Structural and functional changes over time in MacTel patients.

PURPOSE: To investigate functional and morphologic alterations over a 1-year review analysis in patients with type 2 idiopathic juxtafoveal retinal telangiectasia (MacTel). METHODS: Nine eyes of 9 patients with MacTel underwent repeated scotopic and photopic fine matrix mapping (FMM), 10-2 photopic microperimetry, and imaging studies. RESULTS: Early Treatment Diabetic Retinopathy Study visual acuity assessment showed a median difference between examinations of 1.0 letter (range, -3 to 4 letters). The difference of sensitivity values of all test points was 4.5 dB (range, 0.4 -5.5 dB) for microperimetry 1, 0.4 dB (range, -0.8 to 1.7 dB) for photopic, and -1.7 dB (range, -6.1 to 1.0 dB) for scotopic fine matrix mapping, respectively. The difference in test points of more than a 10-dB loss compared with age-matched controls was higher for scotopic than for photopic testing (P= 0.03, Wilcoxon signed ranks test). Small progression of scotoma correlated with a slight increase in retinal blood vessel dilatation and hyperfluorescence and subtle enlargement of pigmented plaques. CONCLUSION: Changes in central visual acuity and microperimetry testing after 1 year most likely do not extend beyond test-retest variability. The deterioration of scotopic sensitivity confirms our previous results of more severe rod compared with cone dysfunction in MacTel. Changes in fine detail visual function over a 1-year period may be useful parameters for interventional trials.

Endocrine complications in transfusion dependent thalassaemia in Penang Hospital.

Frequent blood transfusions can lead to iron overload which may result in several endocrine complications especially in the absence of adequate chelation therapy. The objectives of this study are to determine the prevalence of endocrine complications in transfusion dependent thalassaemia patients and the correlation of endocrine complications with the degree of iron chelation. This retrospective study looked at cases of adult patients with transfusion dependent thalassaemia treated in the Haematology Unit, Penang Hospital. Of the 25 transfusion dependent thalassaemia patients, there were 10 male and 15 female patients respectively with almost equal number of Malay and Chinese patients (13 and 12 patients respectively). Short stature was seen in 36.0% of our patients. In our cohort, 12 patients had delayed puberty (male 70.0% and female 33.3%). Prevalence of osteoporosis was 36.0%. Hypogonadism was noted in 40.0% of males and 46.7% of females. 53.4% of the female population had menstrual abnormalities with prevalence of primary and secondary amenorrhoea at 26.7% each. The prevalence of other endocrinopathies was much lower: 8.0% had diabetes mellitus and only one patient had hypocortisolism. Iron chelation appeared insufficient in our study population. The high frequency of endocrine complications noted in our study supports the rationale for regular follow-up of transfusion dependent thalassaemic patients to ensure early detection and timely treatment of associated complications.