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ABSTRAK
Latar belakang. Pada penderita thalassemia yang telah lama mendapat transfusi, nilai hemoglobin pasca transfusi tidak bertahan sesuai dengan yang diharapkan. Telah dilaporkan mengenai terbentuknya aloantibodi dan autoantibodi pada penderita thalassemia, yang kemungkinan menyebabkan hemoglobin tidak bertahan pasca transfusi.

Tujuan. Penelitian ini bertujuan untuk mencari faktor-faktor yang berhubungan dengan kegagalan mempertahankan hemoglobin pasca transfusi pada penderita thalassemia, terkait dengan terbentuknya aloantibodi dan autoantibodi terhadap eritrosit.

Metodologi. Studi potong lintang di poliklinik Hematologi Onkologi Medik IPD RSCM bulan Juli-September 2012 pada penderita thalassemia dewasa yang tergantung transfusi, tanpa penyakit autoimun lain. Dilakukan pemeriksaan sampel darah dengan teknik column gel aglutination untuk melihat adanya aloantibodi dan autoantibodi. Sebelas sel panel reagen digunakan untuk mendeteksi dan mengidentifikasi aloantibodi. Aloantibodi dan autoantibodi positif didefinisikan sebagai pemeriksaan IAT dan DAT yang positif. Dilakukan analisis bivariat antara aloantibodi dan autoantibodi dengan jenis kelamin, jenis rhesus, kadar feritin, jenis kelasi besi, dan aloantibodi.

Hasil. Dari 88 subjek, didapatkan 37,5% subjek nilai hemoglobin pasca transfusi tidak bertahan seperti yang diharapkan. Dari 33 subjek tersebut didapatkan aloantibodi dan autoantibodi positif masing-masing 78,6% dan 72,7%. Dari 24 pasien dengan autoantibodi didapatkan 25% dengan derajat hemolitik yang secara klinis bermakna. Aloantibodi positif berhubungan dengan terbentuknya autoantibodi (p < 0,000). Aloantibodi positif [odss ratio (OR) = 26,32; p < 0,000), autoantibodi positif (OR = 11,99; p < 0,000), dan feritin > 3000 ng/ml (OR = 6,36; p < 0,042) berhubungan dengan kegagalan mempertahankan hemoglobin pasca transfusi.

Simpulan. Proporsi hemoglobin pasca transfusi tidak bertahan sesuai dengan yang diharapkan pada penderita thalassemia dewasa sebesar 37,5%. Proporsi terbentuknya aloantibodi dan autoantibodi pada kelompok tersebut sebesar 78,6% dan 72,7%. Faktor yang berhubungan dengan kegagalan mempertahankan hemoglobin pasca transfusi adalah aloantibodi positif, autoantibodi positif, dan feritin > 3000 ng/ml. Aloantibodi positif berhubungan dengan terbentuknya autoantibodi.

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ABSTRACT
Background. In transfusion dependent thalassemia patients who has got repeated transfusion for a period of time, the haemoglobin level after transfusion could not be maintained appropriately to be expected. The production of erythroyte alloantibody and autoantibody in transfusion dependent thalassemia patients has been reported before. These antibodies were probable related to the failure on maintaning haemoglobin level after transfusion.

Objective. To find related factors of failure on maitaining haemoglobin level after transfusion in adult transfusion dependent thalassemia patients related to erythroyte alloantibody and autoantibody production.

Material and Methods. Cross sectional study of adult transfusion dependent thalassemia patient without others autoimune disease at Haematology and Medical Oncology outpatient clinic in Cipto Mangunkusumo hospital from July to September 2012 was done. The specimen was subjected to erythroyte alloantibody and autoantibody evaluation by column gel agglutination technique. Eleven cell reagent panel were used in screening and identification of alloantibody and autoantibody respectively. Positive alloantibody is defined as positivity of indirect antiglobulin test and positive autoantibody is defined as positivity of direct antiglobulin test. Statistic analysis between erythrocyte alloantibody and autoantibody positivity and sex, type of rhesus, feritin level, type of iron chelation, and alloantibody were done.

Results. From 88 subjects, there were 37,5% thalassemia patients that did not maintain haemoglobin level after transfusion. From 33 of those subjects, there were 78,6% subjects with alloantibody and 72,7% subjects with autoantibody. From 24 patients with autoantibody, there were 25% subjects with severe hemolytic anemia that clinically significant. Positif alloantibodi related to autoantibody production (p < 0,000). Positive alloantibody [odds ratio (OR) = 26,32; p < 0,000], positive autoantibody (OR = 11,99; p < 0,011), and feritin level > 3000 ng/ml (OR = 6,36; p < 0,042) related to failure on maintaining haemoglobin level.

Conclusion. The proportion of failure on maintaining haemoglobin level in adult thalassemia patients were 37,5%. The proportion alloantibody and autoantibody production in adult thalassemia patients that failure on maintaining haemoglobin level were 78,6% and 72,7% respectively. Related factors of those were positive alloantibody and autoantibody, and feritin level > 3000 ng/ml. Positive alloantibody related to autoantibody production.

