Abstract Ref Number = APCP903
Poster Presentation
Bidasari Lubis,Siska Mayasari Lubis Pediatric Hematology Oncology Division Child Health Department Faculty of Medicine University of Sumatera Utara Haji Adam Malik General Hospital Medan Indonesia Pediatric Endocrinology Division, Child Health Department, Faculty of Medicine, University of Sumatera Utara, H Adam Malik General Hospital, Medan, Indonesia
Background : Beta-thalassemia is a severe genetic blood disorder caused by a mutation in the gene encoding for the beta chains of hemoglobin. Individuals with beta-thalassemia major require regular lifelong red blood cell transfusions to survive. Multiple/repeated blood transfusions lead to haemosiderosis. Adverse ocular changes may occur as a result of the disease itself or as side-effects of iron chelators and include cataract, optic neuropathy, retinal pigment epithelial (RPE) degeneration, RPE mottling, retinal venous tortuosity, vitreoretinal hemorrhages and obliteration of iris pattern. Ocular involvement is quite common and may have serious implications Material : A cross-sectional study was conducted in a 2 general hospitals in Medan, Sumatera Utara, Indonesia, from March to May 2018. Subjects were children aged below 18 year-old with beta thalassemia major. This study included a questionnaire, anthropometric measurements, and blood test analysis. Serum ferritin level was performed. Ocular status was documented, subjects were observed for changes in ocular status arising due to the disease process and due to iron chelation therapy.Ocular examinations including visual acuity, slit lamp bio microscopy, direct & indirect Opthalmoscopy. Data analysis was done using Mann Whitney test, p<0.05 was considered as statistically significant. Results : There were 37 children recruited, females and males comprised 19 (48.7%) and 20 (51.3%) respectively. The mean age and ferritin were 2 (1-15) year-old and 2842.0 (430.8-31285.4) ng/mL, respectively. Age at diagnosis was 31.0 (3.0-180) months. 2842.0 (430.8-31285.4). In this study we did not find significant association between serum ferritin with ocular abnormalities in our subjects (p<0.05). However, our study found some ocular abnormalities, we found hyperpigmentation on conjunctival bulbi in 32.4% subjects, hyperpigmentation on anterior chamber in 18.9% subjects, abnormal visual acuity was also found in 20.5% subjects. Papilledema, cataract, and papil atrophy papil were found in 27%, 8.1%, and 5.4%, respectively. Conclusions : Most of the ocular abnormalities in our subjects are attributed to the course and severity of the disease. Although we did not find significant association between serum ferritin and ocular abnormalities, reduction in serum ferritin levels by iron chelating agents and regular ocular examination to look for side-effects of such agents can aid in preventing or delaying ocular complications.
Keywords: Thalassemia Ocular abnormalities Iron Overload
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