Abstract:
PURPOSE:The first Oskar Fehr lecture is given in honour of Professor Fehr, a well respected ophthalmologist, who was head physician of the Department of Eye Diseases at the Rudolf Virchow Hospital from 1918. He practiced there until 1938, when he was forbidden to enter the clinic because he was Jewish and subject to the anti-Semitic laws that were instituted after the rise of the Nazi party. Dr. Fehr escaped to Great Britain, where he practiced ophthalmology into his eighties. He was the first to distinguish between granular corneal dystrophy, lattice corneal dystrophy and macular corneal dystrophy. The topic of the first Oskar Fehr lecture is Schnyder corneal dystrophy (SCD), an autosomal dominantly inherited corneal dystrophy associated with abnormal cholesterol deposition in the cornea. METHODS:The clinical, histopathologic and genetic findings of 115 individuals with SCD followed over 18 years are discussed. The impact of systemic cholesterol metabolism on other diseases is reviewed. RESULTS:Corneal findings in SCD are predictable on the basis of patient age. All patients develop progressive corneal haze because of abnormal deposition of corneal lipid, but only half of patients with SCD have evidence of corneal crystals. The prior name for this disease, Schnyder crystalline corneal dystrophy, led me to create the International Committee for the Classification of Corneal Dystrophies, in order to create a more up-to-date and accurate nomenclature for SCD and other corneal dystrophies. The name was then changed to Schnyder corneal dystrophy. Histopathology of excised SCD corneas demonstrates abnormal deposition of only HDL cholesterol. Mutations in the UBIAD1 gene result in SCD. Three dimensional protein modeling shows that mutations result in impaired vitamin K synthesis, suggesting a common link between vitamin K and cholesterol metabolism. UBIAD1 mutations are associated with other diseases, such as bladder carcinoma and Parkinson's disease like findings in Drosophila. CONCLUSIONS:Studies of the cause of SCD have led to the discovery of UBIAD1 gene mutations; further work has demonstrated the systemic importance of this gene. The association of vitamin K metabolism and cholesterol metabolism may give us insight into other diseases, so that SCD research may also have implications beyond ophthalmology.
journal_name
Klin Monbl Augenheilkdjournal_title
Klinische Monatsblatter fur Augenheilkundeauthors
Weiss JSdoi
10.1055/s-0042-100735subject
Has Abstractpub_date
2016-06-01 00:00:00pages
708-12issue
6eissn
0023-2165issn
1439-3999journal_volume
233pub_type
abstract:BACKGROUND:Spontaneous orbital haemorrhage can occur at any age. The clinical presentation is often dramatic with acute painful proptosis and nausea. Vision may be severely impaired. HISTORY AND SIGNS:A 77 years old lady woke up with sudden retroocular pain, diplopia and proptosis. Her blood pressure was 235 / 95 mmHg...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1027354
更新日期:2008-05-01 00:00:00
abstract::Corneal topographs that measure the anterior and posterior corneal surface with optical methods need a clear cornea for precise measurements. Opacities cause artifacts in the corneal thickness (with measurements usually being too thin) and corneal curvatures. This is important to know as certain pathologies may repeat...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-0032-1327956
更新日期:2012-12-01 00:00:00
abstract:BACKGROUND:We investigated the presence of Nicotinamid-adenin-dinucleotid-phosphat (NADPH)-diaphorase activity in endothelial cells of the human uveal vessels, using a histochemical procedure which presumably localizes the reaction product nitric oxide. MATERIAL AND METHODS:11 donor eyes destined for corneal transplan...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1035414
更新日期:1995-02-01 00:00:00
abstract::In 300 cases the effectiveness of the long-acting anesthetic Meaverin-ultra for retrobulbar anesthesia was systematically investigated. The duration of analgesis and immobilisation for the globe as well as for the eyelids runs over hours; they are considerabley more effective than with other common local anesthetics s...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:
更新日期:1977-09-01 00:00:00
abstract::A case report is given of a vascular disease of right retina of a 37-year-old woman during treatment with contraceptives during 9 years. The retina shows at first a clinical picture of chorioretinitis centralis serosa. After stopping the hormonal preparation and prescription of Dexium (Kalziumdobesilat) the retinal ch...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:
更新日期:1976-10-01 00:00:00
abstract::The disciform macular degeneration is in rare cases preceded by well-known diseases which have in common the destruction of the Bruch's membrane, recurrent haemorrhages with exudation from the choriocapillaris and later on ingrown of fibrovascular tissue into the subretinal space. The authors report on cases with chor...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:
更新日期:1976-02-01 00:00:00
abstract::Report on a patient with several symptoms which had not previously occurred together. The main symptom is palmoplantar keratosis; further alterations were kypho-skoliosis, brachytelephalangia of the thumbs, and in the eyes, keratoconus, a cataract and electroretinographic alterations. ...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1057732
更新日期:1980-12-01 00:00:00
abstract:BACKGROUND:The potential impact of coagulation abnormalities on retinal vascular occlusive diseases, individually and in combination with cardiovascular risk factors, remains unclear. PATIENTS AND METHODS:In a prospective case-control study a cohort of 74 young patients with central, hemicentral or branch retinal vein...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-0028-1109195
更新日期:2009-09-01 00:00:00
abstract:BACKGROUND:Xerophthalmia, the eye manifestation of vitamin A deficiency, is one of the main reasons of blindness in developing countries but is rare in industrial countries. PATIENTS:We report on 2 cases of night blindness and conjunctival and corneal xerosis because of hypovitaminosis A. One patient developed vitamin...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2000-10386
更新日期:2000-07-01 00:00:00
abstract::Endophthalmitis after penetrating keratoplasty is rare but more frequently observed than after other intraocular procedures. The main source may be a contaminated donor cornea. This survey reviews current concepts of donor globe decontamination and avoidance of infection during preservation. Basically, we suggest the ...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章,评审
doi:10.1055/s-2008-1035268
更新日期:1996-07-01 00:00:00
abstract:BACKGROUND:The aim of this study was to compare the results of intraocular pressure (IOP) measurements obtained using the pressure phosphene tonometer Proview with those from Goldmann applanation tonometer (GAT) in normal and glaucomatous eyes. PATIENTS AND METHODS:The IOP in 150 eyes of 62 healthy volunteers and 88 p...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2006-927094
更新日期:2006-11-01 00:00:00
abstract::Complicated ptosis can be corrected by frontal suspension with autologous fascia lata. Via an open approach, the fascia is implanted between the brow and the tarsal plate behind the septum. The oblique implantation between the tarsus and the brow preserves the flexibility of elevation, closure and blinking of the uppe...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章,评审
doi:10.1055/s-0031-1281848
更新日期:2012-01-01 00:00:00
abstract::The generally accepted classification of pituitary adenomas into eosinophilic, basophilic, mixed, and chromophobe types has not been very useful since no clear correlation exists between the staining character of the tumor cells and the clinical syndrome produced. The ultrastructural examination of different adenoma t...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:
更新日期:1977-02-01 00:00:00
abstract:OBJECTIVE:To study the learning risk of a new surgical technique, the deep sclerectomy (DS), which may be complicated with a perforation of the trabeculodescemetic membrane. To do so, we compared the results and the complications of patients who underwent DS transformed in trabeculectomy (DSt) and those of patients who...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1035050
更新日期:1997-05-01 00:00:00
abstract:BACKGROUND:Cell adhesion in the limbal region is of outstanding importance for the regeneration of the corneal epithelium and for repair mechanisms after antiglaucomatous fistulating surgery. In the basement membranes cell adhesion is largely modified by the extracellular matrix protein laminin. The aim of our study wa...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1035120
更新日期:1997-09-01 00:00:00
abstract::Thanks to technical advances the surgical options for corneal grafting have been widely expanded in recent years. Promoted by new instruments and techniques for intrastromal dissection a number of variations of lamellar keratoplasty, including descriptive terms and abbreviations have been suggested. This creative chao...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2005-858461
更新日期:2005-08-01 00:00:00
abstract::A report is given on seventeen cases of spontaneous regression of diabetic retinopathy. The diabetes became manifest, without exception, at an early age, particularly in childhood. Regression came on slowly and inconspicuously, retinopathy disappearing completely in two thirds of the cases. Of the other forms of diabe...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:
更新日期:1976-11-01 00:00:00
abstract:BACKGROUND:Vitamin A deficiency is a leading cause of blindness in childhood in the developing countries. In developed countries xerophthalmia occurs mainly as secondary hypovitaminosis due to malabsorption in chronic alcoholism or intestinal disorders. PATIENT AND METHODS:A 24-year-old female patient complained of ni...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1034956