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Latar Belakang: Proses destruksi trombosit pada pasien trombositopenia imun primer diduga terutama terjadi melalui perantaraan antibodi terhadap glikoprotein permukaan trombosit. Antibodi anti-GPIIb/IIIa dan anti-GPIb/IX merupakan antibodi yang spesifik terhadap trombosit dan megakariosit. Beragamnya karakteristik klinis serta respon terapi pada pasien trombositopenia imun dipikirkan dipengaruhi oleh heterogenitas kompleks glikoprotein spesifik. Belum ada data tentang profil antibodi antiglikoprotein permukaan trombosit pada pasien trombositopenia imun primer dewasa. Tujuan: Mengetahui profil antibodi antiglikoprotein permukaan trombosit pada pasien trombositopenia imun primer dewasa. Metode: Penelitian ini merupakan studi potong lintang dengan subjek pasien trombositopenia imun primer dewasa di RSUPN Cipto Mangunkusumo pada bulan Maret-Oktober 2013. Variabel yang diteliti adalah antibodi anti-GPIIb/IIIa dan anti-GPIb/IX dengan teknik MAIPA direk. Hasil: Didapatkan 40 subjek selama penelitian. Sebanyak 10 subjek dengan diagnosis trombositopenia imun primer newly diagnosed yang belum mendapatkan terapi dan 30 subjek trombositopenia imun primer persisten/kronik yang sudah atau pernah mendapatkan terapi di Poliklinik Hematologi Onkologi Medik Departemen Ilmu Penyakit Dalam RSUPNCM selama penelitian. Median usia seluruh subjek adalah 24,5 tahun (rentang 17-55) dengan 82,5% subjek berjenis kelamin perempuan. Tujuh puluh persen subjek memiliki skor ITP Bleeding Score (IBLS) 2. Persentase respon komplit, respon, dan tidak respon terhadap terapi kortikosteroid adalah 42,5%, 45%, dan 12,5%. Pada kelompok trombositopenia imun kronik/persisten proporsi antibodi anti-GPIIb/IIIa adalah 20/30 dengan median optical density (OD) 0,461(rentang 0,093-2,116) dan proporsi antibodi anti-GPIb/IX adalah 25/30 dengan median OD 0,507(rentang 0,190-1,924). Pada kelompok trombositopenia imun newly diagnosed proporsi antibodi anti-GPIIb/IIIa adalah 7/10 dengan rerata OD 0,802±0,71 dan proporsi antibodi anti-GPIb/IX adalah 8/10 dengan rerata OD 0,82±0,57. Simpulan: Sebesar 85% pasien trombositopenia imun primer memiliki antibodi anti-GPIIb/IIIa dan anti-GPIb/IX. Terdapat proporsi respon terhadap terapi yang hampir sama antara subjek yang memiliki maupun tidak memiliki antibodi anti-GPIIb/IIIa dan anti-GPIb/IX.

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ABSTRAK
Background: Platelet destructions in immune thrombocytopenia was mediated by autoantibodies against platelet antigen. Antibody anti-GPIIb/IIIa and anti-GPIb/IX were two major antibodies spesific for platelet and megakaryocytes. The diversity of clinical characteristics and therapeutic responses was thought to be influenced by heterogeneity of spesific glycoprotein complexes. There were no data about antiplatelet antibody profile in adult primary immune thrombocytopenia (ITP). Objectives: To find out antiplatelet glycoprotein antibody profile in adult primary immune thrombocytopenia. Methods: This is a cross sectional study of adult primary immune thrombocytopenia patients who attend Hematology Medical Oncology Polyclinic Internal Medicine Department Cipto Mangunkusumo Hospital in March to October 2013. Antibodies against the platelet GPIIb/IIIa and GPIb/IX were performed by direct MAIPA technique. Results: A total of 40 patients who had been diagnosed with primary immune thrombocytopenia attended to Polyclinic Hematology Medical Oncology Internal Medicine Department Cipto Mangunkusumo Hospital during the study. The subjects divided in two groups, 10 subjects with newly diagnosed ITP, and 30 subjects with persistent or chronic ITP. Median age for this study is 24,5 (17-55) years, with 82,5% were female. Seventy subjects have ITP Bleeding Score (IBLS) 2. Response to corticosteroid therapy in all subjects were complete response 42,5%, response 45%, and no response 12,5%. In persistent/chronic ITP group proportion of anti-GPIIb/IIIa is 20/30 with median optical density (OD) 0,461(0,093-2,116) and proportion of anti-GPIb/IX is 25/30 with median OD 0,507(0,190-1,924). In newly diagnosed ITP group proportion of anti-GPIIb/IIIa is 7/10 with mean OD 0,802±0,71 and proportion of anti-GPIb/IX is 8/10 with mean OD 0,82±0,57. Conclusion: Eighty five percent of primary immune thrombocytopenia have antibody anti GPIIb/IIIa and anti GPIb/IX. In response to therapy, there were similary response between subjects that with and without antibody anti-GPIIb/IIIa and anti-GPIb/IX.

