Vernal keratoconjunctivitis is a severe ocular condition that mainly affects young males. Vernal keratoconjunctivitis is characterized by different clinical features and therapeutic responses, suggesting the need for a standardized therapeutic approach on the basis of a grading of disease severity.
Patients deafened by Cogan syndrome demonstrated high levels of speech understanding after undergoing cochlear implantation. Obliteration of the cochlea may complicate electrode implantation, requiring modifications of the surgical technique. Stress consequent to the surgical procedure may instigate an acute phase of the basic illness.
The present study confirms the value of the anti-hsp70 test in the serological diagnosis of autoimmune hearing loss. It is still the only available diagnostic marker that identifies an autoimmune origin of hearing loss.
Neuro-Behçet’s disease (NBD) involves the central nervous system; peripheral nervous system involvement is not often reported. NBD is quite common in adult patients and occurs rarely during childhood and adolescence. Young patients may share symptoms and signs of NBD with other neuro-ophthalmological disorders (e.g. idiopathic intracranial hypertension); thus, making the differential diagnosis difficult. Neuroimaging is mandatory and necessary for a correct NBD diagnosis but in children radiological examinations are often difficult to perform without sedation. From 1971 to 2011, 130 patients aged ≤16 years have been reported with NBD, according to retrospective surveys, case series, and case reports. The origin of the reported cases met the well-known geographical distribution of Behçet’s disease (BD); the mean age at presentation of neurological findings was 11.8 years, with male gender prevalence (ratio, 2.9:1). We considered in detail the neuro-ophthalmological features of the 53 cases whose neuroimaging alterations were described with an assigned radiological pattern of the disease (parenchymal: 14 cases, non-parechymal: 35 cases, and mixed: 4 cases). In 19/53 patients (36%), neuro-ophthalmological symptoms anticipated any pathognomonic sign for a BD diagnosis, or only occasional aphtae were recalled by the patients. Family history was positive in 17% of subjects. Headache was reported in 75% of the patients; in those presenting with cerebral vascular involvement, headache was combined to other symptoms of intracranial hypertension. Papilledema was the most frequently reported ophthalmological finding, followed by posterior uveitis. Treatment consisted of systemic steroids in 93% of patients, often combined with other immunosuppressive drugs (especially colchicine and azathioprine). Clinical recovery or improvement was documented in the large majority of patients. Nine subjects had definitive alterations, and one died. Based on our review and personal experience, a delayed diagnosis, and the consequently delayed immunosuppressive treatment, may favour permanent sequelae, in particular, optic atrophy.
ABSTRACT.Purpose: The aim of this study was to assess the feasibility of a visual acuity (VA) test using the Lea Symbols 15-line folding distance chart and its diagnostic validity in detecting VA deficiency in preschool children. Methods: A group of 149 children aged 38-54 months underwent VA examination performed with the Lea 15-line folding optotype at a distance of 3 metres, according to a test protocol described in the Methods section. After the VA test, a complete ophthalmological examination, including cycloplegic retinoscopy, a cover test and examination of the anterior and posterior segments, was performed on each child in order to detect any VA-threatening ocular abnormality. The Lea Symbols test's sensitivity, specificity, positive and negative likelihood ratios (LR +, LR )) and the receiver operating characteristic (ROC) curve were calculated by means of standard procedures using each VA level of the chart from 0.1 to 1 (1-0 logMAR) as a cut-off point. Results: The Lea Symbols test could be successfully used in 95.9% of the population. The most useful cut-off points for screening preschool children were found to be 0.8 (LR + 5.73, LR ) 0.05) or 0.63 (LR + 11.7, LR ) 0.23).
Conclusion:The Lea Symbols test proved to be clinically useful in detecting VA deficiency in preschool children. The choice between the two best performing cut-off levels should be made according to the expected cost-effectiveness of the screening programme.
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