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"[ABSTRAKAnemia defisiensi besi dan thalassemia β trait merupakan penyebabtersering anemia mikrositik hipokrom di Indonesia. Kedua penyakit tersebut sulitdibedakan hanya dengan pemeriksaan hematologi, oleh karena itu diperlukanpemeriksaan tambahan seperti feritin dan analisis hemoglobin. Namun tidak semualaboratorium dapat melakukan pemeriksaan ini. Banyak penelitian yangmembedakan kedua penyakit tersebut dengan indeks eritrosit. Namun indekseritrosit memiliki nilai diagnostik yang berbeda di setiap negara dan belum ada datadi Indonesia. Penelitian ini melakukan uji diagnostik Indeks Mentzer, RDW, Green-King, Sirdah, dan mencari nilai cut-off baru yang memberikan nilai diagnostik lebihbaik.Penelitian terdiri dari 98 subyek definitif anemia defisiensi besi dan 80subyek thalassemia β trait. Nilai diagnostik Indeks Mentzer untuk anemia defisiensibesi adalah sensitivitas 83.6%, spesifisitas 66.2%, NPP 75.2%, NPN 76.8%, RKP2.4, RKN 0.2. Nilai diagnostik Indeks Mentzer untuk thalassemia β trait adalahsensitivitas 66.2%, spesifisitas 83.6%, NPP 76.8%, NPN 75.2%, RKP 4.0, RKN0.4. Nilai diagnostik Indeks RDW untuk anemia defisiensi besi adalah sensitivitas91.8%, spesifisitas 75%, NPP 81.8%, NPN 88.2%, RKP 3.6, RKN 0.1. Nilaidiagnostik 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 diagnostikIndeks 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 diagnostikIndeks Green-King untuk thalassemia β trait adalah sensitivitas 67.5%, spesifisitas96.9%, NPP 94.7%, NPN 78.5%, RKP 22.0, RKN 0.3. Nilai diagnostik IndeksSirdah 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 untukthalassemia β trait adalah sensitivitas 58.7%, spesifisitas 92.8%, NPP 87.0%, NPN73.3%, RKP 8.2, RKN 0.4. Nilai cut-off baru Indeks Mentzer adalah 13.44, RDWI233.4, Green-King 75.06, dan Sirdah 32.52.Keempat indeks eritrosit dapat diaplikasikan untuk orang Indonesia denganIndeks Green-King sebagai indeks yang terbaik.

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ABSTRACTIron deficiency anemia and β trait thalassemia are the most common causesof microcytic hypochromic anemia in Indonesia. Differentiation between them isdifficult when solely based on a hematology examination, so additional laboratorytests are required such as ferritin and hemoglobin analysis. However, not alllaboratories can perform these tests. Many erythrocytes indices have been proposedto determine whether a blood sample is more suggestive for iron deficiency anemiaor β trait thalassemia. Unfortunately these indices have different diagnostic valuein many countries and there is no data about diagnostic value in Indonesia. Thisstudy performs diagnostic test Mentzer, RDW, Green-King, and Sirdah Index anddevelops a new cut-off point that could make a better diagnostic value.This study consists of 98 subjects of iron deficiency anemia and 80 subjectsof β trait thalassemia. Diagnostic values of Mentzer Index for iron deficiencyanemia 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 weresensitivity 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 sensitivity91.8%, specificity 75%, PPV 81.8%, NPV 88.2%, LR+ 3.6, LR- 0.1. Diagnosticvalues of RDW Index for β trait thalassemia were sensitivity 75%, specificity91.8%, 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-KingIndex 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 irondeficiency anemia were sensitivity 92.8%, specificity 58.7%, PPV 73.3%, NPV87.0%, LR+ 2.2, LR- 0.1. Diagnostic values Sirdah Index for β trait thalassemiawere 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 was13.44, 233.4, 75.06, and 32.52 respectively.All indices can be applied for Indonesian people, among which Green-KingIndex had the best diagnostic value, Iron deficiency anemia and β trait thalassemia are the most common causesof microcytic hypochromic anemia in Indonesia. Differentiation between them isdifficult when solely based on a hematology examination, so additional laboratorytests are required such as ferritin and hemoglobin analysis. However, not alllaboratories can perform these tests. Many erythrocytes indices have been proposedto determine whether a blood sample is more suggestive for iron deficiency anemiaor β trait thalassemia. Unfortunately these indices have different diagnostic valuein many countries and there is no data about diagnostic value in Indonesia. Thisstudy performs diagnostic test Mentzer, RDW, Green-King, and Sirdah Index anddevelops a new cut-off point that could make a better diagnostic value.This study consists of 98 subjects of iron deficiency anemia and 80 subjectsof β trait thalassemia. Diagnostic values of Mentzer Index for iron deficiencyanemia 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 weresensitivity 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 sensitivity91.8%, specificity 75%, PPV 81.8%, NPV 88.2%, LR+ 3.6, LR- 0.1. Diagnosticvalues of RDW Index for β trait thalassemia were sensitivity 75%, specificity91.8%, 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-KingIndex 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 irondeficiency anemia were sensitivity 92.8%, specificity 58.7%, PPV 73.3%, NPV87.0%, LR+ 2.2, LR- 0.1. Diagnostic values Sirdah Index for β trait thalassemiawere 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 was13.44, 233.4, 75.06, and 32.52 respectively.All indices can be applied for Indonesian people, among which Green-KingIndex had the best diagnostic value]"