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Latar Belakang. Lean NAFLD lebih sering ditemukan di negara Asia dan prevalensinya di Indonesia masih belum diketahui. Tingginya prevalensi, asimptomatik dan baru bergejala setelah timbul komplikasi, dan tingginya mortalitas lean NAFLD menjadikan perlunya deteksi dini pada populasi dewasa dengan IMT <23 kg/m2. Skrining pada populasi umum tidak direkomendasikan karena meningkatkan biaya kesehatan. Tujuan. Membuat sistem skoring untuk penapisan lean NAFLD pada populasi dewasa di Jakarta. Metode. Studi ini menggunakan desain potong lintang dari laporan pemeriksaan kesehatan individu dewasa >18 tahun dengan IMT <23 kg/m2 yang melakukan pemeriksaan kesehatan di klinik. Parameter yang dianalisis antara lain usia, jenis kelamin, lingkar pinggang, kadar GDP, kolesterol total, HDL, LDL, trigliserida, AST, ALT, dan asam urat. Variabel dengan nilai p <0,25 dilanjutkan ke analisis multivariat untuk pembuatan sistem skoring. Hasil. Sebanyak 276 individu diikutsertakan pada penelitian ini dan didapatkan prevalensi lean NAFLD sebesar 9,8%. Lean NAFLD lebih banyak ditemukan pada laki-laki dan memiliki karakteristik usia lebih tua, IMT, lingkar pinggang, kadar GDP, ALT, dan trigliserida lebih tinggi dibanding lean tanpa NAFLD. Analisis bivariat mendapatkan jenis kelamin laki-laki, usia ≥45 tahun, kadar GDP ≥100 mg/dL, ALT ≥35 U/L, dan trigliserida ≥150 mg/dL berhubungan dengan lean NAFLD. Sistem skoring melibatkan 4 parameter yaitu laki-laki, kadar GDP ≥100 mg/dL, ALT ≥35 U/L, dan trigliserida ≥150 mg/dL dengan masing-masing bernilai 1 poin. Model skoring ini memiliki sensitivitas 44,4%, spesifisitas 84,3%, dan AUROC 0,74. Kesimpulan. Parameter jenis kelamin, kadar GDP, ALT, dan trigliserida dapat digunakan sebagai sistem skoring dengan performans menengah untuk penapisan lean NAFLD dewasa. .....Background. Lean NAFLD is commonly found in Asian countries and its prevalence in Indonesia is still unknown. The high prevalence, asymptomatic until complications occur, and the high mortality of lean NAFLD makes it necessary for early detection in adult with BMI <23 kg/m2. Screening in general population is not recommended due to the high cost burden. Aim. To develop a scoring system for screening lean NAFLD in adults in Jakarta Methods. A cross-sectional study design was conducted from medical examination reports from individual >18 years old and BMI <23 kg/m2 who performed medical check up at the clinic. Several parameters including age, gender, waist circumference (WC), fasting blood glucose (FBG), total cholesterol (TC), HDL, LDL, triglycerides (TG), AST, ALT, and uric acid (UA) were analyzed in this study. Variabels with p-value <0.25 were included in multivariate analysis for the development of scoring systems. Results. A total of 276 people were enrolled in this study. Prevalence of lean NAFLD is 9.8%. Lean NAFLD are more commonly found in men and have older age, higher BMI, WC, GDP, ALT, and TG levels than lean non-NAFLD. In bivariate analysis, male sex, age ≥ 45 years, FBG ≥100 mg/dL, ALT ≥35 U/L, and TG ≥150 mg/dL are associated with lean NAFLD. The scoring system involves four parameters including male, FBG ≥100 mg/dL, ALT ≥35 U/L, and TG ≥150 mg/dL, worth 1 point each. This model has sensitivity 44.4%, specificity 84.3%, and AUROC 0.74. Conclusion. Parameters including gender, FBG, ALT, and TG levels can be used as a scoring system with moderate performance for screening lean NAFLD in adults.

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Latar Belakang. Hemofilia selama ini diketahui menimbulkan komplikasi muskuloskeletal dan penurunan densitas tulang adalah salah satunya. Faktor risiko penurunan densitas tulang pada hemofilia belum diketahui secara pasti. Profil pasien hemofilia dengan penurunan densitas tulang di Indonesia juga belum diketahui. Tujuan. Mengetahui proporsi penurunan densitas tulang pada hemofilia dan karakteristik pasien hemofilia dengan penurunan densitas tulang. Metode. Studi ini merupakan studi deskriptif potong lintang yang dilakukan pada bulan Juni-November 2012. Subyek penelitian adalah pasien hemofilia dewasa berusia 19-50 tahun yang berobat ke Poliklinik Hematologi Onkologi Medik RS Cipto Mangunkusumo atau yang terdaftar di Tim Hemofilia Terpadu berdomisili di area Jabodetabek. Variabel yang dinilai adalah densitas massa tulang, usia, indeks massa tubuh, aktivitas fisik, artropati, penggunaan terapi substitusi, infeksi HIV dan HCV. Densitas tulang diukur dengan Lunar GE Scan. Penurunan densitas tulang didefinisikan sebagai Z-score -2 atau kurang. Aktivitas fisik dinilai dengan kuesioner Hemophilia Activities List. Artropati secara klinis dinilai dengan Hemophilia Joint Health Score. Artropati secara radiologis dinilai pada sendi lutut menggunakan Skor Arnold-Hilgartner. Data numerik dinyatakan dalam mean + SD atau median. Data kategorik dinyatakan dalam n dan persentase. Hasil. Sejumlah 63 subyek hemofilia dewasa berusia 19-46 tahun mengikuti studi ini dengan median usia 26 tahun. Proporsi penurunan densitas tulang pada hemofilia didapatkan sebesar 6,3%. Fraktur terjadi pada 14,3% subyek. Subyek dengan densitas tulang menurun memiliki usia lebih muda (19 tahun vs 26 tahun). Subyek dengan densitas tulang menurun memiliki IMT lebih rendah (18,6 + 2,8 kg/m2 vs 21,5 + 3,8 kg/m2). Subyek dengan densitas tulang menurun menggunakan terapi substitusi lebih banyak daripada subyek dengan densitas tulang normal (4047 IU/bulan vs 2000 IU/bulan). Infeksi HCV terjadi pada 25% subyek dengan densitas tulang menurun sedangkan pada densitas tulang normal sebesar 55,9%. Infeksi HIV hanya terjadi pada 1,6% subyek. Skor aktivitas ditemukan sama antara subyek dengan densitas tulang normal dan menurun. Skor artropati klinis ditemukan lebih baik pada subyek dengan densitas tulang menurun (18,7 + 4,4 vs 23,1 + 11,8). Simpulan. Penurunan densitas tulang pada subyek hemofilia ditemukan sebesar 6,3%. Subyek dengan densitas tulang menurun berusia lebih muda, memiliki IMT lebih rendah, skor sendi lebih baik, lebih sedikit mengalami infeksi transfusi, dan mengalami perdarahan lebih banyak dibandingkan subyek dengan densitas tulang normal.

