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"Latar Belakang: Kondisi besi berlebih, dengan feritin dan saturasi transferin sebagai surrogate marker, akan menimbulkan oksigen radikal bebas (ROS) yang menyebabkan stress oksidatif. Malondialdehid (MDA) merupakan ROS yang terbentuk dari peroksidase lemak sedangkan (manganese)superoksid dismutase (MnSOD) sebagai enzim yang mengubah radikal bebas oksigen menjadi oksigen biasa. Transfusi darah sering digunakan untuk mengatasi anemia pada kanker, namun juga berpotensi meningkatkan beban besi pada tubuh. Penelitian ini melihat peran transfusi darah terhadap feritin serum dan saturasi transferin serta kaitannya dengan stres oksidatif pada pasien kanker nasofaring.Tujuan: Mengetahui peranan transfusi sel darah merah (SDM) dalam kaitannya dengan perubahan kadar feritin serum dan saturasi transferin serta korelasinya dengan stress oksidatif pada pasien kanker nasofaring (KNF) yang menjalani kemoradiasi.Metode: Studi kohort prospektif. Pengambilan data dilakukan di klinik Hematologi-Onkologi Medik IPD RSCM Jakarta secara consecutive sampling pada bulan November 2015-Februari 2016. Pasien KNF yang menjalani kemoradiasi diperiksakan kadar feritin serum, saturasi transferin, MDA, MnSOD pra dan pasca terapi. Dilakukan pencatatan jumlah transfusi sel darah merah yang diterima. Analisa data menggunakan T-test/Mann Whitney dan uji korelasi Spearman.Hasil: Total 38 pasien yang menjalani kemoradiasi, usia rata-rata 47 tahun, laki-laki dan perempuan 4:1. Sebanyak 18 pasien (47,4%) menerima transfusi sel darah merah selama pengobatan. Didapatkan peningkatan rerata saturasi transferin sebesar 15,1% (p=0,016) dan MDA sebesar 1,368 (p=0,001) pada pasien yang mendapatkan transfusi SDM dibandingkan yang tidak mendapatkan transfusi. Tidak didapatkan perbedaan yang signifikan pada feritin serum (p= 0,35) dan MnSOD (p= 0,496) antara yang mendapatkan transfusi SDM dengan yang tidak. Didapatkan korelasi lemah antara feritin serum dengan MDA dan MnSOD (r=0,239 dan r= -0,374) dan tidak didapatkan korelasi antara saturasi transferin dengan MDA dan MnSOD (r=0,191 dan r=0,027).Simpulan: Terdapat peningkatan rerata saturasi transferin dan MDA pada pasien yang mendapatkan transfusi SDM. Tidak terdapat peningkatan feritin serum ataupun penurunan MnSOD. Terdapat korelasi yang lemah antara peningkatan kadar feritin serum dengan MDA dan MnSOD pada pasien KNF pasca kemoradiasi dan transfusi sel darah merah. Tidak terdapat korelasi antara kadar saturasi transferin dengan MDA dan MnSOD pada pasien KNF yang mendapat transfusi sel darah merah.

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Background: The presence of free iron in the circulation will induce the formation of reactive oxygen species (ROS) which result in cell injury. The free radical formed and cause lipid peroxidation which in result cause formation of malondialdehyde (MDA). (manganese)Superoxide Dismutase (MnSOD) as antioxidant enzyme have anti tumor activity and the level often found low in cancer patient. Ferritin and transferrin saturation are predictor of iron overload. Blood transfusion is the therapy often used to resolve anemia in cancer, but also increase iron burden in body. This study focus on the role of blood transfusion to serum ferritin and transferrin saturation and its correlation with oxidative stress in patients with nasopharyngeal cancer (NPC).

Objective: To know the role of red blood cell (RBC) transfusion and its relations to serum ferritin and transferin saturation level and their correlation with oxidative stress in nasopharyngeal cancer patients undergoing chemoradiation.

