[Electroretinography changes in carriers of X-chromosomal retinitis pigmentosa].

Abstract:

:Out of five families with pedigrees typical for X-linked retinitis pigmentosa (RP) the authors examined eight definite and eight possible female carriers. Subjective symptoms were mild and variable, consisting mainly of relative nyctalopia. Ophthalmoscopic examination revealed a normal fundus, or mild changes suggestive of RP, or even marked changes. Perimetry (Goldmann) revealed some narrowing of the outer isopter corresponding to a significant reduction in visual field area. In testing dark adaptation, the final rod threshold was slightly elevated in five out of 16 subjects. Ganzfeld-electroretinography using matched band filters in conditions of dark and light adaptation to selectively stimulate rod and cone signals revealed reduced b-wave amplitudes in most cases. White stimuli, applied in dark adaptation, generated ERGs with less abnormality than the selective rod and cone ERGs. Peak times of the cone b-waves were prolonged in most cases. The ERG abnormalities were more pronounced in definite carriers and in advanced age. In some cases remarkable differences were noted between the two eyes as regards fundus morphology and ERG responses. The authors conclude that the possibility of improved genetic and educational counseling in families afflicted by X-linked RP justifies the considerable diagnostic effort involved in such examinations.

journal_name

Klin Monbl Augenheilkd

authors

Brischnik E,Niemeyer G

doi

10.1055/s-2008-1050130

subject

Has Abstract

pub_date

1988-05-01 00:00:00

pages

387-90

issue

5

eissn

0023-2165

issn

1439-3999

journal_volume

192

pub_type

杂志文章
  • [Topography of the posterior chamber lens (author's transl)].

    abstract::The position of the implanted posterior chamber lens (Shearing type) in human cadaver eyes was photographed from the rear. We were especially interested in the position of the loops. ...

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    doi:10.1055/s-2008-1057359

    authors: Schnaudigel OE

    更新日期:1981-12-01 00:00:00

  • [Effect of corneal collagen shields and epithelial growth promoting factors on epithelialization of the human cornea].

    abstract:PATIENTS AND METHODS:In 28 patients after perforating keratoplasty followed by epithelial abrasion of the transplant the period required for epithelial closure with a therapy including collagen shields and the growth stimulating factors vitamin A and Epidermal Growth Factor (EGF) was determined. RESULTS:A significant ...

    journal_title:Klinische Monatsblatter fur Augenheilkunde

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    doi:10.1055/s-2008-1045517

    authors: Kammann JP,Vollenberg C

    更新日期:1994-10-01 00:00:00

  • [Keratoplasty in private practice].

    abstract::The problems associated with performing penetrating keratoplasty in private practice are briefly discussed: access to an eye bank, high-risk patients, immunosuppressive therapy, complications, and results. One negative aspect is the relatively high incidence (10%) of primary graft failure, but allograft reactions are ...

    journal_title:Klinische Monatsblatter fur Augenheilkunde

    pub_type: 杂志文章

    doi:10.1055/s-2008-1046177

    authors: Witmer R

    更新日期:1990-05-01 00:00:00

  • Sympathetic Ophthalmia - a Contribution to Immunology, Clinic and Current Imaging.

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    authors: Rua D,Pohlmann D,Pleyer U

    更新日期:2020-09-01 00:00:00

  • [Pattern-induced after effects caused by an on-screen workstation].

    abstract::Millions of people work or spend their free time sitting in front of VDUs that induce contingent visual effects. However, only a few notice irritations in the course of everyday work: motion after-images such as the waterfall illusion or tilt after-effects are possible. A new effect may be caused by those new VDU moni...

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    doi:10.1055/s-2006-926691

    authors: Kluxen G

    更新日期:2006-08-01 00:00:00

  • [Luetic papilledema].