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Background. Haemophilia can result in musculoskeletal complications and reduced bone density is one of the recently known musculoskeletal complications in haemophilia patients. Risk factors of reduced bone density in haemophilia have not been completely known yet. Moreover, profile of hemophilia patient with reduced bone density in Indonesia have not been studied. Objectives. To know the proportion of reduced bone density and characteristics of hemophilia patient with reduced bone density. Methods. A cross-sectional study on haemophilia patients aged 19-50 years old was conducted ini Haematology-Medical Oncology Clinic, Cipto Mangunkusumo Hospital from June-November 2012. Bone density, age, body mass index, physical activity, arthropathy, amount of replacement therapy, HIV and HCV infection are analyzed variables. Bone density was measured with GE Lunar Scan. Reduced bone density was defined as Z-score -2 or less. Physical activity was measured with Haemophilia Activities List questionnaire. Joint involvement was measured clinically with Haemophilia Joint Health Score. Joint involvement of the knee was measured radiologically with plain X-ray and graded according to Arnold-Hilgartner Score. Numerical data will be presented in mean + SD or median. Categorical data will be presented as n and percentage. Results. Sixty three haemophilia subjects aged 19-46 years old joined the study with median age 26 years old. Reduced bone density was found in 6,3% of the subjects. History of fractures was found in 14,3% patient. Subjects with reduced bone density have younger age (19 vs 26 years). Subjects with reduced bone density have lower BMI (18,6 + 2,8 kg/m2 vs 21,5 + 3,8 kg/m2). Subjects with reduced bone density used replacement therapy more than their normal counterparts (4047 IU/month vs 2000 IU/month). HCV infection happened in 25% of subjects with reduced bone density while only found in 55% of normal bone density subjects. HIV infection was only found in 1,6% patient. Activity score between normal and reduced bone density was about the same. Clinical arthropathy score was better in reduced bone density subjects (18,7 + 4,4 vs 23,1 + 11,8). Conclusion. Reduced bone density was found in 6,3% subjects. Subjects with reduced bone density have younger age, lower BMI, better joint score, less infection, and experienced more bleeding than subjects with normal bone density.

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LATAR BELAKANG: Prevalensi GERD di Indonesia semakin meningkat.GERD dapat memengaruhi kualitas hidup penderitanya. Kuesioner GERD Qualityof Life GERD-QOL telah diuji keandalan dan kesahihannya di Cina. Penelitianini bertujuan untuk menterjemahkan kuesioner GERD-QOL ke dalam bahasaIndonesia dan menguji keandalan dan kesahihannya. METODE: Sebagai tahap awal, kuesioner GERD-QOL terlebih dahuluditerjemahkan dengan metode forward backward translation ke dalam bahasaIndonesia, dan dievaluasi oleh tim peneliti sehingga dihasilkan kuesioner GERDQOLversi bahasa Indonesia. Sembilan puluh satu orang pasien yang telahdidiagnosis GERD secara klinis sebelumnya berdasarkan kriteria Montreal,diwawancarai dengan menggunakan kuesioner GERD-QOL versi Indonesia dankuesioner SF-36. Keandalan dinilai melalui metode konsistensi internal dan tesulang dengan mewawancarai pasien pada hari pertama dan hari ke-14. Kesahihan dinilai menggunakan kesahihan konstruksi dan kesahihan eksternal melaluiperbandingan dengan SF-36. HASIL: GERD-QOL berbahasa Indonesia memiliki keandalan konsistensiinternal kuesioner yang baik Cronbach alpha: 0,687 ndash;0,842 dengan keandalantes ulang yang baik intra class correlation coefficient: 0,756-0,936, P ......BACKGROUND: GERD prevalence in Indonesia has been increasing. GERDcan affect quality of life. GERD Quality of Life GERD QOL questionnaire hasbeen translated and validated in China. This study is aimed to translate GERDQOLquestionnaire into Indonesian version and to assess its validity andreliability. METHODS: GERD QOL is translated into bahasa Indonesia using forwardbackward translation and compared by experts to original version. Total of 91patients have been diagnosed clinically with GERD based on Montreal consensus,were recruited to complete the questionnaire and validated Indonesian SF 36.Reliability was conducted by using internal consistency and test retest methodwith 14 days interval. Validity was conducted by using construct validity andexternal validity with SF 36 comparison method. RESULT Indonesian version of GERD QOL was internally reliable withCronbach Alpha 0.822 and had good test and retest reliability intra classcorrelation coefficient 0.756 0.936, P