Methods: Prospective study. Sample obtained with consecutive sampling method collected in the Hematology-Medical Oncology Clinic in Cipto Mangunkusumo Hospital Jakarta November 2015 to February 2015. NPC patients undergoing chemoradiation, blood examination performed to measure ferritin, saturation transferrin, malondialdehyde, MnSOD before and after treatment. During treatment the amount of transfusion received is recorded. Data analysis done using T-test/Mann Whitney and Spearman correlation test.

Results: Total of 38 patients received chemoradiation, mean age 47,97 years old, proportion man compare woman is 4:1. During treatment 18 patients (47,4%) received red blood cell transfusion. Difference in mean found between transferrin saturation 15,1% (p=0,016) and MDA serum 1,358 nM (p=0,001) in patient receiving RBC transfusion compare to subject not receving transfusion. There are no significantly differences in serum ferritin and MnSOD level between both group. We found a weak correlation between raise of serum ferritin to the raise of MDA and the declining of MnSOD (r = 0,239 and r= -0,374). There are no correlation between transferin saturation with MDA nor MnSOD

Conclusions : Increase in transferin saturation and MDA level found in NPC receiving RBC transfusion after chemoradiation. There is a weak correlation found between serum ferritin with MDA and MnSOD in nasopharyngeal cancer undergoing chemotherapy radiation therapy receiving RBC transfusion and no correlation between transferin saturation with MDA and MnSOD changes."

"Latar Belakang: Talasemia merupakan kelainan sintesis hemoglobin yang membutuhkan transfusi darah berulang. Kombinasi terapi kelasi dan transfusi darah telah meningkatkan harapan hidup, namun menyebabkan penumpukan besi di organ tubuh seperti kelenjar endokrin. Hipogonadisme yang merupakan salah satu gangguan endokrin yang sering terjadi pada penderita talasemia, umumnya terjadi akibat penumpukan besi di jaringan hipofisis. Penumpukan besi di hipofisis dapat dilihat dengan melihat waktu relaksasi MRI T2 hipofisis.Tujuan: Penelitian ini bertujuan untuk melihat hubungan status besi dengan keadaan hipogonadisme yang dinilai dengan melihat korelasi serum feritin, saturasi transferin dan waktu relaksasi MRI T2 hipofisis dengan kadar FSH, LH dan testosteron.Metode: Penelitian ini merupakan studi potong lintang dengan subjek 32 penderita pria talasemia bergantung transfusi. Pengambilan sampel dilakukan secara konsekutif di poliklinikin talasemia Rumah Sakit Cipto Mangunkusumo. Pemeriksaan serum feritin, saturasi transferin, FSH, LH dan testosteron menggunakan teknik ELISA. Sedangkan pemeriksaan waktu relaksasi MRI T2 hipofisis menggunakan MRI Avanto 1,5 Tesla.Hasil: Pada penelitian ini didapatkan 62,5% pasien tidak mencapai pubertas sempurna. Didapatkan rerata kadar testosteron 23,31 (SB 15,57). Didapatkan 25% pasien memiliki testosteron rendah, dan dari kelompok tersebut seluruhnya memiliki kadar FSH dan LH yang rendah atau normal. Dijumpai adanya korelasi negatif lemah antara waktu relaksasi MRI T2 hipofisis dengan saturasi transferin pada kelompok dengan nilai testosteron normal. Korelasi pada variabel lainnya tidak terdapat yang signifikan.Simpulan: Angka kejadian pasien dengan pubertas tidak sempurna cukup tinggi, tidak sejalan dengan hasil laboratorium. Pada penelitian ini dijumpai korelasi negatif lemah antara saturasi transferin dengan waktu relaksasi MRI T2 hipofisis.

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Background: Thalassemia is a disorder of haemoglobin synthesis that require regular blood transfusion. The combination of chelation therapy and blood transfusion has extended life expectancy. However, repetition of blood transfusions leads to accumulation of iron in organs such as endocrine glands. Hypogonadism is one of the most prevalent endocrine disorder in thalassemia, caused by iron deposition in pituitary gland. Iron overload in pituitary can be measured by pituitary MRI T2 relaxation time.