"Defisiensi besi pada pasien penyakit ginjal kronis dengan hemodialisis (PGK-HD) merupakan penyebab terapi erythropoietin-stimulating agent (ESA) menjadi tidak responsif. Soluble transferrin receptor (sTfR) merupakan marker evaluasi status besi serta respons eritropoiesis yang tidak dipengaruhi inflamasi. Indeks sTfR (rasio sTfR/log feritin) diperkirakan dapat meningkatkan performa sTfR. Tujuan penelitian adalah mengevaluasi performa diagnostik sTfR dan indeks sTfR dalam tatalaksana anemia defisiensi besi pada PGK-HD. Penelitian merupakan studi potong lintang yang melibatkan 127 pasien PGK-HD di Unit Hemodialisis RS Cipto Mangunkusumo pada bulan Agustus-September 2018. Setiap subjek diperiksakan sTfR, indeks sTfR, marker besi, feritin, reticulocyte hemoglobin equivalent (RET-He), serta darah perifer lengkap. Saturasi transferin (TSAT) dan RET-He digunakan sebagai baku emas. Uji diagnostik menggunakan Chi Square dan kurva receiver operating characteristic (ROC). Pada penelitian ini didapatkan titik potong sTfR 2,71 mg/L (sensitivitas 83,3%, spesifisitas 56,5%) dan titik potong indeks sTfR 1,39 (sensitivitas 76,2%, spesifisitas 70,6%). Parameter sTfR dapat bermanfaat sebagai skrining dalam penentuan status besi serta respon eritropoiesis pada pasien PGK-HD. Tata laksana terapi besi yang adekuat akan membuat terapi ESA menjadi efektif, sehingga anemia dapat teratasi, dan kualitas hidup pasien membaik.

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Iron deficiency in patients with chronic kidney disease and hemodialysis (CKD-HD) can cause unresponsiveness to erythropoietin-stimulating agent (ESA). Soluble transferrin receptor (sTfR) is a potential marker to evaluate iron status and erythropoiesis response, that’s not influenced by inflammation. The sTfR index (sTfR/log ferritin ratio) has been proposed could increase the diagnostic efficacy than sTfR alone. We evaluated the diagnostic performance of sTfR and sTfR index for management of iron deficiency in CKD-HD. This cross-sectional study was conducted at Cipto Mangunkusumo Hospital, Indonesia from August-September 2018, involving 127 CKD-HD patients. The sTfR level, sTfR index (sTfR/log ferritin), iron status, ferritin level, reticulocyte hemoglobin equivalent (RET-He), and complete blood count were assessed. Transferrin saturation (TSAT) and RET-He were used as references. Diagnostic tests were analyzed using the chi-square test and receiver operating characteristic curve analysis. We identified sTfR cutoff of 2.71 mg/L (sensitivity 83.3%, specificity 56.5%) and sTfR index cutoff of 1.39 (sensitivity 76.2%, specificity 70.6%). The sTfR might be useful as a screening parameter to evaluate iron status and erythropoietin response in CKD-HD patients. Appropriate iron therapy will make ESA therapy more effective, which will help to overcome anemia, and finally will improve the quality of life of CKD-HD patients.

 

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" ABSTRAKLatar Belakang: Thalassemia merupakan hemoglobinopati herediter yang menyebabkan anemia kronis, sehingga pasien membutuhkan transfusi darah secara rutin yang dapat menyebabkan kelebihan besi. Kelebihan besi dapat memicu beberapa komplikasi, salah satunya adalah gangguan pertumbuhan. Tujuan: Mengetahui hubungan antara kadar hemoglobin dan profil besi dengan gangguan pertumbuhan pada pasien thalassemia. Metode: Studi cross-sectional pada 102 pasien thalassemia di Pusat Thalassemia RSCM Jakarta. Hasil: Empat puluh lima 44,1 subjek adalah perempuan dan 57 55,9 subjek adalah lelaki dengan rentang usia 9-14 tahun. Tiga puluh sembilan 38,2 subjek memiliki perawakan pendek dan 63 61,8 subjek memiliki perawakan normal. Nilai median kadar feritin serum pada pasien perawakan pendek adalah 2062 318-8963 ng/mL dan pada pasien perawakan normal adalah 3315 422,9-12269 ng/mL p.