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Peningkatan kasus diabetes melitus tipe 2 (DMT2) dengan berbagai komplikasinya memberikan dampak gangguan fungsional seseorang dalam bentuk gangguan kognitif dan kapasitas fisik. Keduanya masih reversibel dan baru diketahui berhubungan sehingga disebut sebagai PhysioCognitive Decline Syndrome (PCDS). Kondisi PCDS baru dipelajari pada lansia dan belum spesifik pada penyandang DMT2. Tujuan Mengetahui korelasi antara kendali glikemik dengan komponen physiocognitive decline syndrome pada penyandang DMT2 dewasa usia pertengahan. Metode Studi potong lintang menggunakan consecutive sampling dari pasien di poliklinik metabolik endokrin dan poli jantung terpadu sejak Januari 2021 – November 2022. Subjek DMT2 berusia 40 – 59 tahun diinklusi. Pemeriksaan kekuatan genggam tangan, dan kecepatan berjalan 6-meter diperiksakan di ruangan standar. MoCA-Ina dilakukan oleh dokter yang telah dilatih. Data HbA1c subjek yang diperiksa adalah HbA1c 3 bulan terakhir. Analisis korelasi Pearson’s atau Spearman’s pada SPSS 20.0 dilakukan sesuai sebaran data. Hasil Sebanyak 133 subjek telah dianalisis. Usia median mencapai 53 tahun dengan proporsi laki-laki dan perempuan serta komplikasi pada masing-masing kateori kendali glikemik (batas HbA1c 7,0%) serupa. Subjek didominasi dengan pendidikan SMA dan Sarjana/Diploma. Median durasi terdiagnosisnya diabetes melitus mencapai 7 tahun dengan HbA1c median 7.6%. Nilai MoCA-Ina pada subjek mencapai nilai median 24 dengan kecepatan berjalan rerata 1.02 + 0.23 m/detik dan median kekuatan genggam tangan 24 kg. Terdapat korelasi bermakna hanya pada HbA1c dengan kekutan genggam tangan (r = -0.24, R2 = 0.06, p value <0.01), terutama pada perempuan Kesimpulan Terdapat korelasi bermakna antara kendali glikemik dan kekuatan genggam tangan. ......Increasing cases of type 2 diabetes melitus (T2DM) including its complication have caused functional dysfunction consisted of cognitive decline and physical incapacity. Both cognitive decline and physical incapacity had been just known to be reversible and related to each other, so it is termed as PhysioCognitive Decline Syndrome (PCDS). However, it had been just evaluated in geriatric and not specific to T2DM patient. To investigate the correlation between glycaemic correlation and component of physiocognitive decline syndrome in middle-aged adult with T2DM. A cross sectional study with consecutive sampling in our metabolic and endocrine clinic and integrated heart centre in January 2021 – November 2022 had been conducted. Inclusion criteria was 40 – 59 years old subjects with T2DM. Measurement of HbA1c in the last 3 month were analysed, while hand grip strength and gait speed were done in standard room. MoCA-Ina had been conducted by trained doctor. Correlation analysis using Pearson’s or Spearman’s in SPSS 20.0 was done according to data distribution. 133 subjects were analysed. Median age was 53 years old with both sex and complication within each glycaemic control category (HbA1c 7,0% cut off) were similar. Subjects were dominated by high school and undergraduate/diploma education level. Most subjects were diagnosed in up to 7 years of T2DM. Median of HbA1c levels in our study was 7.6%. MoCA-Ina score was 24 in median with mean of gait speed was 1.02 + 0.23 m/s. Our median for hand grip was 24 kg. Significant correlation was only found in relationship of HbA1c and hand grip strength (r = -0.24, R2 = 0.06, p value <0.01). There was significant correlation between glycaemic control and hand grip strength.