Objective: The purpose of this study was to see the correlation between iron overload with hypogonadal state by analyzing correlation between ferritin serum, transferrin saturation, pituitary MRI T2 relaxation time with FSH, LH and testosterone levels.

Methods: This is a cross-sectional study with 32 subjects of male transfusion-dependent thalassemia. The subjects were collected with consecutive sampling technique in thalassemia outpatient clinic in Rumah Sakit Cipto Mangunkusumo, Jakarta. Measurement of serum ferritin, transferrin saturation, FSH, LH and testosterone were done using ELISA technique. Pituitary MRI T2 relaxation time was done using MRI Avanto 1.5 Tesla.

Results: In this study, secondary sexual characteristics was not fully achieved in 62,5%. The mean of testosterone levels is 23,31 (SD 15,57). Low testosterone levels were found in 25% patients, and all had low or normal FSH and LH levels. There was a weak negative correlation between transferrin saturation and pituitary MRI T2 relaxation time in normal testosterone level group.

Conclusions: This study demonstrated high rate of patients who did not achieved puberty, but low rate of patient with low testosterone. There is a weak negative correlation between transferrin saturation and pituitary MRI T2 relaxation times."

"ABSTRAKLatar 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"

"Penelitian ini bertujuan untuk mengetahui keluhan subjektif mata kering dan gangguan komponen air mata (lipid, akuos, mucin) pada pasien thalassemia mayor dengan riwayat transfusi darah jangka panjang, serta menganalisis hubungan antara kadar feritin serum, durasi, dan frekuensi transfusi darah dengan masing-masing parameter penilaian komponen lapisan air mata. Penelitian ini merupakan studi potong lintang (cross sectional) pada pasien thalassemia mayor yang sudah berusia dan mengalami transfusi darah selama minimal 10 tahun. Penilaian mata kering terdiri dari pengisian kuesioner OSDI untuk menilai keluhan subjektif, pemeriksaan biomikroskopi lampu celah dan nilai tear break up time (TBUT) untuk menilai tingkat keparahan mata kering, pemeriksaan Schirmer basal, Ferning, dan sitologi impressi konjungtiva untuk menghitung jumlah sel goblet. Data perhitungan tingkat keparahan mata kering, nilai uji Schirmer basal, TBUT, dan jumlah sel goblet dianalisis dan dicari hubungannya dengan kadar feritin serum, durasi dan frekuensi transfusi. Pada 77 subyek, mata kering terjadi sebanyak 14.3%, penurunan nilai TBUT (39%), nilai Schirmer basal (37.7%), nilai Ferning (24.7%), dan jumlah sel goblet (45.5%). Tidak terdapat perbedaan bermakna antara tingkat keparahan mata kering, nilai TBUT, nilai Schirmer basal, nilai Ferning, dan jumlah sel goblet dengan kadar feritin serum, durasi, dan frekuensi transfusi. Namun, terdapat hubungan yang bermakna antara tingkat keparahan mata kering dan usia (p = 0.014), serta nilai TBUT (p = 0.012) dan Schirmer (p = 0.014) dengan jenis kelamin. Penelitian ini memperlihatkan 14.3% subyek thalassemia mayor mengalami mata kering berdasarkan kriteria DEWS 2007. Kejadian mata kering pada thalassemia mayor tidak dipengaruhi oleh faktor transfusi dan kadar feritin serum, melainkan dipengaruhi oleh usia dan jenis kelamin.

