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Sunday, November 22, 2020 | History

2 edition of hereditary dystrophies of the posterior pole of the eye. found in the catalog.

hereditary dystrophies of the posterior pole of the eye.

August Frans Deutman

hereditary dystrophies of the posterior pole of the eye.

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  • 25 Currently reading

Published by Van Gorcum in Assen .
Written in English

    Subjects:
  • Dystrophy -- Genetic aspects,
  • Eye -- Diseases.,
  • Heredity of disease,
  • Sclera -- Diseases

  • Edition Notes

    Other titlesThe posterior pole of the eye
    Classifications
    LC ClassificationsRE328 D48
    The Physical Object
    Pagination484p.
    Number of Pages484
    ID Numbers
    Open LibraryOL17391972M


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hereditary dystrophies of the posterior pole of the eye. by August Frans Deutman Download PDF EPUB FB2

The hereditary dystrophies of the posterior pole of the eye [Deutman, A. F] on *FREE* shipping on qualifying offers. The hereditary dystrophies of the posterior pole of the eyeAuthor: A.

F Deutman. Hereditary dystrophies of the posterior pole of the eye. Assen, Van Gorcum, (OCoLC) Online version: Deutman, A.F. (August F.). Hereditary dystrophies of the posterior pole of the eye. Assen, Van Gorcum, (OCoLC) Document Type: Book: All Authors / Contributors: A F Deutman.

Full text Full text is available as a scanned copy of the original print version. Get a printable copy (PDF file) of the complete article (K), or click on a page image below to browse page by page.

The Hereditary Dystrophies of the Posterior Pole of the Eye (Le Distrofie Ereditarie del Polo Posteriore dell'Occhio) Di A. Deutman. Royal Van Gorcum Ltd. - Assen Volume rilegato, con sovracoperta, di 17 × 25 cm; VIII + pagine; illustrazioni in bianco e nero e a colori (13 tavole).

Indice analitico e degli autori. Prezzo non indicato. - Volume 20 Issue 1 - L. The hereditary dystrophies of the posterior pole of the eye. Publication. Publication. Diminished vision as a result of macular degeneration or changes of the posterior pole of the eye constitutes an important ophthalmological problem.

Kornzweig () studied more than rooo eyes and found diminished vision as a result of an affection of the posterior pole in % of patients under So Cited by: hereditary dystrophies, macular degeneration, posterior pole of the eye: Language: English: Type: Doctoral Thesis: Publisher: Erasmus University Rotterdam: Abstract: textabstractDiminished vision as a result of macular degeneration or changes of the posterior pole of the eye constitutes an important ophthalmological problem.

The Hereditary Dystrophies of the Posterior Pole of the Eye. (PMCID:PMC) Full Text Citations ; BioEntities ; Related Articles ; External Links ; Br J Ophthalmol.

November; 55(11): PMCID: PMC The Hereditary Dystrophies of the Posterior Pole of the Eye. Diminished vision as a result of macular degeneration or hereditary dystrophies of the posterior pole of the eye.

book of the posterior pole of the eye constitutes an important ophthalmological problem. Kornzweig () studied more than rooo eyes and found diminished vision as a result of an affection of the posterior pole in % of patients under So and 3S.6% of patients over So. Doyne honeycomb retinal dystrophy (DHRD) is a condition that affects the eyes and causes vision loss.

It is characterized by small, round, white spots known as drusen that accumulate beneath the retinal pigment epithelium (the pigmented layer of the retina). Over time, drusen may grow and come together, creating a honeycomb pattern. For personal accounts OR managers of institutional accounts.

Username *. Password *. A corneal dystrophy is a rare genetic eye condition in which one or more parts of the clear outer layer of the eye (the cornea) lose their normal clarity as a result of a buildup of cloudy material.

The general term corneal dystrophy refers to a group of corneal diseases. Hereditary macular dystrophies, affecting most predominantly the macula area, have no clear demonstrable cause. It is hypothesized that they manifest early in life due to a predetermined genetic malfunction.

The term `macula' is actually a misnomer in that few of these dystrophies are limited to the posterior pole, let alone the macula itself. Deutman, hereditary dystrophies of the posterior pole of the eye.

Thesis, Van Gorcum, Assen, The Netherlands (). Google Scholar. Deutman, A.F. The hereditary dystrophies of the posterior pole of the eye.

Thesis. Van Gorcum & Comp. Assen, The Netherlands, Google Scholar. Corneal dystrophies are a group of rare, genetic diseases that affect the cornea, the front part of your are more than 20 types, each with different symptoms.