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ABSTRACTBackground Thalassemia is a hereditary hemoglobinopathy which causes chronic anemia, thus the patients need regular blood transfusion which can cause iron overload. It leads to some complications, one of them is growth retardation. Aim To determine the association between hemoglobin level and iron profile with growth retardation on thalassemia patients. Methods cross sectional study on 102 patients in Thalassemia Center of RSCM Jakarta. Results Forty five 44.1 subjects are girls and 57 55.9 subjects are boys. Their age range was 9 14 years old. Thirty nine 38.2 subjects had short stature and 63 61.8 subjects had normal stature. Median of serum ferritin level in the short stature patients was 2062 318 8963 ng mL and normal stature was 3315 422.9 12269 ng mL p 0.001 . Median of transferrin saturation in the short stature patients was 88 19 100 and normal stature was 83 35 100 p 0.94 . Mean of pra transfusion hemoglobin level in the short stature patients was 8.14 SD 0.93 g dL and normal stature was 8.07 SD 0.86 g dL p 0.68 . Conclusion there is a significant association between serum ferritin level and growth retardation, but there is no significant association between transferrin saturation and pra transfusion hemoglobin level with growth retardation."

"Berat plasenta umum digunakan untuk mengukur pertumbuhan dan fungsi yang baik dari plasenta. Anemia merupakan salah satu faktor risiko dari berat plasenta yang tinggi. Namun sampai saat ini belum dapat dipastikan apakah anemia karena kekurangan zat besi mempengaruhi berat plasenta. Penelitian ini meneliti hubungan antara anemia defisiensi besi dan berat plasenta pada wanita hamil anemia di Jakarta Timur. Penelitian ini menggunakan desain penelitian potong lintang yang melibatkan 90 ibu hamil anemia di 10 Puskesmas Kecamatan, Jakarta Timur. Prevalensi defisiensi besi pada ibu hamil anemia adalah 36,9%. Berat plasenta rata-rata adalah 549,3 ± 115 gr. Ada hubungan positif antara anemia defisiensi besi dan berat plasenta setelah dikontrol oleh variabel paritas, perokok pasif dan frekuensi kunjungan pemeriksaan kehamilan (B = 0,22; p = 0,038). Berat plasenta yang tinggi pada ibu hamil dengan anemia defisiensi besi menunjukkan adanya mekanisme adaptasi dari plasenta dikarenakan kurangnya oksigen dalam darah.

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Placental weight is a commonly used to measure placental growth and function including nutrient transfer to the fetus. Anemia may link to a risk factor for higher placental weight, however, it is uncertain whether iron deficiency anemia influence a placental weight, and could be used a public health measure for fetal growth and healthy pregnancy. This study investigated the relationship between iron deficiency anemia and placental weight among anemic pregnant women in East Jakarta. We conducted a cross sectional study of 90 anemic pregnant women and their singleton pregnancies in 10 Primary Health Center in East Jakarta. The prevalence of iron deficiency among anemic pregnant women was 36.9%. The mean placental weight was 549.3 ± 115 gr. There was a positive relationship between iron deficiency anemia and placental weight after adjusting for parity, passive smoker and ANC visit frequency (B=0.22; p=0.038). A higher placenta weight was observed among iron deficiency anemic pregnant women, suggesting the adaptive mechanism of placenta to chronic poor oxygenation."

