi. Penelitian ini bertujuan mengetahui perbedaan fungsi fagositosis monosit pada pasien thalassemia mayor pasca splenektomi dan non splenektomi serta mengetahui hubungan fungsi fagositosis monosit dengan kadar feritin serum. Metode: Penelitian dilakukan di Departemen Patologi Klinik RSCM, Jakarta pada September 2013 ? Februari 2014. Desain penelitian potong lintang, dengan subjek penelitian pasien thalassemia mayor, terdiri dari 58 subjek pasca splenektomi dan 58 subjek non splenektomi yang telah dilakukan macthing umur dan jenis kelamin. Dilakukan pemeriksaan fagositosis monosit menggunakan E.coli yang telah diopsonisasi dan dilabel FITC sebagai target, (PhagotestTM) dan diperiksa dengan flow cytometry BD FACSCalibur. Kadar feritin serum diperiksa dengan Cobas e 601. Hasil: Median fagositosis monosit pada 58 subjek pasca splenektomi 5,03 (0,17 ? 22,79) %, dan pada 58 subjek non splenektomi 7,09 (0,11 ? 27,24) %, dan nilai p > 0.05. Kadar feritin serum pada subjek pasca splenektomi 6.724 (644,60 ? 21.835) ng/mL dan subjek non splenektomi 4.702,50 (1.381 ? 14.554) ng/mL, dan nilai p < 0.05. Hasil uji korelasi fungsi fagositosis monosit dengan kadar feritin didapatkan r = 0.13 (nilai p = 1.00). Kesimpulan: Tidak terdapat perbedaan bermakna antara fungsi fagositosis monosit pada pasien thalassemia mayor pasca splenektomi dan non splenektomi. Kadar feritin serum pada pasien thalassemia mayor pasca splenektomi lebih tinggi secara bermakna dibandingkan non splenektomi. Tidak didapatkan hubungan antara fagositosis monosit dengan kadar feritin serum.

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ABSTRACT
Background : Infection is an important cause of death in thalassemia. Increase of risk of infection could be due to iron overload and post-splenectomy. The study aimed to determine the difference of phagocytosis function of monocyte between post-splenectomized and non- splenectomized patients with thalassemia major, and the correlation of phagocytosis function of monocyte and serum ferritin level. Methods : The study was conducted in Department of Clinical Pathology Cipto Mangunkusumo hospital, Jakarta, in September 2013 ? Februari 2014. It was a cross sectional study. The study subjects consisted of 58 post-splenectomized patients and 58 non-splenectomized patients with age and sex matching. Phagocytosis function of monocyte was determined using E.coli opsonized and labelled with FITC as target, (Phagotest TM) and was measured by flow cytometry BD FACSCalibur. Serum ferritin level was measured using Cobas e 601. Result : Median phagocytosis of monocyte was 5,03 (0,17 ? 22,79) %, in 58 post- splenectomized subjects and 7,09 (0,11 ? 27,24) % in non-splenectomized subjects; p value > 0.05. Serum ferritin level was 6.274 (644,60 ? 21.835) ng/mL in post-splenectomized subjects and 4.702,50 (1.381 - 14.554) ng/mL in non-splenectomy subjects; p value < 0.05. The correlation between phagocytosis function of monocyte and serum ferritin level was r = 0.13 ( p value = 1.00). Conclusion : There was no statistical difference of phagocytosis function of monocyte between post-splenectomized subjects and non-splenectomized subjects. Serum ferritin level in post- splenectomized was higher than non-splenectomized subjects. There was no correlation between phagocytosis function of monocyte and serum ferritin level.;Background : Infection is an important cause of death in thalassemia. Increase of risk of infection could be due to iron overload and post-splenectomy. The study aimed to determine the difference of phagocytosis function of monocyte between post-splenectomized and non- splenectomized patients with thalassemia major, and the correlation of phagocytosis function of monocyte and serum ferritin level. Methods : The study was conducted in Department of Clinical Pathology Cipto Mangunkusumo hospital, Jakarta, in September 2013 – Februari 2014. It was a cross sectional study. The study subjects consisted of 58 post-splenectomized patients and 58 non-splenectomized patients with age and sex matching. Phagocytosis function of monocyte was determined using E.coli opsonized and labelled with FITC as target, (Phagotest TM) and was measured by flow cytometry BD FACSCalibur. Serum ferritin level was measured using Cobas e 601. Result : Median phagocytosis of monocyte was 5,03 (0,17 – 22,79) %, in 58 post- splenectomized subjects and 7,09 (0,11 – 27,24) % in non-splenectomized subjects; p value > 0.05. Serum ferritin level was 6.274 (644,60 – 21.835) ng/mL in post-splenectomized subjects and 4.702,50 (1.381 - 14.554) ng/mL in non-splenectomy subjects; p value < 0.05. The correlation between phagocytosis function of monocyte and serum ferritin level was r = 0.13 ( p value = 1.00). Conclusion : There was no statistical difference of phagocytosis function of monocyte between post-splenectomized subjects and non-splenectomized subjects. Serum ferritin level in post- splenectomized was higher than non-splenectomized subjects. There was no correlation between phagocytosis function of monocyte and serum ferritin level., Background : Infection is an important cause of death in thalassemia. Increase of risk of infection could be due to iron overload and post-splenectomy. The study aimed to determine the difference of phagocytosis function of monocyte between post-splenectomized and non- splenectomized patients with thalassemia major, and the correlation of phagocytosis function of monocyte and serum ferritin level. Methods : The study was conducted in Department of Clinical Pathology Cipto Mangunkusumo hospital, Jakarta, in September 2013 – Februari 2014. It was a cross sectional study. The study subjects consisted of 58 post-splenectomized patients and 58 non-splenectomized patients with age and sex matching. Phagocytosis function of monocyte was determined using E.coli opsonized and labelled with FITC as target, (Phagotest TM) and was measured by flow cytometry BD FACSCalibur. Serum ferritin level was measured using Cobas e 601. Result : Median phagocytosis of monocyte was 5,03 (0,17 – 22,79) %, in 58 post- splenectomized subjects and 7,09 (0,11 – 27,24) % in non-splenectomized subjects; p value > 0.05. Serum ferritin level was 6.274 (644,60 – 21.835) ng/mL in post-splenectomized subjects and 4.702,50 (1.381 - 14.554) ng/mL in non-splenectomy subjects; p value < 0.05. The correlation between phagocytosis function of monocyte and serum ferritin level was r = 0.13 ( p value = 1.00). Conclusion : There was no statistical difference of phagocytosis function of monocyte between post-splenectomized subjects and non-splenectomized subjects. Serum ferritin level in post- splenectomized was higher than non-splenectomized subjects. There was no correlation between phagocytosis function of monocyte and serum ferritin level.]