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    journal_title:Klinische Monatsblatter fur Augenheilkunde

    pub_type: 杂志文章

    doi:

    authors: Bodechtel G,Lund OE

    更新日期:1979-12-01 00:00:00

  • [Local anesthetic effect and subjective tolerance of a single dose of 0.25% bupranolol in probands with healthy eyes. Randomized parallel double-blind study].

    abstract::.25% Bupranolol in an oily solution decreases corneal sensitivity statistically significant from the 3. to the 15. minute after the application. The maximum of decrease is found between the 6. and the 15. minute in contrast to beta-blockers in aqueous solution (1.-3. minute). The topical subjective compatibility of 0....

    journal_title:Klinische Monatsblatter fur Augenheilkunde

    pub_type: 临床试验,杂志文章,随机对照试验

    doi:10.1055/s-2008-1045923

    authors: Bastian B,Höh H

    更新日期:1991-01-01 00:00:00

  • [Medallion lenses, 500 implantations].

    abstract::A report is given on the implantation of 500 Medallion lenses, most of which were implanted after intracapsular cataract operation (88%) and were kept under observation for between 3 and 88 months (average 22 months). In 1.2% a severe keratopathy resulted from an operation that was too traumatic, from decentration of ...

    journal_title:Klinische Monatsblatter fur Augenheilkunde

    pub_type: 杂志文章

    doi:10.1055/s-2008-1054737

    authors: Fechner PU

    更新日期:1983-02-01 00:00:00

  • [Effect of nifedipine on ocular pulse amplitude in normal pressure glaucoma].

    abstract:BACKGROUND:Ocular pulse amplitude (OPA) is reduced in normal tension glaucoma (NTG) patients when compared to non-glaucomatous, healthy control subjects. This might be related to a vasospastic reaction. The objective of this study was to determine if low OPA in NTG is associated with a vasospastic reaction and its resp...

    journal_title:Klinische Monatsblatter fur Augenheilkunde

    pub_type: 杂志文章

    doi:10.1055/s-2008-1035074

    authors: Schmidt KG,von Rückmann A,Geyer O,Mittag TW

    更新日期:1997-06-01 00:00:00

  • [Corneal endothelial cell density following strabismus surgery].

    abstract:BACKGROUND:The short-term impact of eye muscle surgery on the blood-aqueous barrier has been described by iris fluoresceinangiography and laser tyndallometry. The aim of this study was to investigate the long-term effects of eye muscle surgery on the oxygen supply of the anterior segment of the eye by determination of ...

    journal_title:Klinische Monatsblatter fur Augenheilkunde

    pub_type: 杂志文章

    doi:10.1055/s-2007-962981

    authors: Gusek-Schneider GC,Kamoun R,Klaas D,Seitz B

    更新日期:2007-03-01 00:00:00

  • [High altitude retinal hemorrhages in mountain climbers (author's transl)].

    abstract::A report is given on three patients who noticed an acute deterioration of visual acuity after a sojourn at 5000 altitude caused by retinal hemorrhages. We documented both the ophthalmological examinations such as visual acuity, fundus, perimetry and fluorescence-angiography and the systemic results. During the period ...

    journal_title:Klinische Monatsblatter fur Augenheilkunde

    pub_type: 杂志文章

    doi:

    authors: Vogt C,Greite JH,Hess J

    更新日期:1978-05-01 00:00:00

  • [Developments in ophthalmologic electrophysiology].

    abstract::During the last years electrophysiology in ophthalmology has been facilitated to a big extent by computerized methods. Thus it has become a suitable method for an increasing number of relevant diagnostic problems. To make comparisons between test results of different diagnostic places easier an international standard ...

    journal_title:Klinische Monatsblatter fur Augenheilkunde

    pub_type: 杂志文章,评审

    doi:10.1055/s-2008-1045575

    authors: Rüther K,Zrenner E

    更新日期:1993-02-01 00:00:00

  • [Virological diagnosis of ocular adenovirus infection].

