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"Latar belakang: Thalassemia adalah kelainan sel darah merah berupa gangguan sintesis hemoglobin (Hb) yang diturunkan secara autosomal resesif. Transfusi PRC pada pasien transfussion-dependent thalassemia (TDT) menyebabkan akumulasi besi pada berbagai organ, salah satunya kelenjar endokrin. Hal tersebut akan menyebabkan hemosiderosis di hipofisis anterior dan keterlambatan pubertas.Tujuan: Mendeteksi keterlambatan pubertas menggunakan MRI T2* hipofisis di poliklinik Thalassemia Departemen Ilmu Kesehatan Anak Rumah Sakit Cipto Mangunkusumo Jakarta.Metode: Pemeriksaan MRI dilakukan menggunakan MRI Avanto 1,5 Tesla, sekuen T2 SE. Penghitungan nilai MRI T2* hipofisis menggunakan perangkat lunak CMR Tools TM yang berfungsi menghitung deposit besi pada organ. Dilakukan pemeriksaan kadar feritin serum, FSH, LH testosteron (lelaki), dan estradiol (perempuan) menggunakan electro-chemiluminscence immunoassay (ECLIA). Hubungan antara MRI T2* hipofisis dengan kadar feritin serum, saturasi transferin, FSH, LH, testosteron, dan estradiol dianalisis menggunakan analisis korelasi Pearson.Hasil: Sebanyak 56 pasien TDT dibagi menjadi 27 subyek usia prapubertas, 14 subyek pubertas normal dan 15 subyek pubertas terlambat. Hasil menunjukkan nilai MRI T2* hipofisis pasien TDT pubertas normal lebih tinggi secara signifikan dibandingkan pubertas terlambat (p=0,000). Terdapat hubungan bermakna antara nilai MRI T2* hipofisis dengan feritin serum (r = -0,502) dan tidak ada hubungan antara MRI T2* hipofisis dengan saturasi transferin, FSH, LH, testosteron, dan estradiol.Simpulan: Nilai MRI T2* hipofisis TDT pubertas normal lebih tinggi dibandingkan pubertas terlambat.

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Background: Thalassemia is an autosomal recessive red blood cells disorder characterized by abnormal hemoglobin (Hb) synthesis. PRC transfusion to transfussion-dependent thalassemia (TDT) patients results in iron accumulation in organs, including endocrine system. It further cause hemosiderosis at anterior hypophysis which leads to delayed puberty.

Objective: To detect patients with delayed puberty using MRI T2* in Thalassemia Clinic Department of Pediatrics Cipto Mangunkusumo Hospital Jakarta.

Method: MRI was assesed with MRI Avanto 1,5 Tesla,T2 SE sequence, whilst T2* hypophysis result being done with CMR Tools TM software, which aims to measure iron deposit. Levels of ferritin serum, FSH, LH, testosterone (male subjects), and estradiol (female subjects) were observed with electro-chemiluminescence immunoassay (ECLIA). Correlation between MRI T2* hypophysis with levels of ferritin serum, transferrin saturation, FSH, LH, testosterone, and estradiol were analyzed with Pearson correlation analysis.

Results: 56 TDT patients were consist of 27 prepuberty subjects, 14 normal puberty subjects, and 15 delayed puberty subjects. Results showed that MRI T2* hypophysis of normal puberty TDT patients was significantly higher compared to delayed puberty patients (p = 0,000). There was significant correlation between MRI T2* hypophysis with ferritin serum (r = -0,502). Meanwhile, there was no significant correlation between MRI T2* hypophysis with transferrin saturation, FSH, LH, testosterone, and estradiol.

Conclusions: Pituitary MRI T2* of TDT patients higher in normal group than that in delayed puberty group."

"Latar Belakang: Di Indonesia diperkirakan terdapat 4100 kasus baru kanker anak setiap tahunnya. Kejadian paling banyak adalah leukemia limfoblastik akut LLA sebesar 80 . Permasalahannya adalah angka kejadian kekambuhan pasien anak cukup tinggi sekitar 20-40 . Data pasien anak di Bagian Hematologi/Onkologi Departemen Ilmu Kesehatan Anak RSCM/FKUI periode tahun 2005-2011 kejadian kambuh mencapai 40 . Tujuan penelitian ini adalah mengetahui apakah ada hubungan kekambuhan pasien anak LLA terkait terapi vinkristin dengan polimorfisme gen MDR1.Metode: Studi dilakukan di Departemen Ilmu Kesehatan Anak RSCM/FKUI selama periode Februari 2014 sampai Januari 2015 dengan disain penelitian cohort. Kriteria pasien dengan LLA berusia lebih dari satu tahun hingga usia kurang dari 18 tahun. Sampel adalah pasien baru didiagnosis LLA dan pasien sedang menjalani kemoterapi fase induksi. Pemeriksaan sensitifitas obat in vitro dari sampel darah metode flow cytometry dengan menghitung Leukemia Cell Survival Index LCSI . Penelitian pendahuluan gen MDR1 dengan RFLP dan polimorfisme dengan Multiplex PCR. Data klinis pasien dilihat secara retrospektif dari catatan medis. Inform consent diperoleh dari pasien atau orang tua pasien.Hasil: Sampel memenuhi kriteria inklusi sebanyak 101 pasien terdiri dari 63 62.4 laki-laki dan 38 37.6 perempuan. Kekambuhan pasien anak LLA dipengaruhi oleh faktor usia,kelompok risiko dan jumlah lekosit p

