Klinische Monatsblatter fur Augenheilkunde最新文献

{"title":"Standardized Assessment of Health-Related Quality of Life in Patients with Congenital Aniridia.","authors":"Zamira Hoxha, Fabian Norbert Fries, Dietmar Hecker, Berthold Seitz, Barbara Käsmann-Kellner, Annamária Náray, Neil Lagali, Christina Grupcheva, Nóra Szentmáry, Tanja Stachon","doi":"10.1055/a-2463-3879","DOIUrl":"10.1055/a-2463-3879","url":null,"abstract":"<p><strong>Introduction: </strong>Congenital aniridia is a rare panocular disorder that is associated with varying degrees of impairment of visual acuity. The COST Action (CA18116) developed a survey (aniridia-net.eu) to assess patient-reported experiences with congenital aniridia and its impacts on vision and daily life. Here, we correlate the survey responses of German patients with congenital aniridia with clinical ophthalmology data acquired at the Homburger Aniridia Center.</p><p><strong>Patients and methods: </strong>The patients completed the German-language version of a 20-point ANIRIDIA-NET survey. The survey included demographic information, the most common symptoms caused by the disease, difficulties caused by visual impairment in various life situations, and the frequency of using visual aids in daily life. As for clinical data, best-corrected visual acuity (BCVA) as well as corneal, lens, and glaucoma status were collected.</p><p><strong>Results: </strong>A total of 71 participants, 27 (38.0%) children and 44 (61.7%) adults, completed the questionnaire, with an age range of 28.8 ± 20.2 years (6 - 78 years). Among them, 55 (77.4%) reported daily light sensitivity, 34 (47.8%) experienced dry eyes, 17 (23.9%) had fluctuating vision, 11 (15.4%) reported eye pain, and 5 (7.0%) experienced daily watering eyes. Older patients reported significantly more eye complaints than children (p < 0,001). Notably, patients with more advanced aniridia-associated keratopathy (AAK) exhibited a discernibly lower quality of life (ρ = 0.28, p = 0.027). Similarly, cataract surgery early in life was associated with a more pronounced decline in quality of life (ρ = - 0.36, p = 0.002). Thirty-five (49.2%) patients never needed assistance for their commute to school/work, 27 (38.0%) and 22 (30.9%) never needed assistance for their daily routines at home or various social activities, respectively. Regarding the use of visual aids, 39 (24.9%) reported that they always used visual aids at work or school, 24 (33.8%) during social activities, and 32 (45.1%) during free time activities.</p><p><strong>Conclusions: </strong>Although congenital aniridia is associated with reduced visual acuity, the majority of affected individuals, especially during childhood, report that they were able to manage personal communication and various life situations independently and without significant difficulties, despite their eye-related issues. Visual aids serve as crucial support for them during their transition into adulthood and as they age. Symptoms of congenital aniridia subjects, described by the ANIRIDIA-NET survey, correlated well with clinical findings. Therefore, the questionnaire may provide important information for the treating ophthalmologist for follow-up examination of these patients and improvement in their life quality.</p>","PeriodicalId":17904,"journal":{"name":"Klinische Monatsblatter fur Augenheilkunde","volume":" ","pages":"228-239"},"PeriodicalIF":0.8,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142854660","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Die neue Generation der Anti-VEGF-Wirkstoffe: Sind sie wirklich besser?","authors":"Horst Helbig","doi":"10.1055/a-2370-7414","DOIUrl":"10.1055/a-2370-7414","url":null,"abstract":"","PeriodicalId":17904,"journal":{"name":"Klinische Monatsblatter fur Augenheilkunde","volume":" ","pages":"196-198"},"PeriodicalIF":0.8,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143007956","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Acceptance of Novel Perimetry Methods in Germany: Virtual Reality Perimetry and Tablet-based Perimetry in Glaucoma Patients.","authors":"Silvia Schrittenlocher, Vincent Lüke, Hanne Irle, Jithmi Weliwitage, Jan Niklas Lüke, George Kong, Algis J Vingrys, Alexandra Lappas, Claus Cursiefen, Thomas S Dietlein","doi":"10.1055/a-2511-4087","DOIUrl":"10.1055/a-2511-4087","url":null,"abstract":"<p><strong>Purpose: </strong>The aim of this study was to investigate the acceptance of innovative perimetry methods such as virtual reality perimetry (VRP) and tablet-based perimetry (TBP) in comparison to conventional hemisphere perimetry (HP) in glaucoma patients for the first time in Germany.