    abstract::For laboratory diagnosis, virus isolation and identification from conjunctival swabs should be combined with serology (two blood specimens: early and 14 days or later after onset of disease). The group-specific complement fixation fails in almost one-half of the cases. Additional type-specific reactions (neutralizatio...

    journal_title:Klinische Monatsblatter fur Augenheilkunde

    pub_type: 杂志文章

    doi:10.1055/s-2008-1055142

    authors: Wigand R,Keller D

    更新日期:1982-06-01 00:00:00

  • [Recurrent fibrous histiocytoma at the corneoscleral limbus].

    abstract::This case report describes a rare fibrous histiocytoma at the corneoscleral limbus in a 10-year-old boy. In our patient the tumour was histologically classified as benign, however, it presented with an invasive growth pattern and recurrency, so that a penetrating sclerokeratoplasty became necessary. This case is set i...

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    doi:10.1055/s-2008-1027125

    authors: Maier PC,Auw-Hädrich C,Reinhard T

    更新日期:2008-01-01 00:00:00

  • [Influence of corneal transparency on the quality of topographies].

    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...

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    doi:10.1055/s-0032-1327956

    authors: Franko Zeitz P,Kohlhaas M

    更新日期:2012-12-01 00:00:00

  • [Tubulointerstitial nephritis and uveitis (TINU syndrome) - comorbidity and complications in four patients].

    abstract:BACKGROUND:TINU syndrome probably is a frequently overlooked disease where uveitis occurs in association with acute tubulointerstitial nephritis. Diagnostic criteria have been published recently. PATIENTS AND METHODS:In this retrospective case series the charts of four consecutive patients with TINU syndrome (follow-u...

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    pub_type: 杂志文章

    doi:10.1055/s-2002-33583

    authors: Hudde T,Heinz C,Neudorf U,Hoeft S,Heiligenhaus A,Steuhl KP

    更新日期:2002-07-01 00:00:00

  • [Corneal wound healing after experimental penetrating keratoplasty after excimer laser trephination in free form].

    abstract:BACKGROUND:The postoperative clinical course after penetrating keratoplasty and trephination in free form using a guided excimer laser beam has been published before. Here the findings of light-microscopy comparing corneal wound healing after experimental penetrating keratoplasty after laser trephination and after conv...

    journal_title:Klinische Monatsblatter fur Augenheilkunde

    pub_type: 评论,杂志文章

    doi:10.1055/s-2006-927097

    authors: Schmitz K,Lang GK,Behrens-Baumann W

    更新日期:2006-12-01 00:00:00

  • [The bilateral paralysis of the abducens nerve. A contribution to industrial medical judgement (author's transl)].

    abstract::After a brief statistical survey the anotomic basis is explained in a broad outline. In the description of a case with bilateral paralysis of the abducens nerve, the suffering of the patient in his working life is examined and the phenomenon of inacurrate depth projection, of "pointing past", is discussed. As a result...

    journal_title:Klinische Monatsblatter fur Augenheilkunde

    pub_type: 杂志文章

    doi:

    authors: Unterkircher K

    更新日期:1976-02-01 00:00:00

  • [Autoregulation in ischemia of the optic nerve in the human].

    abstract:BACKGROUND:Autoregulation is defined as the maintenance of constant blood flow in a vascular system in spite of changes in perfusion pressure (PPm). MATERIALS AND METHODS:PPm was decreased by increasing the intraocular pressure (IOP) with a suction cup and optic nerve blood flow was measured with the laser Doppler flo...

    journal_title:Klinische Monatsblatter fur Augenheilkunde

    pub_type: 杂志文章

    doi:

    authors: Titzé P,Hero M,Petrig BL,Cranstoun SD,Riva CE

    更新日期:1997-05-01 00:00:00

  • [Early vitrectomy in the treatment of severe eye injuries].

    abstract::Several authors regard early vitrectomy as the best guarantee of success in cases of severe ocular injury. The present authors reviewed their own group of 27 patients, which included 13 cases with intraocular foreign bodies, 6 cases of blunt trauma and 8 with perforating injuries. Aside from cases which later develope...

