ABSTRACT
BACKGROUND: Ocular involvement in patients with transfusion-dependent ß-thalassemia is quite common, and its frequency differs among studies. This case series aimed to describe the ocular abnormalities occurring in ß-thalassemia patients who need regular blood transfusions and receive iron chelation therapy. CASE SERIES: This is a case series prospectively studied 32 ß-thalassemia patients from Northern Greece receiving regular blood transfusions and iron-chelating therapy. Patients' average age was 35.5 years. Eighteen patients with major phenotypes and fourteen patients with intermedia type underwent comprehensive ophthalmic examination at the time of enrolment, including visual acuity evaluation, refraction and color vision tests, Amsler grid test, slit-lamp, and dilated-pupil fundus examination. Additionally, we performed visual field testing and optical coherence tomography in all patients and fluorescein angiography only in selected cases. After six months, patients' complete ophthalmic examination was repeated for any new ocular findings due to the disease process and iron chelation therapy. Ocular involvement was detected in 46.87 % of the patients. Lesions were most frequently seen in elderly patients with thalassemia major. Lens opacities were present in 21.8 %, and degeneration of the retinal pigment epithelium was described in 15.6 % of the patients, representing the commonest fundus alteration observed, followed by fundus atrophy. The most severe and vision-threatening condition described in this study was the presence of angioid streaks with choroidal neovascularisation. Six months follow-up of patients did not reveal any new ocular findings. CONCLUSION: Early detection of severe ocular abnormalities is important in patients with thalassemia; thus, an ophthalmologic examination should be included at regular check-ups. An annual examination is currently indicated for asymptomatic patients, while in symptomatic and complicated cases, patients should be closely followed-up. HIPPOKRATIA 2021, 25 (2):79-82.
ABSTRACT
PURPOSE: To demonstrate the use of the multifocal electroretinogram (mfERG) in addition to the full-field electroretinogram (ERG) in defining varying clinical pictures in children within a family with Bardet-Biedl syndrome (BBS). METHODS: All members from a family generation underwent a detailed history and examination before proceeding to a detailed ERG in accordance with the International Society of Clinical Electrophysiology for Vision protocol and a rapid, low-resolution mfERG. Of the sibling pair, the 13-year-old boy showed reduced vision and atypical maculopathy and the 10-year-old sister showed normal vision and atrophic maculopathy. Parents had normal ocular examination. RESULTS: The male sibling had reduced rod and cone full-field ERG responses with a relatively spared central response from the mfERG suggesting central macular sparing. In contrast, for the female sibling, the ERG was normal for the cone pathway although reduced for rod pathway, with mfERG showing central involvement. The mother had rod responses at the lower end of normal range, a normal cone pathway, and a normal mfERG. The father showed a normal ERG and mfERG. CONCLUSION: The mfERG is a useful adjunct to full-field ERG in the paediatric population and in family studies.
Subject(s)
Bardet-Biedl Syndrome/diagnosis , Electroretinography , Photoreceptor Cells, Vertebrate/physiology , Retinal Dystrophies/diagnosis , Adolescent , Bardet-Biedl Syndrome/genetics , Bardet-Biedl Syndrome/physiopathology , Child , DNA Mutational Analysis , Female , Humans , Male , Microtubule-Associated Proteins/genetics , Mutation , Pedigree , Photic Stimulation , Retinal Dystrophies/genetics , Retinal Dystrophies/physiopathology , Siblings , Visual AcuityABSTRACT
PURPOSE: To assess the young people's knowledge regarding the ophthalmic effects of smoking. METHODS: 198 students (111 males and 87 females) with a mean age of 27 years old (+/- 6 years) participated in the study. A simple questionnaire was used and the participants were requested to fill out the questionnaire themselves. RESULTS: 77% (152) of the participants were smokers. 67% (130) have never heard about the adverse effect of smoking to the eyes. 87% (172) have not heard about the relationship between smoking and thyroid eye disease. 84% (166) were not aware that smoking could ccontribute to the formation of cataract. 50% (99) have no knowledge about the association between smoking and age-related macular degeneration. 80% (158) were unaware that smoking is a risk factor for visual loss. CONCLUSIONS: The level of knowledge of young people regarding the ophthalmic effects of smoking is not satisfactory. There is an urgent need to promote the awareness of the population regarding the adverse effect of smoking on visual function.
Subject(s)
Cataract/epidemiology , Health Knowledge, Attitudes, Practice , Macular Degeneration/epidemiology , Smoking/epidemiology , Adult , Cataract/etiology , Causality , Comorbidity , Eye Diseases/epidemiology , Eye Diseases/etiology , Female , Humans , Macular Degeneration/etiology , Male , Population Surveillance , Risk Factors , Smoking/adverse effects , Surveys and QuestionnairesABSTRACT
PURPOSE: To report a case of sympathetic ophthalmia (SO) following purulent postoperative endophthalmitis and final evisceration of the affected eye. METHODS-RESULTS: A 64-year-old male underwent phacoemulsification complicated by endophthalmitis. Five months latter the eye was painful and had no light perception so an evisceration was performed. Two weeks latter granulomatous posterior uveitis developed in the fellow eye. SO was diagnosed and the patient was started on prednisone and cyclosporine. The inflammation subsided and visual acuity improved to 20/30. CONCLUSIONS: Bacterial endophthalmitis cannot prevent the development of SO. Prompt diagnosis and management is the most important factor for visual prognosis.
