Astigmatism and maternal myopia as important factors affecting success rate of DIMS lens treatment.

OBJECTIVE: To assess the efficacy of myopia control spectacle lenses (defocus incorporated multiple segments/DIMS) in slowing myopia progression among a diverse Central European paediatric population and investigate the contribution of baseline parameters on treatment outcomes. METHODS AND ANALYSIS: This retrospective observational study included 62 individuals aged 4-17 years (mean±SD: 10.21±2.70) with progressing myopia but without ocular pathology with a range of -0.88 to -8.25 D spherical equivalent refraction (SER) (-3.73±1.56), coupled with astigmatism up to -3.25 D cylindrical. All participants were prescribed DIMS (Hoya MiyoSmart) spectacles. Key outcome variables were cycloplegic SER, measured for all participants and axial length (AL), assessed in a subset of patients, recorded at baseline, 6 months and 12 months. Quality of life assessments were conducted at baseline, at 2 weeks, and 3, 6, 9 and 12 months. Additionally, parental myopic dioptre was recorded when applicable. RESULTS: At the 12-month mark, myopia progression in patients (mean±SE: -0.40±0.05) mirrored findings from prior European DIMS studies, but with 50% of patients showing no progression. A multivariate analysis of covariance model revealed that baseline astigmatism and younger age adversely affected therapy outcomes in both SER and AL, while severe maternal myopia led to greater SER progression. In contrast, only young age but not astigmatism was associated with AL increase in a comparable group of children with myopia, part of the LIFE Child Study, wearing single-vision spectacles. Patients reported consistent satisfaction with treatment, with minimal side effects, which diminished over the year. CONCLUSION: In the European population, astigmatism, young age and severe maternal myopia are risk factors for suboptimal outcomes following DIMS therapy. Further research is necessary to elucidate the impact of astigmatism on myopic defocus therapy.

[Management of Fabry disease]. / Fabry-betegség - terápiás útmutató

Fabry disease is a rare, X-linked lysosomal storage disorder that leads to accumulation of globotriaosylceramide in different tissues of the body. The disease is progressive and the first symptoms usually present in childhood. Consequences of the disease are disability and premature death. The disease in females could be as severe as in males although women may be asymptomatic. The possibility of enzyme replacement therapy has made it necessary to elaborate a comprehensive guideline for the diagnosis and treatment follow-up. The guideline has been summarized by a Hungarian multi-disciplinary working group consisting of physicians who are involved in diagnosis and care of Fabry patients. Previous clinical studies, published articles, and recently established international treatment guidelines were reviewed by the group.

[Fabry disease--diagnostic guideline]. / Fabry-betegség. Diagnosztikai útmutató.

Fabry disease is a rare, X-linked lysosomal storage disorder that leads to accumulation of globotriaosylceramide in different tissues of the body. The disease is progressive, first symptoms usually present in childhood. Consequencies of the diseases are disability and premature death. The disease in females could be as severe as in males although women may also be asymptomatic. The possibility of enzyme replacement therapy has made it necessary to elaborate a comprehensive guideline for the diagnosis and treatment follow-up. The guideline was established by a Hungarian multi-disciplinary working group, consisting of physicians who are involved in health care of Fabry patients. Previous clinical studies, published materials, and recently established international treatment guidelines were reviewed by the group.