</p><p><strong>Patients and methods: </strong>We examined 204 glaucoma patients using the 30 - 2 TOP algorithm (Octopus, Haag-Streit) followed by one of the two new methods: Group 1 (VRP; n = 101) and Group 2 (TBP using the Melbourne Rapid Fields Glaucoma App; n = 103). Subsequently, patients were asked to fill out a questionnaire on their experience with the new examination compared to the conventional examination.</p><p><strong>Results: </strong>More than three-quarters of patients at 77% in Group 1 (VR) and 89.9% of patients in Group 2 (TBP) found the respective new examination method \"easier\" or \"much easier\" than the conventional HP. An overwhelming majority of patients in Group 1 and Group 2 found the new method more comfortable at 86% and 90.9%, respectively. Examination durations were: 2.67 ± 0.98 min for HP, 6.26 ± 1.88 min for VR perimetry, and 4 ± 0.71 min for the tablet method; figures given as means ± standard deviation. Just over half the patients at 51% found HP and VR headset to be similar for duration with 54% preferring the tablet over conventional hemisphere perimetry. By far the most patients in Groups 1 and 2 at 80% and 81.8%, respectively, reported willingness to use the new perimetry method regularly in future follow-up examinations.</p><p><strong>Conclusion: </strong>Most glaucoma patients surveyed consistently responded favourably to VR headset and tablet-based perimetry, preferring these examinations to conventional hemispherical perimetry.</p>","PeriodicalId":17904,"journal":{"name":"Klinische Monatsblatter fur Augenheilkunde","volume":" ","pages":"219-227"},"PeriodicalIF":0.8,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142950840","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Die klinische Kontroverse: Pro oder Kontra?","authors":"Lars-Olof Hattenbach, Siegfried Priglinger","doi":"10.1055/a-2370-7363","DOIUrl":"https://doi.org/10.1055/a-2370-7363","url":null,"abstract":"","PeriodicalId":17904,"journal":{"name":"Klinische Monatsblatter fur Augenheilkunde","volume":"242 3","pages":"191-192"},"PeriodicalIF":0.8,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143700715","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Die neuen ANTI-VEGF Präparate: Evolution oder Revolution?","authors":"Ramin Khoramnia, Lucy J Kessler, Gerd U Auffarth, Michael Albrecht","doi":"10.1055/a-2370-7389","DOIUrl":"10.1055/a-2370-7389","url":null,"abstract":"","PeriodicalId":17904,"journal":{"name":"Klinische Monatsblatter fur Augenheilkunde","volume":" ","pages":"193-195"},"PeriodicalIF":0.8,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143256137","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Pupillary Dysfunction, Anisocoria and Iris Depigmentation Following COVID-19].","authors":"Daniela Claessens, Klaudia Huber-van der Velden, Ronald Krüger","doi":"10.1055/a-2118-0290","DOIUrl":"10.1055/a-2118-0290","url":null,"abstract":"","PeriodicalId":17904,"journal":{"name":"Klinische Monatsblatter fur Augenheilkunde","volume":" ","pages":"250-252"},"PeriodicalIF":0.8,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10004969","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[N-Acetylcysteine (NAC) for Retinitis pigmentosa].","authors":"Kristina Pfau, Josep Callizo, Petra Rossouw, Chrysoula Gabrani, Frank Holz, Peter Charbel Issa, Ulrich Kellner, Rupert Strauss, Laura Kühlewein, Katarina Stingl, Nicolas Feltgen, Maximilian Pfau","doi":"10.1055/a-2525-4419","DOIUrl":"10.1055/a-2525-4419","url":null,"abstract":"<p><p>Retinitis pigmentosa (RP) is a genetically mediated degenerative retinal disease that leads to progressive photoreceptor degeneration and, ultimately, blindness. Oxidative stress plays a central role in the pathogenesis of RP. This study examines the effect of N-acetylcysteine (NAC), an antioxidant and glutathione precursor, on the disease progression in RP patients. NAC can reduce oxidative stress in the retina and has shown potential neuroprotective effects in preclinical models. Initial clinical studies indicate improvements in visual acuity and macular function with NAC therapy, though no structural changes have yet been demonstrated. The ongoing multicentre Phase III trial \"NAC Attack\" aims to evaluate the long-term efficacy and safety of NAC in RP.