Abstrak :
Latar Belakang: Panjang interval QTc dipengaruhi oleh berbagai faktor, salah satunya oleh inflamasi. Pada pasien COVID-19 sering terjadi badai sitokin sehingga menyebabkan peningkatan signifikan dari sitokin inflamasi, termasuk interleukin 6. Peningkatan interleukin 6 menyebabkan perubahan pada kanal ion kardiomiosit sehingga menyebabkan pemanjangan interval QTc yang berisiko aritmia. Tujuan: Mengetahui korelasi dengan melihat beda rerata kadar interleukin 6 dan panjang interval QTc, nilai titik potong kadar interleukin 6 terhadap panjang interval QTc > 500 ms dan kekuatan kadar interleukin 6 dalam menilai risiko aritmia ventrikular. Metode: Penelitian ini menggunakan desain studi potong lintang dengan mengambil data sekunder rekam medik pasien COVID-19 yang menjalani rawat inap di RSCM Kiara sejak November 2020 hingga Maret 2021. Pada penelitian ini dilakukan analisis bivariat menggunakan uji Spearman. Selanjutnya dilakukan analisis terhadap beda rerata kadar interleukin 6 pada kelompok subyek dengan panjang interval QTc > 500 ms dan kelompok subyek dengan panjang interval QTc normal. Dilakukan analisis dengan Receiver Operating Curve (ROC) untuk melihat Area under curve (AUC) dan menentukan titik potong kadar interleukin 6 terhadap panjang interval QTc > 500 ms. Hasil: Pada penelitian ini didapatkan korelasi kadar interleukin 6 dan panjang interval QTc (r=0,72). Median kadar interleukin 6 pada kelompok subyek dengan interval QTc > 500 ms yaitu 99,36 pg/ml sedangkan pada kelompok subyek dengan interval QTc normal yaitu 19,51 pg/mL. Didapatkan AUC=0,852 untuk menentukan titik potong kadar interleukin 6 terhadap panjang interval QTc > 500 ms dengan nilai 59 pg/ml, dengan sensitivitas 80,6% dan spesifisitas 80%. Kejadian aritmia ventrikular tidak ditemukan sehingga tidak dapat dilakukan analisis untuk menilai kekuatan kadar interleukin 6 untuk menentukan risiko aritmia ventrikular. Kesimpulan: Terdapat korelasi kadar interleukin 6 dan panjang interval QTc dengan beda rerata kadar interleukin 6 pada subyek dengan interval QTc > 500 ms 5 kali lebih besar dibandingkan kelompok subyek dengan panjang interval QTc normal. Kadar interleukin 6 59 pg/mL ditentukan sebagai nilai titik potong terhadap panjang interval QTc > 500 ms. ......Background: The length of the QTc interval is influenced by various factors, one of which is inflammation. In COVID-19 patients, cytokine storms often occur, causing a significant increase in inflammatory cytokines, including interleukin 6. An increase in interleukin 6 can cause changes in the ion channels of cardiomyocytes, which can lead to prolonged QTc interval which is at risk of arrhythmias. Objective: Knowing the correlation by looking at the differences in interleukin 6 levels and the length of the QTc interval, the cut-off value of interleukin 6 levels to the length of the QTc interval > 500 ms and the strength of interleukin 6 levels in assessing the risk of ventricular arrhythmias. Method: This study used a cross-sectional study design by taking secondary data from the medical records of COVID-19 patients who were hospitalized at RSCM Kiara from November 2020 to March 2021. In this study, a bivariate analysis was carried out using the Spearman test. Furthermore, an analysis of the mean difference in interleukin 6 levels was carried out in the subject group with a QTc interval length> 500 ms and the subject group with a normal QTc interval length. Analyzes were performed using the Receiver Operating Curve (ROC) to see the area under curve (AUC) and determine the interleukin 6 cutoff point for the QTc interval length> 500 ms. Result: The correlation between interleukin 6 levels and the length of the QTc interval (r=0.72) was found. The median level of interleukin 6 in the group of subjects with a QTc interval > 500 ms was 99.36 pg/ml while in the group of subjects with a normal QTc interval it was 19.51 pg/mL. AUC = 0.852 was obtained to determine the cut-off point for interleukin 6 levels to the QTc interval length > 500 ms with a value of 59 pg/ml, with a sensitivity of 80.6% and specificity of 80%. The incidence of ventricular arrhythmias was not found so that an analysis could not be performed to assess the power of interleukin 6 levels to determine the risk of ventricular arrhythmias. Conclusion: There is a correlation between levels of interleukin 6 and the length of the QTc interval. The mean difference of interleukin 6 levels in subjects with QTc intervals> 500 ms was 5 times greater than those in groups of subjects with normal QTc interval lengths. The level of interleukin 6 59 pg / mL was determined as the cutoff value for the QTc interval length> 500 ms.

Abstrak :
Hipertrofi ventrikel kiri atau Left ventricle hypertrophy (LVH) adalah faktor risiko independen terjadinya gagal jantung pada pasien hipertensi. Diagnosis dini LVH diperlukan untuk mencegah kerusakan lebih lanjut pada otot jantung. Cardiotropin-1 (CT-1) diproduksi oleh kardiomiosit dan fibroblas, yang kadarnya dilaporkan meningkat pada pasien hipertensi primer. Tujuan : Membuktikan manfaat CT-1 serum untuk mendeteksi LVH pada pasien hipertensi primer. Metode : Penelitian uji diagnostik dilaksanakan di RSCM Jakarta periode Februari s/d Maret 2013. Subyek penelitian adalah 75 pasien hipertensi primer dengan atau tanpa LVH. Diagnosis LVH dilakukan dengan ekokardiografi sebagai baku emas dan elektrokardiografi/EKG (kriteria Sokolow Lyon voltage, Cornell voltage dan Cornell product). Kadar CT-1 serum diperiksa dari sampel darah vena dengan metode ELISA. Hasil : Berdasarkan ekokardiografi 46 orang (61,3%) LVH dan 29 orang (38,7%) tidak LVH. Kadar CT-1 subyek LVH adalah 82,96 ± 351,843 pg/mL dan subyek tanpa LVH 4,55 ± 1,281 pg/mL (p=0,01). Korelasi CT-1 dengan LVMI adalah tidak bermakna (p=0,1). Luas area dibawah kurva ROC CT-1 untuk diagnosis LVH adalah 0,67 (p=0,01). Nilai cut-off CT-1 adalah 4,45 pg/mL. Uji diagnostik CT-1: Sensitifitas 54,4%, spesifisitas 75,9, NDP 78,1%, NDN 51,2 dan akurasi 61,3%. Uji diagnostik kombinasi CT-1 dan EKG (salah satu kriteria positif LVH): sensitifitas 67,4%, spesifisitas 72,4% , NDP 79,5%, NDN 58,3% dan akurasi 69,3%. Simpulan. CT-1 kurang sensitif namun cukup spesifik untuk diagnosis hipertrofi ventrikel kiri (LVH). Kombinasi CT-1 dengan EKG meningkatkan nilai diagnostik pemeriksaan untuk deteksi LVH pada pasien hipertensi primer.

