Contrast sensitivity in patients with Beta-thalassemia major and sickle cell disease under regular transfusions and treatment with desferrioxamine.

PURPOSE: Evaluation of contrast sensitivity in patients with beta-thalassemia major and sickle cell disease which undergo regular transfusions and chelation therapy with desferrioxamine (DFX). MATERIALS AND METHODS: We studied contrast sensitivity in 48 eyes (24 patients) with beta-thalassemia major (group A) and in 42 eyes (21 patients) with sickle cell disease (group B), compared to 60 eyes of 30 healthy individuals (control group). Contrast sensitivity was measured using B-VAT II-SG Mentor Video Aquity Tester. RESULTS: We found no statistically significant difference between group A and group B in any spatial frequency, while we found statistically significant differences between group B and control group in all spatial frequencies studied. Between group A and control group we found statistically significant differences in spatial frequencies of 2.3, and 6 cyc/deg. CONCLUSION: DFX causes retinal alterations that may explain reductions in contrast sensitivity function and justifies regular evaluations that may allow early detection of visual function deterioration.

Temporomandibular joint involvement in juvenile idiopathic arthritis: clinical predictors of magnetic resonance imaging signs.

The aim of the study was to define clinical predictors of magnetic resonance imaging (MRI) findings of temporomandibular joint (TMJ) involvement in juvenile idiopathic arthritis (JIA). Forty-six patients, aged 2.08-36.7 years, with JIA (oligoartitular 18, polyarticular 17, systemic type 11) were examined with standard plain and contrast-enhanced sequences. Of 88 TMJs examined, an abnormal condyle was observed in 32%, flattened articular eminence in 27%, flattened articular disk in 17%, intra-articular fluid in 10%, enhancing pannus in 45% and restricted condylar motion in 9%. Logistic regression analysis revealed that for abnormal condyle and flattened articular eminence, independent predictors were type of JIA (P < 0.015), age at onset (P < 0.038), and duration of disease activity (P < 0.001). Plots of the logistic regression models showed that TMJ involvement approached certainty for systemic sooner than for the other JIA types. Pannus was present with probability >0.5 when the disease started before 4 years of age. In conclusion, the systemic type of JIA, young age at onset and long duration of activity are risk factors for TMJ damage. MRI of the TMJ should be performed in patients who are less than 4 years of age at the onset of JIA, and in those with the systemic type, whatever the age of onset.