Abstract:
BACKGROUND:Bilateral stromal corneal opacifications are a differential diagnostical challenge to identify associated systemic diseases. CASE-REPORT:A 47-year old civil engineer (height 167 cm) with bilateral stromal corneal clouding presented with visual loss for the last 27 years: VA 20/100 OD and 20/50 OS (following penetrating keratoplasty OS). The cornea showed milky-whitish, cloudy, diffuse stromal deposits without a separate lipoid arc. The posterior segment showed tapetoretinal degeneration. Scotopic ERG was decreased. A suspicious stiffness of interphalangeal joints on both hands was observed. There was an aortic and mitral insufficiency grade I. Serum levels of LDL, HDL and triglycerides were normal. The biomicroscopical diagnosis of Scheie's syndrome (mucopolysaccharidosis I-S) was confirmed by a deficiency of alpha-L-iduronidase in leukocytes (0.02 nmol/min/mg protein, normal range: 0.3 - 1.5). CONCLUSION:The differential diagnosis of bilateral corneal stromal opacification includes in addition to the mucopolysaccharidoses HDL-deficiency diseases (LCAT deficiency, Tangier disease, Fish eye disease), Schnyder's crystalline stromal dystrophy, cystinosis, gout and mucolipidoses. MPS I-S may easily be detected by alpha-L-iduronidase deficiency in leukocytes and increased mucopolysaccharides in the urine. Furthermore, patients with MPS I-S need general medical care because of cardiovascular abnormalities, joint stiffness and myopathies.
journal_name
Klin Monbl Augenheilkdjournal_title
Klinische Monatsblatter fur Augenheilkundeauthors
Viestenz A,Shin YS,Viestenz A,Naumann GOdoi
10.1055/s-2002-35688subject
Has Abstractpub_date
2002-10-01 00:00:00pages
745-8issue
10eissn
0023-2165issn
1439-3999journal_volume
219pub_type
杂志文章abstract::27 children with severe brain injuries were examined by an ophthalmologist and had several paediatric examinations during an eight or ten weeks' course of rehabilitation. With highly reduced levels of consciousness they had hardly any cortical visual function but frequently had brainstem controlled reactions of eyelid...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1045861
更新日期:1992-07-01 00:00:00
abstract::On the basis of a number of examples the authors describe specific glaucomas which have often reached their final stages by the time the patients are referred to the clinic. They include sub-acute and chronic angle-closure glaucoma, pseudoexfoliation syndrome, and pigmentary glaucoma. An unpleasant feature of these gl...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1046389
更新日期:1989-05-01 00:00:00
abstract:BACKGROUND:Disturbances in the microcirculation are a source of retinal diabetic alterations. Comprehensive studies of blood flow parameters have been performed, but it is not known how ageing, duration of diabetes, blood sugar, glycolysed haemoglobin and elevated systemic blood pressure act in the early stages of diab...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2007-963156
更新日期:2007-05-01 00:00:00
abstract::Binocular perimetry with Aulhorn's Phase difference haploscope is described. Of 62 patients with primary micro-squint, 21 patients showed no scotoma, 15 patients showed a fixation or zero point scotoma of 0.5-1 degrees, 26 patients showed a larger scotoma (average: 3.7 degrees). In addition, 15 patients with larger an...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:
更新日期:1978-10-01 00:00:00
abstract::Scanning laser tomography (HRT, HRT II), scanning laser polarimetry (GDx nerve fibre analyser, GDx VCC), retinal thickness analyser (RTA) and optical coherence tomography (OCT, stratus OCT) provide objective measurements of the optic disc and the retinal nerve fibre layer suitable for the follow-up of glaucoma patient...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章,评审
doi:10.1055/s-2005-859004
更新日期:2006-06-01 00:00:00
abstract::Oral doses of propranolol and metoprolol were tested on 24 patients without glaucoma. The aim of this study was to compare the decrease in intraocular pressure in normal eyes following a single oral dose of propranolol or metoprolol. A statistically significant decrease in IOP was noticed, but no difference was seen b...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:
更新日期:1980-01-01 00:00:00
abstract::Presbyopia and cataract patients' wish to be increasingly independent of spectacles after surgery and this is one of the main drivers for the development of multifocal intraocular lenses (MIOL) and extended depth of focus (EDOF) intraocular lenses (IOL). As education, biometry, diagnostics, surgical techniques, and MI...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章,评审