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This study is aimed to understand subjective complaints for dry eyes and disruption of component of tear fluid (lipid, aqueous, mucin) in patients with major thalassemia with a history of long-term blood transfusions and to analyse the correlation between serum ferritin level, duration and frequency of blood transfusion. This study is a cross-sectional study. The subject of this study is patients with major thalassemia age minimum of 10 years old and have had blood transfusion for at least 10 years. OSDI questionnaire, slit-lamp biomicroscopy examination, tear break up time (TBUT), and basal Schirmer test was used to assess dry eyes severity. Ferning and conjunctiva impression cytology examination was used to assess mucin quality and count the amount of goblet cells. The correlation analysis between the result of these assessments and serum ferritin level and duration and frequency of blood transfusion was done. In 77 subjects, the prevalence of dry eyes is 14.3%. There is a decrease in TBUT (39%), basal Schirmer (37.7%), Ferning (24.7%), and goblet cells (45.5%). There is no significant correlation between dry eyes severity and TBUT, basal Schirmer, Ferning, and the amount of goblet cells with serum ferritin level, duration, and frequency of blood transfusion. There is a significant correlation between dry eyes severity and patient s age (p = 0.014); TBUT (p = 0.012), as well as, Schirmer (p = 0.014) with sex. This study showed that 14.3% of patients with major thalassemia suffer from dry eyes with severity level grade 2 according to DEWS 2007. The incidence of dry eyes is not influenced by transfusion and serum ferritin level but is influenced by age and sex."

"Latar belakang. Kadar hemoglobin pre-transfusi dan feritin serum mempengaruhi pertumbuhan anak dengan thalassemia B-mayor. Penelitian tentang thalassemia sudah dilakukan di Indonesia, namun penelitian tentang hubungan thalassemia dengan pertumbuhan fisik masih terbatas. Tujuan. Mengetahui pengaruh kadar Hb pre-transfusi dan feritin serum berpengaruh terhadap pertumbuhan fisik pasien thalassemia ?-mayor. Metode. Dilakukan bulan Agustus-Oktober 2017 pada pasien anak dengan thalassemia B-mayor yang berobat ke Thalassemia-Centre RSUD Pekanbaru. Penelitian berupa analitik observasional potong lintang, menganalisis pengaruh kadar Hb pre-transfusi dan feritin serum terhadap parameter perawakan pendek dan sangat pendek, gizi kurang dan buruk, usia tulang yang terlambat. Hasil. Subjek 41 orang, rentang usia 18-204 bulan. Jenis kelamin laki-laki lebih banyak daripada perempuan 53,7 vs 46,3. 40 subjek mengalami retardasi pertumbuhan. Terdapat korelasi bermakna antara kadar Hb pre-transfusi dengan Z-score TB/U r=0,507, p=0,001 dan LILA/U r=0,467, p=0,02. Hb pre-transfusi berpengaruh terhadap interpretasi duduk/umur p=0,007, IK95 -1,5 - -0,3, subischial leg length/umur p=0,002, namun tidak pada interpretasi rasio segmen atas/bawah dan usia tulang. Hasil berbeda pada kadar feritin yang tidak memiliki korelasi terhadap semua variabel. Simpulan. Terdapat pengaruh yang bermakna secara statistik antara kadar Hb pre-transfusi dengan parameter penelitian serta tidak terdapat pengaruh yang bermakna secara statistik antara kadar feritin serum dengan parameter tersebut.

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Background. The level of pre transfusion hemoglobin and ferritin serum affect physical growth on patient with thalassemic mayor. Study about thalassemia is mainly reported but its relationship with physical growth is limited.

Objective. The main objective of the present study was to evaluate the relationship of pre transfusion Hb and serum ferritin level in patient with thalassemic mayor.

Material and method. In this analytical cross sectional study, the growth parameters weight, standing height, sitting height, subischial leg length, nutritional status, bone age were measured in 41 patients attending Thalassemia Centre at RSUD in Pekanbaru from August October 2017.

Results. 41 patients with mean age 18 204 months. The results are boys dominated girls in sex criteria 53,7 vs 46,3. As much as 40 subjects have growth retardation. There rsquo s correlation in pre transfusion hemoglobin with Z score height for age r 0,507, p 0,001 and subischial length r 0,467, p 0,02. This study shows relationship in pre transfusion hemoglobin with sitting height p 0,007, IK95 1,5 0,3, subischial leg length p 0,002, but not in segment length and bone age. Serum ferritin level has no correlation to one of those parameters.