Abstrak :
ABSTRACT
Angka penderita Thalasemia di Indonesia diprediksi akan meningkat setiap tahun dan penyakit genetik tersebut dapat menimbulkan dampak psikologis dan juga dampak finansial bagi penderita, keluarga, dan negara. Penelitian ini bertujuan untuk melihat hubungan persepsi akan hambatan (PB) dan pertimbangan konsekuensi masa depan (CFC) terhadap intensi (BI) melakukan skrining/deteksi dini Thalasemia pada dewasa muda. Penelitian ini diikuti oleh 411 partisipan dewasa muda berusia 18 sampai 25 tahun (M = 20,47) yang berdomisili di wilayah Jabodetabek. Partisipan mengikuti penelitian dengan mengisi kuesioner daring dan menonton video animasi mengenai Thalasemia. Instrumen penelitian yang digunakan adalah alat ukur skala Preventive Health Behavior (Cronbachs α = ,71), alat ukur CFC-14 Scale (Cronbachs α = ,82), dan alat ukur BI dengan single-item. Hasil analisis regresi menggunakan PROCESS adalah: (1) Pb memiliki hubungan yang negatif signifikan dengan BI (b1 = -2,019; p < 0,05), (2) CFC tidak memiliki hubungan signifikan dengan BI (b2 = -1,639; p > 0,05), (3) Interaksi antara PB dan CFC tidak memiliki hubungan signifikan dengan BI (b3 = -,808; p > 0,05),. Hasil temuan lain adalah self-efficacy yang dikontrol secara statistik terbukti memiliki hubungan dengan BI dan menjadi prediktor kuat terhadap intensi perilaku sehat, yaitu perilaku pencegahan Thalasemia melalui skrining.

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ABSTRACT
Number of Thalassemia patients is predicted to increase every year in Indonesia and this genetic disease can cause psychological impacts as well as huge costs for patients, families and nurses, and also the government. The purpose of this study is to analyze the correlation of Perceived Barriers (PB) and Considerations Future Consequences (CFCs) and their interactions with Behavioral Intention (BI) to take Thalassemia screening as part of preventive health behaviors. Participants were 411 young adults, ranging from 18 to 25 years (M = 20.47) who lived in the Greater Jakarta area. Participants take steps through self-report online questionnaire and must watch a short animated video about Thalassemia. Measurements used in this study are adapted versions of the Preventive Health Behavior Scale (Cronbach's, = .71), CFC-14 Scale (Cronbach's  =, 82), and the BI Scale, with a single item size. The results using the PROCESS regression analysis showed that, (1) there were negative and significant results from PBto BI (b1 = -2,019; p <0.05), and (2) CFC had insignificant results on BI (b2 = -1,639; p> 0.05), (3) PB and CFC interactions have insignificant results on BI (b3 = -808; p> 0.05). Other findings show that self-efficacy as a control variable is proven. has a significant correlation with BI and is a strong predictor of intentions to enforce preventive health behaviors in the form of thalassemia screening.