Skripsi

Kejadian Perawakan Pendek dan Proporsi Tubuh pada Anak Thalassemia β Mayor dan β-HbE Usia Prepubertal = Short Stature and Body Proportion of Prepubertal β Major and β-HbE Children.

ndonesia tergolong dalam negara sabuk thalassemia yang memiliki prevalensi thalassemia yang tinggi. Thalassemia β mayor dan β-HbE merupakan dua jenis thalassemia yang paling banyak ditemukan di Indonesia. Studi-studi sebelumnya menemukan bahwa thalassemia menyebabkan gangguan pertumbuhan, di antaranya berupa perawakan pendek dan abnormalitas proporsi tubuh. Belum ada penelitian di Indonesia yang mencari perbedaan kejadian perawakan pendek dan proporsi tubuh antara anak thalassemia β mayor, β-HbE, dan normal usia prepubertal. Penelitian ini dilakukan dengan desain potong-lintang dengan menyertakan sampel berusia 5-12 tahun sebanyak 130 orang. Prevalensi perawakan pendek pada anak thalassemia β mayor ditemukan sebesar 34,0%, pada anak thalassemia β-HbE sebesar 24,3%, dan pada anak normal sebesar 9,4%. Tidak terdapat perbedaan rasio segmen atas terhadap segmen bawah pada anak thalassemia β mayor, β-HbE, dan kontrol. Rerata rasio rentang lengan terhadap tinggi badan lebih pendek pada anak thalassemia β mayor dan β-HbE dibanding pada anak normal. Jenis kelasi, usia diagnosis, durasi sakit, frekuensi transfusi, rerata Hb pretransfusi dalam 6 bulan terakhir, dan rerata ferritin dalam 6 bulan terakhir tidak berbeda antara anak thalassemia yang pendek dan yang tidak pendek.
Kata kunci: thalassemia β mayor, thalassemia β-HbE, perawakan pendek, proporsi tubuh


Indonesia belongs to thalassemia belt countries which has high prevalence of thalassemia. β major and β-HbE thalassemia are the two most common types of thalassemia found in Indonesia. Previous studies have found that thalassemia causes growth disorders, including short stature and abnormalities of body proportions. There have been no studies in Indonesia that looked for differences in the incidence of short stature and body proportion between β major and β-HbE thalassemia and normal children in prepubertal age. This research was conducted as cross-sectional study towards 130 children with age of 5-12. The prevalence of short stature was found to be 34.0% in β major thalassemia, 24.3% in β-HbE thalassemia, and 9.4% in normal children. There was no difference in upper segment to lower segment ratio between β major and β-HbE thalassemia and control. Arm range to stature ratio is shorter in β major and β-HbE thalassemia than control. Types of chelating agent, age of diagnosis, duration of sickness, frequency of transfusion, mean pretransfusion Hb in the last 6 months, and mean ferritin in the last 6 months did not differ between short and not short thalassemia children.
Keywords: β major thalassemia, β-HbE thalassemia, short stature, body proportion