doi:10.1055/a-1111-9380
更新日期:2020-08-01 00:00:00
abstract::In anophthalmos the forces which stimulate development and growth of the bony orbit are absent. As a result these cases have not only a tiny socket but also an underdeveloped bony orbit, which cannot retain an artificial eye. The small conjunctival sac and bony orbit can be mechanically enlarged by means of Hema socke...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1050502
更新日期:1987-09-01 00:00:00
abstract::Since more than 40 years the judgement of the degree of disability in cases of unilateral aphakia is discussed. This means the effort toward an advisory objectivity on the one hand and human understanding for the patient's damage on the other hand. It is supposed to repeal the 4th emergency ordinance of 1932 and to pa...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:
更新日期:1979-07-01 00:00:00
abstract::These techniques are used in far-advanced cases of glaucoma when the campimetric method is not practicable due to a considerable loss of visual field. The patient takes a mixture of glycerol and ascorbic acid as a hypotensive agent, his visual field or visual acuity and intraocular pressure are checked every 30 minute...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1055143
更新日期:1982-06-01 00:00:00
abstract:BACKGROUND:The motorist obtains about 90% of the information required to drive a vehicle from the sense of vision. In road traffic, various situations with respect to size, distance, and position have to be evaluated at different speeds. Does depth perception based on disparity play an essential role in these situation...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2000-10343
更新日期:2000-09-01 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::We monitored 300 patients who had undergone ambulatory cataract operations. Patients are more and more preferring this type of surgery, which allows them to go home immediately after the operation. We compare ambulatory ECCE and phacoemulsification operations and their complications, advantages and disadvantages. We s...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1035536
更新日期:1994-05-01 00:00:00
abstract::The present study consists of light- and electron-microscopic examination of excised cornea in a case of lipid keratopathy (Schnyder) in which a perforating keratoplasty was performed. It demonstrates the deposition of cristalline and non-cristalline substances in the corneal epithelium as well as the anterior half of...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:
更新日期:1978-09-01 00:00:00
abstract::Entopic perception of the central choriocapillaris is documented by personal experience. Seen in this manner, the choriocapillaris appears due to circulatory conditions as a wavy convolute of fresh capillaries whose paths are paralleled by numerous, irregularly distributed, round, black structures. The latter have bee...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1046095
更新日期:1991-11-01 00:00:00
abstract:BACKGROUND:Moisture chambers can protect the eye in corneal lubrication or eyelid closure disorders. However, in cases with protrusio bulbi or pronounced chemosis conjunctivae direct damaging contact of the cornea or conjunctiva on the one hand and the inside of the moisture-chamber-bandage on the other hand are possib...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1034745
更新日期:1999-01-01 00:00:00
abstract::Description of various affections of the iris which may be treated with argon-laser. The problem of the laser iridectomy in the treatment of glaucoma is illustrated by a histologic study of the evolution of the lesions up to four months after coagulation of rabbit's iris. ...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1055016
更新日期:1982-01-01 00:00:00
abstract:BACKGROUND:Cat scratch disease (CSD) is a common infectious disease, however its association with a disciforme keratitis is to our knowledge a previously unreported ocular complication. PATIENT:A 65-year old female was treated unsuccessfully with topical antibiotics, acyclovir and corticosteroids for several weeks bec...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2000-10372
更新日期:2000-11-01 00:00:00
abstract::36 malignant tumours of the eyelids (basaliomas, basal-cell carcinomas and squamous-cell carcinomas) have been treated by intrapalpebral injections of a 5% to 25% solution of urea as recommended by E.D. Danopoulos. 1.5 to 3.0 ml of the solutions were injected around the tumours twice per week. Six malignant tumours di...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:
更新日期:1976-07-01 00:00:00