更新日期:1998-08-01 00:00:00
abstract::A "false faden operation was performed in 13 cases. In contrast to Cüpper's faden operation it had no lasting effect. The results of other authors were not confirmed. ...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:
更新日期:1981-03-01 00:00:00
abstract::There are multiple possible intraocular manifestations in patients with systemic neoplasms. At first line there are uveal, retinal and vitreous metastases from carcinomas, melanomas and lymphomas. In patients with von Hippel Lindau syndrome or familiar adenomatous polyposis coli (FAP), Gardner or Turcot syndromes, fun...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章,评审
doi:10.1055/s-0031-1273474
更新日期:2011-07-01 00:00:00
abstract::Report on 530 lens implantation procedures performed in the years from 1981 to 1985. Different complications with different lens models are mentioned, in particular cystoid macular edema, subluxation or luxation of the artificial lens, vitreous complication, secondary cataract, and retinal detachment. A comparison was...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1054355
更新日期:1985-11-01 00:00:00
abstract:BACKGROUND:A meticulous fundoscopy is an essential examination before administering an orthoptic occlusion therapy (patching), even in cases with a "clear indication" for patching. In this case of an 8-year-old hyperopic boy the subsequent fundoscopy revealed a bilateral maculopathy that explained the stagnation of the...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1027270
更新日期:2008-05-01 00:00:00
abstract::Visual acuity can be assessed by different strategies. The constant-stimulus-, the stair-case-, the Best-PEST-strategies, and the EN ISO 8596 are discussed. In cases of presumed psychogenic visual impairment and malingering it can be useful to modify these strategies striving for a determination of the (minimum) visua...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2004-812893
更新日期:2004-07-01 00:00:00
abstract:BACKGROUND:Gene therapy has gained increasing attention and a number of ongoing clinical trials have been initiated. This article provides current perspectives and limitations on gene therapy in ophthalmology. Since a number of comprehensive studies on gene therapy for retinal diseases already exist, we focus attention...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章,评审
doi:10.1055/s-2001-13073
更新日期:2001-03-01 00:00:00
abstract::On the basis of SEM studies it has been shown that the cilia are arranged into groups of 10 to 40 in the individual epithelial cells of the lacrimal sac. The cilia have a length of 15 to 20 micron. ...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:
更新日期:1977-03-01 00:00:00
abstract::In everyday practice, intravitreal therapy in an "as needed" regimen (pro re nata, PRN) is less predictable and requires more visits (monitoring and injections taken together) with poorer functional results than with the treat and extend (T&E) regimen. Current literature supports the benefit of a switch. However, prac...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章,评审
doi:10.1055/s-0042-118184
更新日期:2018-01-01 00:00:00
abstract:BACKGROUND AND PURPOSE:With the discrepancy between availability and demand for corneal donors, the purpose of our study was to identify significant factors influencing the suitability and affecting the main reasons for discarding organ-cultured corneas. We thus aimed to minimise the percentage of unsuitable corneas in...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/a-1141-3703
更新日期:2020-11-01 00:00:00
abstract:BACKGROUND:Infections with varicella zoster virus may involve the optic nerve and the retina. Different pathomechanisms have been discussed. We present a case with an autoimmune inflammatory reaction according to the clinical course. PATIENT:A 69-year-old female was referred to our clinic because of suspected bilatera...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1034771
更新日期:1999-03-01 00:00:00
abstract::The serum of 63 patients suffering from various clinical types of endogenous uveitis with unknown etiology was investigated for the presence of circulating immune complexes (CIC). None of the patients was suffering from a systemic disease usually associated with CIC. Significantly elevated CIC levels were detected in ...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1054906
更新日期:1983-09-01 00:00:00
abstract::An intralental metallic foreign body was identified by computerized tomography and removed by extracapsular cataract extraction. A Simcoe posterior chamber lens was implanted without any problem. The problems of lens implantation in younger patients are pointed out. ...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1050752
更新日期:1986-07-01 00:00:00