"ABSTRAKPubertas terlambat merupakan salah satu komplikasi utama pada pasien thalassemia mayor. Penyebab utama pubertas terlambat pada pasien thalassemia mayor adalah penumpukan besi pada kelenjar hipofisis. Selain itu, anemia kronis pada pasien thalassemia mayor juga dapat menyebabkan pubertas terlambat. Tujuan: Penelitian ini bertujuan untuk mengetahui hubungan antara profil besi dan kadar hemoglobin pra-transfusi dengan status pubertas pasien thalassemia mayor remaja di Pusat Thalassemia RSCM. Metode: Penelitian ini merupakan studi cross-sectional yang melibatkan 47 pasien thalassemia mayor dengan rentang usia 13-18 tahun untuk pasien perempuan dan 14-18 tahun untuk pasien lelaki di Pusat Thalassemia RSCM. Profil besi subjek ditentukan dari kadar feritin serum dan saturasi transferin subjek. Status pubertas subjek ditentukan berdasarkan Tanner Staging. Hasil & Diksusi: Berdasarkan kadar feritin serum, terdapat 47 (100%) subjek yang mengalami kelebihan besi, dengan 35 (75%) diantaranya mengalami kelebihan besi berat. Nilai median feritin serum subjek adalah 3645 (1415-12636) ng/mL. Berdasarkan saturasi transferin, sebesar 36 (77%) subjek mengalami kelebihan besi, dengan nilai median saturasi transferin sebesar 85 (28-100)%. Terdapat 42 (89%) subjek yang mengalami anemia, dengan nilai median kadar hemoglobin pra-transfusi sebesar 8,0 (4,8-9,5) g/dL. Pubertas terlambat ditemukan pada delapan (17%) subjek. Secara statistik, tidak terdapat hubungan yang bermakna antara feritin serum dengan status pubertas (p = 0,183), saturasi transferin dengan status pubertas (p = 0,650), dan kadar hemoglobin pra-transfusi dengan status pubertas (p = 0,932). Berdasarkan hasil tersebut, profil besi dan kadar hemoglobin pra-transfusi tidak berhubungan dengan status pubertas pasien thalassemia mayor remaja di Pusat Thalassemia RSCM.

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ABSTRAKIntroduction Delayed puberty is a major complication in thalassemia major patients. Delayed puberty occurs due to accumulation of iron in the pituitary gland. In addition, chronic anemia in thalassemia major patients can cause delayed puberty.Objectives This study aims to find the association between iron profile and pre transfusion hemoglobin level with pubertal status in adolescent thalassemia major patients in Thalassemia Centre RSCM.Methods This was a cross sectional study that involved 47 thalassemia major patients aged 13 to 18 years for female patients and 14 to 18 years for male patients in Thalassemia Centre RSCM. Iron profile was determined from serum ferritin level and transferrin saturation. Pubertal status was determined by Tanner Staging.Results Discussion Based on serum ferritin level, 47 100 subjects had iron overload, in which 35 75 subjects had severe iron overload. The median of serum ferritin level was 3645 1415 12636 ng mL. Based on transferrin saturation, 36 77 subjects had iron overload. The median of transferrin saturation was 85 28 100 . Forty two 89 subjects were found anemic. The median of pre transfusion hemoglobin level was 8,0 4,8 9,5 g dL. Delayed puberty occurred in eight 17 subjects. Statistically, no significant associations were found between serum ferritin level and pubertal status p 0.183 , transferrin saturation and pubertal status p 0.650 and pre transfusion hemoglobin level and pubertal status p 0,932 . Based on the results, iron profile and pre transfusion hemoglobin level are not associated with pubertal status in adolescent thalassemia major patients in Thalassemia Centre RSCM."

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

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

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

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

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

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

"ABSTRAK Latar Belakang: Defisiensi besi merupakan salah satu penyebab anemia pada anak dengan penyakit ginjal kronik PGK derajat 5 yang menjalani hemodialisis HD regular. Pemberian besi intravena IV dosis loading terbukti bermanfaat mengoreksi kekurangan besi, namun belum ada studi yang baik mengenai manfaat pemberian besi IV dosis rumatan setelah terjadi replesi besi, untuk mempertahankan kadar hemoglobin Hb dan profil besi. Metoda: Disain studi adalah kohort retrospektif menggunakan data rekam medis, pada anak usia ABSTRACT Background Iron deficiency is a common cause of anemia in children with chronic kidney disease CKD on hemodialysis HD . Iron repletion with intravenous IV iron sucrose formulations has been studied in children, however effectiveness of maintenance IV iron regimens has not been reported extensively. Methods We conducted a retrospective cohort study on children with CKD on HD. Medical records were reviewed on all patients at the Children rsquo s Kidney Center, Cipto Mangunkusumo Hospital between January 1, 2015 and May 31, 2016. Patients with normal hemoglobin Hb and iron values were grouped into patients received IV iron sucrose maintenance and patients without IV iron sucrose maintenance. In the first group, patients received 2 mg kg dose of IV iron sucrose once every other week for 2 doses. Laboratory tests for Hb and iron values were recorded twice. First laboratory test was the baseline, shows normal Hb and iron values in both groups. The second laboratory test was taken after 6 weeks of baseline test. Objectives We assessed effects of IV iron sucrose maintenance by the difference in proportions of iron deficiency anemia between two groups based on repeat laboratory test 6 weeks after baseline test. Results During the study period, a total of 74 children had normal Hb and iron values at the beginning of the study. Forty seven patients received IV iron sucrose maintenance and 27 children did not receive IV iron maintenance. Repeat laboratory test 6 weeks after baseline test shows proportion of iron deficiency anemia was 5 47 10.64 in patients with IV iron and 16 27 59.26 in patients without IV iron. Between group difference for proportions was 48.62 P"