Tesis

Korelasi antara muatan besi berlebih dengan fungsi endokrin pada pasien dewasa thalassemia beta bergantung transfusi yang mengalami retardasi pertumbuhan = The correlation between iron overload and endocrine function in adult transfusion dependent beta thalassemia patients with growth retardation.

Latar Belakang: Muatan besi berlebih merupakan masalah utama pada pasien thalassemia bergantung transfusi beta karena menyebabkan toksisitas pada jaringan atau organ. Laporan mengenai korelasi antara muatan besi berlebih dengan fungsi endokrin pada pasien dewasa TDT beta yang mengalami retardasi pertumbuhan di Indonesia belum pernah dilaporkan. Tujuan: Penelitian ini bertujuan mendapatkan profil muatan besi dengan fungsi endokrin pada pasien dewasa TDT beta yang mengalami retardasi pertumbuhan. Metode: Dilakukan studi potong lintang pada pasien thalassemia beta mayor homozigot dan beta HbE usia dewasa yang mendapatkan transfusi darah di Poliklinik Hematologi-Onkologi Medik IPD dan Poliklinik Thalassemia Kiara RSCM Jakarta, mulai dari bulan November sampai Desember 2017. Muatan besi berlebih diwakili oleh feritin serum (FS) dan saturasi transferin (ST), dan fungsi endokrin yang diperiksa adalah hormon TSH, fT4, dan IGF-1. Feritin serum, fT4 dan TSHs diperiksa dengan metode electrochemiluminescence immunoassay, sedangkan saturasi transferin dihitung dari kadar besi serum dibagi total iron binding capacity dikali 100%. Kadar IGF-1 diperiksa berdasarkan metode SolidPhase Enzyme-Labeled Chemiluminescent Immunoassay (Solid-Phase ECLIA). Analisa data menggunakan Analisa Bivariat dan uji korelasi Pearson serta Spearman. Hasil: Penelitian mendapatkan 58 subjek penelitian dari 224 pasien TDT dewasa, yang terdiri dari 31 subjek laki-laki (53,4%) dan 27 subjek perempuan (46,6%). Median usia pasien adalah 21 (18-24) tahun. Proporsi hipotiroid subklinis sebesar 32,7% dan kadar IGF-1 rendah ditemukan pada 79,3% subjek penelitian. Didapat juga proporsi MRI T2* pankreas dengan nilai rendah sebanyak 87,5 %. Terdapat korelasi negatif lemah antara FS dengan fT4 ( r = -0,361; p=0,003), dan IGF-1( r=-0,313; p=0,008), namun tidak terdapat korelasi antara FS dengan kadar TSHs (r=0,074; p=0,29). Tidak terdapat korelasi antara ST dengan TSHs (r =0,003; p=0,492), fT4 (r=0,018; p=0,448), dan IGF-1(r=-0,142; p=0,143). Simpulan: Terdapat korelasi negatif antara muatan besi berlebih yang dinilai dari feritin serum dengan fungsi endokrin yang dinilai dengan fT4 dan IGF-1.
Kata kunci: IGF-1, TSHs, fT4, Feritin Serum, Saturasi Transferin, Thalassemia bergantung transfusi


Background: Iron overload is a major problem in patients with transfusion dependent beta- thalassemia, because it causes toxicity to tissues or organs. Reports on the correlation between iron overload and endocrine function with growth retardation in adult TDT beta patients in Indonesia have not been reported. Objective: This study aims to obtain a profile of iron load and endocrine function of adult TDT beta patients and their correlation with growth retardation. Methods: Cross sectional studies were performed on beta-homozygous beta and adult HbE beta patients receiving blood transfusions at the Hematology Medical Oncology Clinic and Thalassemia Kiara RSCM Jakarta Clinic, December 2017. Iron overload was represented by serum ferritin ( FS) and transferrin saturation (ST), and the endocrine function examined is the hormone TSHs, fT4, and IGF-1. Serum ferritin, fT4 and TSHs were examined by electrochemiluminescence immunoassay method, while transferin saturation was calculated from serum iron content divided by total iron binding capacity multiplied by 100%. IGF-1 levels were examined according to the Solid-Phase Enzyme-Labeled Chemiluminescent Immunoassay (Solid-Phase ECLIA) method. Data analysis using Bivariate Analysis and Pearson and Spearman correlation test. Results: During the study period, 58 study subjects were obtained from 224 adult TDT patients, consisting of 31 male subjects (53.4%) and 27 female subjects (46.6%). The median age of the patient was 21 (18-24) years.Subclinical hypothyroid proportion was 32,7% and low IGF-1 level was found in 79.3% of subjects. Low Pancreatic MRI T2* proportion was found in 87.5% There is a weak negative correlation between FS and fT4 (r = -0.361; p = 0.003), and IGF-1 (r = -0.313; p = 0.008), but there was no correlation between FS and TSHs (r = 0.074 ; p = 0.29). No correlation was found between ST with TSHs (r= 0,003; p = 0,492), fT4 (r= 0,018; p = 0,448), and IGF-1 (r = -0,142; p = 0,143). Conclusion: There was negative correlation between iron overload based on serum ferritin with endocrine function based on fT4 and IGF-1.
Keywords: IGF-1, TSHs, fT4, Ferritin Serum, Transferrin Saturation, Transfusion Dependent Thalassemia