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Left ventricle hypertrophy (LVH) is independent risk factor of heart failure on hypertension patients. Early detection of LVH is necessary to prevent extensive damage of heart muscle. Cardiotropin-1 (CT-1) is produce by cardiomyosite and fibroblast, that the level of CT-1 has been reported increase on primary hypertension patients. Aim : To prove the benefit of CT-1 serum to detect LVH on primary hypertension patients. Methods : A diagnostic study has been conducted on RSCM Jakarta on the periode of February to March 2013. Research subjects were 75 primary hypertension patients with and without LVH. LVH diagnosis was performed by echocardiography examination as gold standard and electrocardiography/ECG (Sokolow Lyon voltage, Cornell Voltage and Cornell product criterias). CT-1 level was measured by ELISA method from vein blood sample. Results : Based on echocardiography examination 46 patients (61.3%) were diagnosed as LVH and 29 patients (38.7%) without LVH. The level of CT-1 of patients with LVH was 82.96 ± 351.843 pg/mL and 4.55 ± 1.,281 pg/mL on patients without LVH (p=0.01). Correlation between CT-1 and Left Ventricular Mass Index was not significant (p=0.1). Area under the ROC curve was 0.67 (p=0.01). The cut-off of CT-1 level for diagnosis of LVH was 4.45 pg/mL. Diagnostic test yield the sensitivity of CT-1 for diagnosis of LVH was 54.4%, specificity 75.9%, PPV 78.1%, NPV 51.2% and accuracy was 61.3%. Diagnostic test of combination CT-1 and ECG (positive LVH by one or more ECG’s criteria) yield sensitivity 67.4%, specificity 72.4% , PPV 79,5%, NPV 58.3% and accuracy 69.3%. Conclusion. CT-1 examination was not sensitive but specific for LVH diagnosis. Combination of CT-1 and ECG examination was improve diagnostic value of CT-1 for detection of LVH on primary hypertension patients.

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ABSTRAK Hemartrosis berulang dan artropati merupakan morbiditas utama pada hemofilia A berat. Bagi negara berkembang seperti Indonesia, terapi profilaksis dosis standar tidak terjangkau karena memerlukan biaya yang sangat mahal. Penelitian ini bertujuan mengetahui efektivitas terapi profilaksis sekunder dosis rendah dibandingkan terapi on-demand pada anak hemofilia A berat. Uji klinis acak terbuka selama 24 minggu telah dilakukan pada anak hemofilia A berat berusia 4?18 tahun dengan riwayat perdarahan sendi berulang, di Poliklinik Hematologi Departemen Ilmu Kesehatan Anak FKUI/RSCM. Subjek dialokasikan secara acak menjadi dua kelompok yaitu kelompok profilaksis dan on-demand. Kelompok profilaksis mendapat terapi faktor VIII 10 IU/kgBB 2 kali seminggu, sedangkan kelompok on-demand mendapat terapi sesuai protokol standar. Luaran primer adalah kekerapan perdarahan sendi dan luaran sekunder adalah skor HJHS) dan skor ultrasonografi (HEAD-US). Penelitian ini juga membandingkan kadar CTX-II urin dan inhibitor faktor VIII (Bethesda Assay) pada kedua kelompok. Sejak bulan Juni 2015?Februari 2016 didapatkan 50 subjek yang memenuhi kriteria inklusi. Kekerapan perdarahan sendi pada kelompok profilaksis (5 ± 4,3) lebih baik dari pada kelompok on-demand (8 (3?30)), IK95% 0.9?6.99; p = 0,009. Perubahan skor HJHS pada kedua kelompok menunjukkan perbaikan klinis pada kelompok profilaksis dan perburukan pada kelompok on-demand, walaupun tidak bermakna secara statistik (IK95% -0.99?3; p = 0,320). Skor HEAD-US kelompok profilaksis lebih baik dibandingkan kelompok on-demand (IK95% 2? 8,81; p = 0,003). Perubahan kadar CTX-II urin pada kedua kelompok berbeda bermakna (IK95% 2.777?16.742; p < 0,001). Tidak didapatkan subjek yang terbentuk inhibitor faktor VIII pada kedua kelompok selama penelitian. Dari hasil penelitian ini disimpulkan bahwa terapi profilaksis sekunder dosis rendah efektif mengurangi kekerapan perdarahan sendi, memperbaiki skor HEAD-US dan kadar CTX-II urin, dibandingkan terapi on-demand.

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ABSTRACT Repeated joint bleeds leading to irreversible progressive joint damage (hemophilic arthropathy) is the main problem in children with hemophilia. Current standard prophylacytic treatment in developed countries is beyond our capability as Indonesia has constraint resources. This study aimed to investigate the efficacy and safety of low dose secondary prophylaxis compare to on-demand treatment in children with severe hemophilia A. An open, randomized controlled trial was conducted on severe hemophilia A children aged 4?18 years in Pediatric Hematology-Oncology Division Dr. Cipto Mangunkusumo Hospital for 24 weeks. Eligible subjects were randomized into 2 groups: prophylaxis and on-demand group. All subjects were evaluated at week-0 and week-24 for inhibitor factor VIII (Bethesda Assay), ultrasonography (HEADUS scores) of six index joints (bilateral knees, ankles and elbows), HJHS (version 2.1, 2011) and urinary CTX-II (EIA). Subjects in prophylaxis group received factor VIII 10 IU/kgBW 2 times per week for 24 weeks. Any bleeding episodes in both groups were treated according to standard treatment (on-demand). During June 2015?February 2016 there were 50 subjects enrolled in the study. Mean age in prophylaxis group was 12 ± 3.5 years and median age in on-demand group was 11.9 (6.518.2) years. Mean frequency of joint bleeds in prophylaxis group was 5 ± 4.3 compare to 8 (3?30) in on-demand group (95%CI 0.9?6.99; p = 0.009). Mean difference of HJHS between two groups was not significant (95% CI -0.99?3; p = 0.320). HEAD-US scores and urinary CTX-II in prophylaxis group was significantly better compare to on-demand group (95%CI 2?8.81; p = 0.003 and 95%CI 2,777?16,742; p < 0.001 respectively). No subjects showed showed inhibitor factor VIII in both groups. We conclude that secondary low dose prophylaxis was effective to decrease joint bleeding episodes and improved HJHS scores, HEAD-US scores and urinary CTX-II, compared to on-demand treatment.