Conclusion. There is a significant relationship in physical growth based on parameters mentioned above with pre transfusion Hemoglobin, but not with serum ferritin level. "

"Latar Belakang: Talasemia merupakan penyakit kelainan hemoglobin (Hb) dengan prevalensi tinggi di Indonesia maupun dunia. Komplikasi pada talasemia dapat terjadi akibat kadar Hb pre-transfusi yang rendah dan penumpukan feritin serum. Pengetahuan, sikap, dan perilaku yang baik terhadap suatu penyakit dibutuhkan untuk mencapai kesehatan yang diinginkan dan mencegah komplikasi. Studi ini bertujuan untuk mencari hubungan antara pengetahuan, sikap, dan perilaku terhadap talasemia, Hb pre-transfusi dan kadar feritin serum pada pasien remaja talasemia karena mereka memiliki prevalensi tertinggi. Metode: Kuesioner pengetahuan, sikap, dan perilaku (PSP) melalui google form disebarkan untuk mendapatkan data dari pasien talasemia remaja yang memenuhi kriteria studi. Pengetahuan akan dibagi menjadi adekuat atau tidak adekuat, sikap dibagi menjadi positif atau negatif, perilaku dibagi menjadi baik atau buruk berdasarkan hasil skor kuesioner. Kadar Hb pre-transfusi dan feritin serum diambil dari rekam medik pasien, dan dikelompokkan menjadi Hb dan serum ferritin yang tinggi atau rendah. Hasil Penelitian: Dari 85 subjek, terdapat 49.4% pasien dengan pengetahuan adekuat, 91.8% pasien dengan sikap positif, dan 72.9% pasien dengan perilaku baik. Pasien masih kurang memahami fasilitas skrining dan pentingnya suplementasi vitamin. Pasien perlu meningkatkan sikap positif terhadap skrining thalassemia dan perilaku baik terhadap kepatuhan obat. Terdapat hubungan yang tidak bermakna secara statisik antara pengetahuan, sikap dan perilaku terhadap kadar Hb pre-transfusi dan kadar ferritin (p >0.05) pada remaja dengan talasemia. Kesimpulan: Remaja talasemia di Rumah Sakit Cipto Mangunkusumo memiliki pengetahuan yang tidak adekuat, namun dengan sikap dan perilaku yang baik. Perlu adanya edukasi berkala untuk meningkatkan pengetahuan.

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Introduction: Thalassemia is a hemoglobin (Hb) disorder that has a high prevalence in Indonesia and the world. Complications in thalassemia can occur due to low pre-transfusion Hb and accumulation of serum ferritin. A good knowledge, attitude, and practice towards a disease are needed to achieve desired health outcomes and prevent complications. This study aims to find the relationship between knowledge, attitude, and practice towards thalassemia, pre-transfusion Hb, and serum ferritin levels in thalassemic adolescents as they have the highest prevalence. Methods: Knowledge, attitude, and practice (KAP) questionnaire through google form were distributed to adolescent thalassemic patients who met the criteria. Knowledge will be divided into adequate or inadequate; attitudes are divided into positive or negative; practice is divided into good or bad based on the questionnaire results. Level of Pre-transfusion Hb and serum ferritin were taken from the patient's medical record and grouped into high or low Hb and ferritin. Result: Out of 85 subjects, there were 49.4% patients with adequate knowledge, 91.8% patients with positive attitude, and 72.9% patients with good practice. Patients still lack understanding of screening facilities and the importance of vitamin supplementation. Patients need to increase positive attitude towards thalassemia screening and good behavior towards treatment adherence. There was a statistically insignificant relationship between knowledge, attitude, and practice on thalassemia with pre-transfusion Hb and serum ferritin (p > 0.05) in thalassemic adolescents. Conclusion: Thalassemic adolescents at Cipto Mangunkusumo Hospital have inadequate knowledge, but with good attitudes and behavior. Periodic education is needed to increase knowledge."

