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The Indian Journal of Pediatrics

, Volume 84, Issue 7, pp 509–514 | Cite as

Ophthalmic Evaluation in Beta-Thalassemia

  • Rashid H. Merchant
  • Hrishikesh Punde
  • Neepa Thacker
  • Deepak Bhatt
Original Article

Abstract

Objectives

To determine the association of ocular manifestations in beta-thalassemia with the patient’s age, blood transfusion requirements, average serum ferritin and dose and duration of iron chelation therapy.

Methods

Sixty multi-transfused beta thalassemia patients of 12 to 18 y of age on chelation therapy were included in this cross-sectional analysis. Structural and functional evaluation of the retina was done using Optical coherence tomography (OCT) and Electroretinography (ERG), including flash ERG and Pattern ERG (PERG). Routine ophthalmic examination and B scan of the eye was also done. Flash ERG a-waves and b-waves were recorded, however only a-wave amplitude was evaluated. Pattern ERG n35, n95 and p50 waves were recorded and p50 wave amplitude was evaluated. The a-wave on flash and p50 on pattern waves represent retinal photoreceptor epithelium (RPE) photoreceptor response, which is mainly affected in beta-thalassemia.

Results

Ocular changes were detected in 38.3% and a significant correlation was noted with increase in age (p = 0.045) but not with serum ferritin, transfusion requirements or chelation therapy. Refractive errors were found in 14 cases (23%), such as myopia with astigmatism in 13 (21.7%) and only myopia in 6 subjects (10%). OCT abnormality was noted in 1 patient (1.7%) who had thinning of central retina; right eye 132 μm and left eye 146 μm (n > 200 μm). Abnormalities were noted in a-wave amplitude on flash ERG in 20% of cases, while reduced p50 amplitude on PERG was noted in 15%.

Conclusions

A significant correlation was noted between ocular findings and increase in age, but not with serum ferritin, transfusion requirements or chelation therapy. ERG appears to be a promising tool for screening patients with beta-thalassemia and can serve as a follow-up test for evaluating retinal function.

Keywords

Beta-thalassemia Electroretinography Optical coherence tomography 

Notes

Acknowledgements

The authors would like to thank the children with thalassemia who shared their information.

Contributions

HP: Collected data and drafted the manuscript; RHM: Revised and prepared the manuscript, supervised the study and will act as guarantor of the study; NT and DB: Ophthalmic evaluation.

Compliance with Ethical Standards

Conflict of Interest

None.

Source of Funding

None.

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Copyright information

© Dr. K C Chaudhuri Foundation 2017

Authors and Affiliations

  • Rashid H. Merchant
    • 1
  • Hrishikesh Punde
    • 1
  • Neepa Thacker
    • 2
  • Deepak Bhatt
    • 3
  1. 1.Department of PediatricsNanavati Super-Speciality HospitalMaharashtraIndia
  2. 2.Department of OphthalmologyNanavati Super-Speciality HospitalMumbaiIndia
  3. 3.U B M InstituteMumbaiIndia

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