ABSTRAK
Latar belakang: Thalassemia merupakan kelainan genetik terbanyak di dunia, termasuk Indonesia. Pasien thalassemia mayor berisiko mengalami gangguan fungsi neurokognitif akibat anemia kronik dan penumpukan besi. Tujuan: mengetahui prevalens abnormalitas hasil EEG dan tes IQ, menganalisis faktor-faktor yang diduga berhubungan dengan gangguan fungsi neurokognitif pada anak dengan thalassemia mayor usia saat diagnosis, lama transfusi, pendidikan pasien, rerata Hb pra-transfusi, kadar feritin serum, saturasi transferin, dan komplians terhadap obat kelasi besi , serta untuk mengetahui apakah gangguan neurokognitif dapat memengaruhi fungsi sekolah. Metode: Penelitian potong lintang deskriptif analitik antara April 2016-April 2017. Pengukuran tes IQ menggunakan WISC-III. Hasil: Total subyek adalah 70 anak thalassemia mayor berusia antara 9 hingga 15,5 tahun. Prevalens hasil EEG abnormal adalah 60 dan prevalens skor IQ abnormal

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ABSTRACT
Background Thalassemia is the most common hereditary disorders worldwide, including Indonesia. Chronic anemia and iron overload in thalassemia major lead to several risk factors including neurocognitive problems. Aim To investigate the prevalence of abnormal EEG and IQ test, to identify the factors related to neurocognitive function in children with thalassemia major age at diagnosis, years of transfusion, patients education, pre transfusion haemoglobin level, ferritin, transferrin saturation, and compliance to chelation , and to identify whether neurocognitive dysfunction affects child rsquo s school performance. Methods A cross sectional descriptive analitic study. Subjects were recruited from April 2016 April 2017. Cognitive function assessed by the WISC III. Results A total 70 children aged from 9 to 15.5 years old were recruited. The prevalence of abnormal EEG and abnormal IQ score