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    doi:10.1055/s-2008-1046190

    authors: Stucchi CA,Vignanelli M

    更新日期:1990-05-01 00:00:00

  • [Bilateral neuroretinitis with zoster infection].

    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...

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    doi:10.1055/s-2008-1034771

    authors: Nicaeus T,Wilhelm H

    更新日期:1999-03-01 00:00:00

  • [Silent sinus syndrome: an unusual cause of vertical diplopia].

    abstract:BACKGROUND:Causes of transient vertical diplopia include myasthenia, dysthyroid orbitopathy, multiple sclerosis, decompensation of a pre-existing state. PURPOSE:To describe an unusual cause of transient diplopia, silent sinus syndrome. METHODS:Clinical, radiological and pathology findings from a 55-year-old man with ...

    journal_title:Klinische Monatsblatter fur Augenheilkunde

    pub_type: 杂志文章

    doi:10.1055/s-2008-1034917

    authors: Durig J,Borruat FX,Jaques B

    更新日期:1998-05-01 00:00:00

  • [Specifity of optic disc evaluation in healthy subjects with large optic discs and physiologic cupping using confocal scanning laser ophthalmoscopy].

    abstract:PURPOSE:Imaging methods of the optic nerve head appear to have an increasing impact in glaucoma diagnosis. The aim of this study is to evaluate the specifity of the Heidelberg Retina Tomograph (software version 1.7 and 3.0) in subjects with physiological cupping and large optic discs. PATIENTS AND METHODS:27 eyes of 2...

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    doi:10.1055/s-0032-1328094

    authors: Plange N,Hirsch T,Bienert M,Remky A

    更新日期:2014-02-01 00:00:00

  • [Hema socket expander in primary conservative treatment of congenital anophthalmos].

    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...

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    更新日期:1987-09-01 00:00:00

  • [Management of periorbital and orbital infections].

    abstract:BACKGROUND:Infections of the orbit and of the periorbital region are not uncommon. Even today they constitute a serious problem, in spite of modern antibiotic treatment, with a potential risk of lethal complications. Orbital infections are most prevalent in children and adolescents. The acute orbit has many causes, but...

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    doi:10.1055/s-2004-813682

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    更新日期:2004-11-01 00:00:00

  • [Indications for Retinal Laser Therapy Revisited].

    abstract:BACKGROUND:Laser therapy is an important treatment option in retinal diseases, especially in cases of vascular involvement. Most approaches are based on coagulation of retinal structures. As there is increasing use of agents targetting vascular endothelial growth factor in the treatment of macular diseases, indications...

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    authors: Enders P,Schaub F,Fauser S

    更新日期:2018-12-01 00:00:00

  • [Ocular Manifestation of Mucopolysaccharidosis I-S (Scheie's Syndrome)].

    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...

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    更新日期:2002-10-01 00:00:00

  • [Hereditary Optic Neuropathies].

    abstract::Hereditary optic nerve disorders are rare. For ophthalmologists, Leber's hereditary optic neuropathy (LHON) and autosomal dominant optic atrophy (ADOA) are of particular relevance. LHON and ADOA are diseases of the retinal ganglion cells and are caused by mitchochondrial dysfunction. LHON is based on mutations of the ...

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    pub_type: 杂志文章,评审

    doi:10.1055/a-0583-6290

    authors: Rüther K

    更新日期:2018-06-01 00:00:00

  • [Visual field defects and fluorescein angiography in acute anterior ischemic optic neuropathy (author's transl)].

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    pub_type: 杂志文章

    doi:

    authors: Reuscher A,Chromek W,Kommerell G

    更新日期:1978-07-01 00:00:00

  • [Intrastromal keratorhexis with the Nd:YAG laser--a possible method of refractive surgery?].

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