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Background There are an estimated 4100 new cases of childhood cancer each year in Indonesia. The most common cancer in children is acute lymphoblastic leukemia ALL which is approximately 80 . The problem faced is the incidence of relapse in children is quite high, about 20 40 . Data from Hematology Oncology Division of Child Health Department RSCM FKUI period 2005 2011, showed that the incidence of relapse reached 40 . The purpose of this study is to investigate whether there is an association of polymorphism related to vincristine therapy.Methods The study was conducted in the Child Health Department RSCM FKUI with cohort design from February 2014 to January 2015. The number of patients with ALL included in the study was 101. The patients participated in the study were 1 18 years old, newly diagnosed of ALL, and undergoing chemotherapy induction phase. In vitro examination of drug sensitivity from blood samples was conducted using flow cytometry to determine leukemia cell survival index LCSI . The preliminary study was done by RFLP and polymorphism by Multiplex PCR. Clinical data of patients were obtained retrospectively from medical records.The informed consent was obtained from the patient or the patient 39 s parents.Results From 101 patients, there were 63 male 62.4 and 38 female 37.6 . The occurence of relapse of ALL patients was affected by age, risk group and leukocyte count p"

"ABSTRAK Latar belakang: Minimal residual disease MRD adalah faktor prediktor yang sensitif pada leukemia limfoblastik akut LLA dengan menggunakan flowsitometri. Minimal residual disease dapat mendeteksi 1 sel blas diantara 10.000 sel normal 0,01 . Pemerikasaan MRD dapat digunakan untuk menyempurnakan status remisi induksi pada LLA. Metode: Penelitian uji potong lintang selama 4 bulan dilakukan di bagian Onkologi Anak RSKD, Departemen Ilmu Kesehatan Anak RSAB Harapan Kita; divisi Hematologi-Onkologi RS Kramat 128 pada Febuari ndash; Juni 2017. Subjek adalah pasien yang terdiagnosis LLA yang menyelesaikan kemoterapi pasca fase induksi. Pemeriksaan morfologi sumsum tulang, imunofenotiping leukemia dan MRD untuk evaluasi pasca fase induksi dilakukan dilakukukan di bagian Patologi Klinik RSKD.Hasil penelitian: Pada penelitian ini diikutsertakan 52 subjek dengan usia rerata 6.4 tahun. Subjek lelski 62 lebih banyak dibanding perempuan 38 . Semua pasien dengan leukemia sel B. Stratifikasi Risiko Biasa RB 46 adalah lebih sedikit dibandingkan Risiko Tinggi RT 54 . Minimal residual disease 0,01 42,3 dengan morfologi yang juga remisi. Stratifikasi RB dengan pemeriksaan MRD kuantitatif ABSTRACT Introduction Minimal residual disease MRD is the most powerful predictor of outcome in acute leukemia and is useful in therapeutic stratification for acute lymphoblastic leukemia ALL protocols. Nowadays, the most reliable methods for studying MRD in ALL are multiparametric flow cytometry. It provides a MRD level of 0,01 of normal cells, that is, detection of one leukemic cell in up to 10.000 normal nucleated cells. Evaluation after induction phase, is the most informative time to predict danger of relapse.Methods A cross sectional study was conducted at Pediatric Hematology Oncology Division, Department of Child Health, Pediatric Oncology Division of ldquo Dharmais rdquo Cancer Hospital Women and Children Harapan Kita Hospital Pediatric Hematology Oncology Division Kramat 128 Hospital on February June 2017. Morphology, immunophenotyping, MRD assessment was performed. Bone marrow aspiration and MRD detection performed after induction phase to evaluate remission.Results A total of 52 diagnosed ALL patients enrolled in this study. The mean age was 6.4 years. Incidence in male 62 is higher than female 38 . All patients are B lineage. Standard risk SR patients 46 is less than high risk HR patients 54 . Minimal residual disease 0,01 1 42,3 and morphological remission. Standard risk stratification with quantitative minimal residual disease "