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ABSTRAK
Sepsis adalah disfungsi organ yang mengancam jiwa akibat gangguan regulasi pejamu sebagai respons terhadap infeksi. Renjatan sepsis adalah subset sepsis dengan abnormalitas sirkulasi, selular, dan metabolisme yang berkaitan dengan risiko kematian. Penelitian ini bertujuan untuk menilai peran ekokardiografi, biomarker kardiovaskular, fungsi ginjal dan saturasi oksigen vena sebagai prediktor kematian pasien renjatan sepsis. Pada pemeriksaan ekokardiografi dinilai fungsi diastolik E/e rsquo;, Fraksi Ejeksi Bilik Kiri, Indeks Kardiak, TAPSE, sedangkan biomarker kardiovaskular dinilai Troponin I dan NT Pro BNP, dengan disain penelitian kohort prospektif. Tempat penelitian di Rumah Sakit Umum Kabupaten Tangerang, Banten. Selama periode 2 tahun penelitian ada 111 pasien masuk dalam kriteria renjatan sepsis yaitu adanya infeksi, hipotensi MAP < 65 mmHg dan Laktat darah > 2 mmol/L. Pada hari pertama dan kelima dilakukan pemeriksaan ekokardiografi dan laboratorium darah pada semua pasien renjatan sepsis. Pada pengamatan selama 10 hari diperoleh pasien yang meninggal 64 58 dan yang hidup 47 42 . Rerata umur pasien 48 18 tahun. Analisis bivariat ditemukan Fraksi Ejeksi Bilik Kiri abnormal memiliki risiko kematian 1,6 kali dibanding normal RR 1,6; p = 0,034 . Biomarker Troponin I abnormal menunjukkan risiko kematian 1,6 kali dibanding normal RR 1,6; p = 0,004 . Pasien dengan gangguan fungsi ginjal memiliki risiko kematian 1,5 kali RR 1,5; p = 0,024 . Pasien dengan Troponin I abnormal dengan atau tanpa gangguan fungsi ginjal menunjukkan peningkatan risiko kematian, demikian pula pada pasien dengan Troponin I normal yang disertai gangguan fungsi ginjal. Hasil analisis multivariat menunjukkan prediktor kematian pasien renjatan sepsis adalah kadar Troponin I dan Fraksi Ejeksi Bilik Kiri RR 1,83; IK95 1,049 ? 3,215; p = 0,043 dan RR 1,99; IK95 1,009 ? 3,956; p = 0,047 Simpulan: Troponin I dan Fraksi Ejeksi Bilik Kiri merupakan prediktor kematian pasien renjatan sepsis. Kata kunci :Ekokardiografi, Kematian, NT Pro BNP, Renjatan Sepsis, Troponin I.

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ABSTRACT
Sepsis is a life threatening organ dysfunction caused by host regulation disorder in response to infections. Septic shock is a subset of sepsis with circulatory, cellular, and metabolic abnormalities associated with the risk of mortality. The aim of this study is to assess the role of echocardiography, cardiovascular biomarker, renal function and oxygen vein saturation as predictors of mortality in patients with septic shock. In this study, echocardiography examination including diastolic function E e 39 , Left Ventricle Ejection Fraction LVEF , Cardiac Index CI , and TAPSE, whereas cardiovascular biomarker Troponin I and NT Pro BNP were assessed. Research design of this study is cohort perspective. The study took place in Tangerang Regional General Hospital, Banten Province. During two years of research, there were 111 patients included in septic shock category, which indicated by the presence of infections, hypotension MAP 65 mmHg and serum lactate 2 mmol L. On the first and the fifth day, examinations on echocardiography and laboratory blood test were conducted on each patient of septic shock. During ten days of observation, 64 patients died 54 and 47 patients were survived 42 . The mean age of the patients was 48 18 years old. Bivariate analysis showed abnormal LVEF had 1.6 times higher mortality risk than normal RR 1.6 p 0.034 . Abnormal Troponin I biomarker showed 1.6 higher mortality risk, compared to normal RR 1.6 p 0.004 . The patients with kidney function disorder had 1.5 times higher mortality risk RR 1.5 p 0.024 . Patients with abnormal Troponin I with or without kidney function disorder showed increase in mortality risk. Normal Troponin I with kidney function disorder also increase in mortality risk. Multivariate analysis showed Troponin I and Left Ventricular Ejection Fraction as predictors of mortality in patients with septic shock RR 1.83 CI95 1.049 3.215 p 0.043 dan RR 1.99 CI95 1.009 3.956 p 0.047 In conclusion, Troponin I biomaker and Left Ventricular Ejection Fraction are predictors of mortality in patients with septic shock. Keyword Echocardiography, Death, NT Pro BNP, Septic Shock, Troponin I