"ABSTRAKLatar Belakang: Muatan besi berlebih yang diduga dapat menyebabkan peningkatan stres oksidatif malondialdehyde merupakan masalah utama pada pasien thalasemia beta mayor dan intermedia, baik TDT maupun NTDT. Transfusi darah dan kelasi besi merupakan terapi utama thalasemia. Kadar malondialdehyde MDA plasma belum diteliti mendalam di Indonesia, terutamapada pemberian transfusi serta korelasinya dengan muatan besi berlebih.Tujuan: mendapatkan profil kadar MDA pada pasien thalasemia dewasa; membandingkan kadarnya antara sebelum dan setelah transfusi antara pasien TDT dan NTDT serta mendapatkan korelasinya dengan feritin serum FS dan saturasi transferin ST .Metode: Penelitian potong lintang serta pre dan post study pada penderita dewasa thalasemia beta yang mendapatkan transfusi darah serta dengan/atau tanpa kelasi besi. Sampel darah diambil sesaat sebelum transfusi dan satu hari setelah transfusi. Kadar MDA plasma diperiksa berdasarkan metode Wills.Hasil: Sebanyak 63 orang pasien dilibatkan dalam studi, terdiri dari 51 TDT dan 12 NTDT. Median kadar MDA adalah 0,49 0,21-1,33 ?mol/L. Kadar tersebut tidak berbeda bermakna antara sebelum dan setelah transfusi, antara pasien TDT dan NTDT. Didapatkan korelasi lemah antara FS dengankadar MDA sebelum transfusi sedangkan tidak ada korelasi antara FS dengan kadar MDA setelah transfusi dan antara ST dengan kadar MDA.Simpulan: Median kadar MDA plasma pada pasien dewasa dengan thalasemia beta mayor dan intermedia sebelum transfusi 0,49?mol/L.Tidak ada perbedaan bermakna antara kadar MDA sebelum dan setelah transfusi dan antara pasien TDT dan NTDT. Terdapat korelasi lemah antara FS dengan MDA sebelum transfusi dan tidak terdapat korelasi FS dengan MDA setelah transfusidan ST dengan kadar MDA.

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ABSTRACTAbstract Background Iron overload is a major problem in thalassemic patients, either in TDT or NTDT. Iron overload may increase oxidative stress malondialdehyde MDA . Blood transfusion and chelating iron are the main therapy for beta thalassemia major TDT. However, plasma MDA levels have not been well studied in Indonesia, especially its correlation with iron overload.Objective This study aimed to profile MDA levels in adult thalassemic patients to compare its level between before and after transfusion and between TDT and NTDT patients and to obtains its correlation with serum ferritin SF and transferrin saturation TS .Method A cross sectional as well as pre and post study in adult patients with thalassemia major and intermedia who received blood transfusion with or without chelating iron.Blood samples were withdrawn immediately before transfusion and one day after transfusion. Plasma MDA levels were assayed according to Wills method. Results Total of 63 patients were enrolled, consisting 51 TDT and 12 NTDT patients. Median MDA level was 0.49 0,21 1,33 mol L. The level was not significantly different between before and after transfusion, between TDT and NTDT patients. Weak correlation was observed between SF and MDA levels before transfusion and there is no correlation was observed between SF and MDA levels before transfusion and also between TS and MDA levels.Conclusion Median plasma MDA levels in adult patients with beta thalassemia major and intermedia before transfusion 0,49 mol L. No significant different is found between MDA before and after transfusion and between TDT and NTDT patients as well. Plasma MDA levels have weak correlation with serum feritin levels before blood transfusion."