</p>","PeriodicalId":17904,"journal":{"name":"Klinische Monatsblatter fur Augenheilkunde","volume":" ","pages":"199-204"},"PeriodicalIF":0.8,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143047127","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Pharmakotherapie von hereditären Netzhautdystrophien.","authors":"Claudia Priglinger, Katarina Stingl","doi":"10.1055/a-2511-6878","DOIUrl":"https://doi.org/10.1055/a-2511-6878","url":null,"abstract":"","PeriodicalId":17904,"journal":{"name":"Klinische Monatsblatter fur Augenheilkunde","volume":"242 3","pages":"189-190"},"PeriodicalIF":0.8,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143700746","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"IgG4-Related Ophthalmic Disease following COVID-19 Infection: Is it Associated or Incidental?","authors":"Busra Coban, Asli Kirmaci Kabakci, Ilknur Mansuroglu, Arzu Taskiran Comez","doi":"10.1055/a-2102-9109","DOIUrl":"10.1055/a-2102-9109","url":null,"abstract":"","PeriodicalId":17904,"journal":{"name":"Klinische Monatsblatter fur Augenheilkunde","volume":" ","pages":"245-249"},"PeriodicalIF":0.8,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10160769","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Enzyme Replacement Therapy in CLN2-Associated Retinopathy.","authors":"Claudia Priglinger, Carolina Courage, Esther M Maier","doi":"10.1055/a-2528-7886","DOIUrl":"https://doi.org/10.1055/a-2528-7886","url":null,"abstract":"<p><p>Neuronal ceroid lipofuscinoses, also known as Batten disease, are comprised of a group of genetically heterogenous neurodegenerative conditions, characterized by dementia, epilepsy, motor deterioration, and blindness. The underlying pathology is a dysregulation of lysosomal catabolic protein metabolism, resulting in an accumulation of lipofuscein-like material within the lysosomes in neuronal tissue, which ultimately leads to atrophy in the central nervous system and in the retina. Ceroid lipofuscinosis type 2 (CLN2) is caused by biallelic pathogenic variants in the <i>TPP1</i> gene, encoding lysosomal tripeptidyl peptidase 1 (TPP-1). The classic late-infantile phenotype of CLN2 disease has an age of onset between 2 and 4 years and manifests with seizures and speech delay, followed by progressive cognitive and motor decline, vision loss, and early death. Vision loss occurs secondary to retinal degeneration and begins in the perifoveal ellipsoid zone, leading to bull's eye maculopathy, followed by generalized retinal thinning. In 2017, an intracerebroventricular enzyme replacement therapy (ERT) using recombinant human TPP1 (rhTPP1), cerliponase alfa, was approved as a disease-modifying treatment for CLN2 disease. The therapy slows psychomotor decline but fails to prevent loss of vision. In a canine model of CLN2 disease, intravitreal rhTPP1 was shown to halt retinal degeneration. A prospective, interventional, controlled, open-label compassionate-use study evidenced safety of 0.2 mg intravitreal rhTPP1 every 8 weeks in humans and its efficacy in reducing the rate of macular volume loss in patients who were still in the degenerative phase. One ongoing clinical phase I/II study is investigating the safety and efficacy of intravitreal rhTPP1 at 4 weekly intervals over 24 months (NCT05152914); another clinical phase II dose escalation trial is planned. In this review, we summarize the current knowledge on ERT for CLN2 retinopathy, complemented with our own experience from an individual treatment. The treatment now appears to be safe and markedly delays retinal degeneration, thereby preserving visual function and increasing the quality of life of the patient. This could be particularly relevant for those patients who were started on intracerebroventricular ERT early and still have good motor and language function. For this patient population, intravitreal ERT could be a valuable bridging therapy until other therapies such as gene therapy become available.</p>","PeriodicalId":17904,"journal":{"name":"Klinische Monatsblatter fur Augenheilkunde","volume":"242 3","pages":"213-218"},"PeriodicalIF":0.8,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143700726","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}