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 mitochondrial, ADOA of the nuclear DNA. LHON is a disease that usually leads to severe visual impairment (visual acuity < 0.1). Since there is an approved therapy for LHON (Idebenone [Raxone]), the diagnosis has to be confirmed immediately by means of molecular genetic diagnostics. ADOA usually shows less severe visual impairment, begins in childhood, and progresses gradually, often for a long time without any noticeable worsening. Family history (dominant heredity) and blue colour vision disorder are the leading indicators for the diagnosis of ADOA, which should be confirmed by molecular genetic testing. Optic nerve diseases can also occur in the context of hereditary, syndromic disorders. Ophthalmologically relevant are Wolfram syndrome, Friedreich's ataxia and Charcot-Marie-Tooth disease. In all hereditary optic nerve disorders, foods containing cyanide and smoking should be avoided. Excessive alcohol consumption is considered to be a trigger of LHON and possibly harmful in other hereditary optic neuropathies. In the management of patients with hereditary optic nerve disorders, aspects of rehabilitation as well as social and psychological care should be considered. :Trotz der Seltenheit haben die erblichen Sehnerverkrankungen den Vorteil, dass sie oftmals durch die Anamnese erkannt werden können und die Diagnosesicherung heute sicher durch eine molekulargenetische Untersuchung geleistet werden kann. Als isolierte erbliche Sehnerverkrankungen sind im augenheilkundlichen Alltag die Leberʼsche hereditäre Optikusneuropathie (LHON) und die autosomal-dominante Optikusatrophie (ADOA) relevant.
journal_name
Klin Monbl Augenheilkdjournal_title
Klinische Monatsblatter fur Augenheilkundeauthors
Rüther Kdoi
10.1055/a-0583-6290subject
Has Abstractpub_date
2018-06-01 00:00:00pages
747-763issue
6eissn
0023-2165issn
1439-3999journal_volume
235pub_type
杂志文章,评审abstract::The results of campimetric investigations performed in 127 eyes (83 patients) are presented. The vertical diameter of the blind spot and intraocular pressure were measured several times before and after oral intake of a dose of glycerol combined with ascorbic acid. The tonometric value obtained when the blind spot is ...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1055032
更新日期:1982-02-01 00:00:00
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. ...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1057359
更新日期:1981-12-01 00:00:00
abstract::In a series of cataract operations with a corneal incision PDS 10-0 absorbable sutures were tested. In contrast to deeper PDS sutures, such as are used in corneoscleral incisions, the corneal sutures opened as early as between 13 and 30 days postoperatively in 5 out of 7 cases. Postoperative irritation was more severe...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1054984
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journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章,评审
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journal_title:Klinische Monatsblatter fur Augenheilkunde
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更新日期:2005-11-01 00:00:00
abstract::In 14 out of 25 patients with retinoblastoma there was a family history of malignancies. Most of the malignant tumours had been carcinomas. In 2 families relatives died because of leukaemia; in these cases the tumour was a so-called diffuse retinoblastoma occurring in elder children. A third family with a retinoblasto...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:
更新日期:1976-05-01 00:00:00
abstract::A- and B-scan sonography was performed in 83 patients with choroidal detachment; a serous choroidal detachment is characterized by high, bifurcated echospikes and an echo-free subchoroidal space in A-scan sonography. The B-scan presents a typical arc shape. In hemorrhagic choroidal detachment multiple echoes with low ...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1054708
更新日期:1983-01-01 00:00:00
abstract::The clinical application of TV fluorescein angiography with subsequent image analysis as performed on a patient with familial Mediterranean fever (FMF), who was suffering from an arterial branch occlusion, is discussed. In addition, the diagnostic relevance of rheologic parameters to the diagnostic evaluation of retin...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1050888
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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:
更新日期:1997-05-01 00:00:00
abstract::Symptoms, genetics and differential diagnosis of this rare, congenital deformity of the lids are described. Strategies for the conservative and surgical therapy on the basis of own experiences and an extensive review of the literature are presented. ...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章,评审
doi:10.1055/s-2008-1035418
更新日期:1995-03-01 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::Kaposi's sarcoma occurs as a complication in numerous cases of AIDS, the much-discussed acquired immune deficiency syndrome. Considering the latter's near-epidemic incidence, the clinical and histological features of Kaposi's sarcoma are recalled to mind. Hitherto only sporadically recorded in non-African countries, t...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1050977
更新日期:1985-07-01 00:00:00
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journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-0041-111801
更新日期:2016-04-01 00:00:00
abstract:BACKGROUND:No data is available to date on the cost effectiveness of laser in situ keratomileusis (LASIK) with regard to the German-speaking health care system. Hence we compared the clinical outcome and cost data of two German LASIK centres in respect of procedure cost effectiveness from the patient perspective. MATE...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章,多中心研究
doi:10.1055/s-2003-39424
更新日期:2003-05-01 00:00:00
abstract::In 1876 A. Weber introduced chemically from P. jaborandi isolated Pilocarpium muriaticum into the ophthalmological therapy. One year later it was used as a local drug to lower the intraocular pressure in glaucoma and replaced in the following years the extract of Calabar bean (Eserine). Clinical and pharmacological da...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 历史文章,杂志文章
doi:
更新日期:1976-11-01 00:00:00
abstract::Report on a rare case of an iris-ringmelanoma. Arising from the iris base the primary tumor spread in a continuous line along the anterior chamber angle and a shedding of tumor cells in other places of the iris surface could be stated as well. A unilateral dark spotted colouring of the iris with a secondary glaucoma w...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:
更新日期:1976-03-01 00:00:00
abstract::Light phenomena evoked by a Kratz elliptical posterior chamber lens, a Mark Choyce IX anterior chamber lens, and a four-loop Binkhorst anterior chamber lens were investigated in a model with pupils of various sizes. Besides monocular diplopia, the edge of the lens produces scattered light phenomena which vary accordin...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1050471
更新日期:1987-07-01 00:00:00
abstract::Defects of the medial canthal area with partial full-thickness loss of the upper and lower eye-lid can be repaired by the following technique: closure of the larger lid defect by a free transplant of tarsus and lid margin from the opposite lower lid and a sliding skin flap. The minor lid defect is closed by a pedicled...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:
更新日期:1976-08-01 00:00:00
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journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
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journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:
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journal_title:Klinische Monatsblatter fur Augenheilkunde
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journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章,评审
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更新日期:2012-10-01 00:00:00
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journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
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更新日期:1987-01-01 00:00:00
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journal_title:Klinische Monatsblatter fur Augenheilkunde
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更新日期:2011-08-01 00:00:00
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journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章,评审
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更新日期:2011-12-01 00:00:00
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journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 临床试验,杂志文章,随机对照试验
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更新日期:1996-08-01 00:00:00
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journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章,评审
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更新日期:2006-08-01 00:00:00
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journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1057727
更新日期:1980-12-01 00:00:00
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journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
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journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
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更新日期:1990-12-01 00:00:00