"ABSTRAKLatar 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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ABSTRACTBackground 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. "

"Penelitian ini bertujuan untuk mengetahui aktivitas laktat dehidrogenase LDH serum dan korelasinya dengan asupan karbohidrat pada pasien kanker paru stadium lanjut di Rumah Sakit Kanker Nasional Dharmais Jakarta. Pada sel kanker terjadi efek Warburg yaitu kecenderungan sel kanker untuk melakukan glikolisis anaerob. Enzim LDH berfungsi sebagai katalisator untuk mengubah piruvat menjadi laktat pada keadaan anaerob. Peran laktat pada pada sel kanker meliputi inisiasi pertumbuhan tumor, menjaga kelangsungan sel kanker, proliferasi, angiogenesis, dan metastasis. LDH dapat digunakan sebagai marker diagnostik, penentu prognosis, sensitivitas dan resistensi tumor terhadap terapi, dan target potensial untuk kemoterapi. Subjek didapatkan melalui consecutive sampling yang melibatkan 56 subjek kanker paru stadium lanjut. Rerata usia hasil adalah 56,98 10,36 tahun, sebanyak 55,4 berjenis kelamin laki-laki. Asupan karbohidrat berdasarkan food recall 1 x 24 jam adalah 57,64 10,85 , sedangkan berdasarkan food frequency questionnaire FFQ semikuantitatif adalah 57,98 10,50 . Nilai median aktivitas LDH adalah 541,5 164 ndash;6539 IU/L yang sebanyak 60,7 aktivitasnya meningkat. Pada penelitian ini didapatkan korelasi negatif yang bermakna dengan kekuatan sedang p = 0,017, r = - 0,317 antara asupan total karbohidrat per hari dalam gram berdasarkan metode food recall 1 x 24 jam dengan aktivitas LDH serum. Tidak didapatkan korelasi yang bermakna antara total karbohidrat per hari dalam gram berdasarkan metode FFQ semikuantitatif dan asupan karbohidrat terhadap total energi dengan aktivitas LDH baik berdasarkan metode FFQ semikuantitatif dan food recall 1 x 24 jam.Kesimpulan: Asupan karbohidrat dalam 24 jam berkorelasi negatif bermakna dengan aktivitas LDH serum pada pasien kanker paru stadium lanjut.

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The aim of this study is to determine serum lactate dehydrogenase LDH activity and its correlation with carbohydrate intake in advanced lung cancer patients at Dharmais National Cancer Hospital Jakarta. Cancer cells are characterized by increase anaerobic glycolysis termed the Warburg effect. LDH enzyme catalyzes the convertion of lactate to pyruvate in anaerobic condition. Activity of lactate in cancer influences on tumor growth initiation, tumor survival, proliferation, angiogenesis and metastasis. Serum LDH activity can be used as a diagnostic marker, prognostic marker, predictive marker for tumor sensitivity and resistancy to therapy, and potensial target for chemotherapy. 56 subjects of advanced lung cancer are recruited by consecutive sampling. The mean of age subjects is 56,98 10,36 years old and 55,4 were male. Carbohydrate intake based on food recall 1 x 24 hours is 57,64 10,85 , while based on food frequency questionnaire FFQ semiquantitative is 57,98 10,50 . The median of LDH activity is 541,5 164 ndash 6539 IU L and 60,7 is increse. This study show medium negative significant correlation p 0,017, r 0,317 between total carbohydrate intake per day in grams based on food recall 1 x 24 hours with LDH serum activity. There is no significant correlation between total carbohydrate intake per day in grams based FFQ semiquantitative and carbohydrate intake of total energy with LDH serum activity based on food recall 1 x 24 hours and FFQ semiquantitative. In conclusion, there is medium negative significant correlation between carbohydrate intake in 24 hours with LDH serum activity in advanced lung cancer patients."