abstract:OBJECTIVE:The aim of this study is to describe the effectiveness of aflibercept in treatment-naive patients with neovascular age related macular degeneration (nAMD) and the impact of patients' residential distance to the clinic and patients' mobility on therapy in a pro re nata regime. METHODS:A retrospective dataset ...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/a-1008-9357
更新日期:2020-06-01 00:00:00
abstract::Nine patients with peripheral chronic cyclitis and cystic edema of the macula were treated with azathioprine and small doses of corticosteroids. The results were quite good. In fact the inflammatory signs subsided nearly completely, while the cystic edema was still present in 2 cases. Besides excellent compatibility, ...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 临床试验,杂志文章
doi:
更新日期:1977-03-01 00:00:00
abstract:BACKGROUND:Laser peripheral iridotomy is a useful method in primary angle-closure eyes to prevent angle-closure attack and development of glaucomatous optic nerve damage. The aim of this study was to quantify morphological changes after LPI and their impact on intraocular pressure, and to evaluate predictive parameters...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 临床试验,杂志文章
doi:10.1055/s-0035-1545793
更新日期:2015-04-01 00:00:00
abstract::The value of a register of corneal grafts is that it can provide new data about the long-term outcome of corneal transplantation and may allow a more reasoned distribution of scarce donor corneas. The large numbers of cases and events can accommodate wide variations in practice patterns without losing statistical powe...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1045527
更新日期:1994-11-01 00:00:00
abstract::A Pakistani wall plate depicting the anatomy of the eye is described and illustrated. The picture is partly Greek (the hollow optic nerve), in part medieval Arab (the optical image on the disk), and in part modern (e.g., the refraction anomalies). A brief account is given of how Greek science passed to the Arabs and t...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 历史文章,杂志文章
doi:10.1055/s-2008-1050281
更新日期:1988-10-01 00:00:00
abstract::In 20 cases of herpes simplex keratitis the efficacy of Vidarabin ointment has been tested, in 19 cases after corneal abrasion. The eyes were treated once a day and padded until the epithelial defects had closed. Thereafter the ointment was applied 4 times per day for about one week more. On the average epithelial clo...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 临床试验,杂志文章
doi:
更新日期:1978-02-01 00:00:00
abstract::A 31-year-old patient was buried at canal-building. He sustained Purtscher's retinopathy on his left eye. A fluorescein-angiography displayed six years after the trauma a large neovascular frond and intraretinal neovascular network. These findings demonstrate the severe lesions of the retinal microcirculation caused b...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:
更新日期:1976-06-01 00:00:00
abstract:AIM:The necessity for routine disinfection of floors between two surgical procedures or disinfection only after visible contamination was assessed in two identical ophthalmological operating theatres equipped with laminar air flow ventilation. METHOD:Over a period of four weeks, one of the two tested operating rooms w...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章,随机对照试验
doi:10.1055/s-0029-1245657
更新日期:2010-11-01 00:00:00
abstract::Normal tension glaucoma is a variant of primary open angle glaucoma. It is characterized by normal intraocular pressure. Although there are several pathophysiological explanations (e. g. vascular dysregulation, the role of the lamina cribrosa), none of these explanations can fully explain its pathophysiology. The opti...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/a-0809-5692
更新日期:2019-02-01 00:00:00
abstract:BACKGROUND:Duane retraction syndrome (DURS) accounts for 1 - 4 % of all cases of strabismus. Approximately 90 % of the cases are sporadic with a preponderance for females and the left eye. Many associated ocular and systemic findings have been described. Recently, mutations of SALL4 have been found in patients with aut...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 临床试验,杂志文章
doi:10.1055/s-2004-813662
更新日期:2004-10-01 00:00:00
abstract::A comparative study of the intraocular pressure-lowering effects of timolol and phospholine iodide in cases of aphakic glaucoma was carried out. The authors compared the I.O.P.-lowering effect of these two drugs in 30 eyes (30 patients) with aphakic glaucoma over an eight-month observation period. The following was es...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1057292
更新日期:1981-09-01 00:00:00