"Pendahuluan: Thalassemia adalah suatu kelainan genetik akibat kegagalan sintesis rantai globin, mengakibatkan terjadinya anemia berat akibat peningkatan aktivitas eritropoiesis yang inefektif dan hemolisis. Peningkatan aktivitas eritropoiesis akan memacu peningkatan absorpsi besi di usus sehingga terjadi kelebihan besi dalam tubuh. Transfusi darah dilakukan secara berkala untuk mengatasi anemia yang timbul pada pasien thalassemia mayor. Pemberian transfusi berulang akan mempercepat terjadi secondary iron overload, untuk mengatasinya diberikan terapi kelasi rutin. Tujuan : Mendapatkan perubahan nilai indeks transferin, saturasi transferin, dan feritin sebelum dan sesudah transfusi dan juga sebelum dan sesudah terapi kelasi pada pasien thalassemia mayor. Mendapatkan perbedaan indeks transferin dan saturasi transferin, dan feritin sebagai parameter untuk menilai perubahan status besi pada pasien thalassemia mayor pasca transfusi dan terapi kelasi. Metode: Desain penelitian kohort prospektif. Subjek penelitian terdiri dari 35 pasien thalassemia mayor usia 7-18 tahun yang mendapat transfusi berulang dan kelator besi rutin. Dilakukan pemeriksaan kadar besi serum, UIBC, TIBC, feritin, transferin, saturasi transferin dan indeks transferin pre transfusi, pasca transfusi dan pasca terapi kelasi. Hasil: Rerata indeks transferin pasca transfusi 124±22 % lebih rendah secara bermakna dari pre transfusi dengan nilai p=0,016, sedangkan pasca kelasi 123 ± 34.5 % (p=0,045). Saturasi transferin pasca transfusi 96 (51 – 100)% meningkat secara bermakna dibangdingkan pre transfusi 87(69-100)% dengan nilai p=0,026, namum tidak berbeda bermakna pada pasca kelasi 87 (39-100). Kadar feritin serum pasca transfusi 3737 (649 -17.094) mg/dL, meningkat secara bermakna dibandingkan pre transfusi 3315 (544,7-14.964) mg/dL (p=0,018). Perbedaan indeks transferin dan saturasi transferin pre transfusi 45(22-153)% lebih tinggi secara bermakna dibandingkan pasca transfusi 35(6-89)% dengan nilai p=0,000, sedangkan pasca kelasi adalah 41±25 dengan nilai p=0,036. Kesimpulan: pemeriksaan indeks transferin untuk pemantauan efektifitas terapi kelasi pada pasien thalasemia mayor dapat dipertimbangkan.

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Introduction: Thalassemia syndromes are a group of hereditary disorder characterized by genetic deficiency in the synthesis of β-globin chains. It is associated in severe anemia caused by an increase in ineffective erythropoiesis activity and hemolysis. Erythropoiesis activity will spur increased iron absorption in the intestine so there will be an excess of iron in the body. Blood transfusion is used routinely to treat anemia arising in patients with thalassemia major. Repeated transfusion will accelerate occur secondary iron overload, to solve given chelation therapy routine.

Objective: To know the index value changes transferrin, transferrin saturation, and ferritin before and after transfusion and also before and after chelation therapy in patients with thalassemia major. To know difference transferrin index and transferrin saturation, and ferritin as a parameter to assess changes iron status in patients thalassemia major post-transfusion and chelation therapy.

Methods: This was prosphective cohort, There were 35 patients with thalassemia major who receive repeated transfusions and iron kelator routine, with age 7-18 years. Examination of serum iron levels, UIBC, TIBC, ferritin, transferrin, transferrin saturation and transferrin index before transfusion, after transfusion, and after chelation therapy.

Results: Mean transferrin index post-transfusion 124±22% was significantly lower than pre transfusion (p=0.016), as well as post-chelation 123±34.5% with a value of p=0.045. Transferrin saturation post-transfusion 96 (51-100)% increased significantly with pre transfusion 87 (69-100)% with a value of p=0.026, However no significant difference were observed in post chelation therapy 87 (39-100). Post-transfusion serum ferritin level 3737 (649-17094) mg/dL, increased significantly compared to pre transfusion 3315 (544.7-14,964) mg/dL (p=0.018). Differences transferrin index and transferrin saturation pre transfusion was 45 (22-153)% significantly higher than the post-transfusion 35 (6-89)% with a value of p=0.000, while the post chelation thyrapy was 41±25% (p=0.036).

Conclusion: Transferrin index can be considered for monitoring the effectiveness of chelation therapy in patients with thalassemia major."