The hereditary macular dystrophies are a group of diseases that share certain similar features. In-herited in a monogenic fashion, all the typicalmendelian modes (autosomal dominant, autosomal recessive, and X-linked recessive) have been documented.

A year-old man was asymptomatic with 20/20 vision in each eye. The entire posterior pole. Posterior polymorphous dystrophy (PPD). PPD is an autosomal dominant dystrophy that starts earlier than Fuchs dystrophy and follows a more benign course.

14 It is characterized by the early appearance of vesicle-like lesions, bands or diffuse opacities. The posterior pole of each eye showed a diffuse yellowish sheen associated with some scattered yellowish dots, and the retinal periphery was normal (Fig. A fluorescein angiogram showed diffuse central transmitted hyperfluorescence without leakage and without the typical nodular hyperfluorescence of drusen (Fig.

The symptoms of corneal dystrophies result from the accumulation of abnormal material within the cornea, the clear outer layer of the eye. The cornea serves two functions – it protects the rest of the eye from dust, germs and other harmful or irritating material, and it acts as the eye’s outermost lens, bending incoming light onto the inner lens, where the light is then directed to the.

Hereditary macular dystrophies are progressive degenerations of the neurosensory retina, retinal pigment epithelium, or choroid. They affect predominantly and most profoundly the macular area.

Historically the hereditary nature of these disorders was demonstrated via pedigree analysis. More recently, modern advances in genetic testing including genetic linkage analysis, karyotyping, and the. The typical appearance in fundus flavimaculatus 6, 7 is these yellowish spots or flecks occupying the entire posterior pole out to the midperiphery, with or without a pigmentary maculopathy.

Studies of large groups and families 8, 9, 10 and long-term evaluations suggest a continuum between the typical appearances of each disease. The hereditary choroidal dystrophies are divided into (1) geographic choroidal dystrophies (central areolar, peripapillary, generalised), (2) gyrate atrophy, and (3) choroideremia.

Three X-linked forms of progressive cone-rod dystrophies each with mutations in different genes have been identified. RPE changes in the macula often give it a granular appearance and there may be a bull's-eye configuration. The hypopigmentation is most pronounced in the posterior pole and peripapillary region.

A diffuse yellow metallic sheen is seen in the posterior pole (a pigmented chorioretinal lesion is an incidental finding). E, F. Progressive cone dystrophy. The typical bull's eye maculopathy is associated with a golden orange reflex. Author: Robert E.

Anderson Publisher: Springer Science & Business Media ISBN: Size: MB Format: PDF, Docs Category: Medical Languages: en Pages: View: Get Book. Book Description: During the last few years, an explosion of infonnation has come from human genetics and molecular and cell biological studies as to the genetic basis for a number of fonns of inherited.

The Hereditary Dystrophies of the Posterior Pole of the Eye. Assen: Van Gorcum; Kinnick TR, Mullins RF, Dev S, Leys M, Mackey DA, Kay CN, et al. Autosomal recessive vitelliform macular dystrophy in a large cohort of vitelliform macular dystrophy patients.

Retina. Mar. 31(3) Falls HF. The symptoms of corneal dystrophies result from the accumulation of abnormal material within the cornea, the clear outer layer of the eye. The cornea serves two functions; it protects the rest of the eye from dust, germs and other harmful or irritating material, and it acts as the eye’s outermost lens, bending incoming light onto the inner lens, where the light is then directed to the retina.

The inherited macular dystrophies comprise a heterogeneous group of disorders characterised by central visual loss and atrophy of the macula and underlying retinal pigment epithelium (RPE). The different forms of macular degeneration encompass a wide range of clinical, psychophysical and histological findings.

The complexity of the molecular basis of monogenic macular disease is now. Accumulation of material at the level of the RPE has been observed in various hereditary retinal dystrophies, eg, Best disease, 1 Stargardt disease, 2 and dominant radial drusen.

8 To the best of my knowledge, gray, serpentine-like deposits have not been reported previously. The clinical and functional findings of a novel form of cone-rod dystrophy with serpentine-like deposits at the posterior pole are. Deutman A.

The hereditary dystrophies of the posterior ‘pole. Assen Van Gorcum. – Deutman AF. Electro-oculography in families with vitelliform dystrophy of the fovea.

Detection of the carrier state. Arch Ophthalmol. ; – Krill AE, Morse PA, Potts AM, Klien BA. Hereditary vitelliruptive macular degeneration. Purpose: To describe a case of extensive macular atrophy and pseudodrusen complicated by bilateral choroidal neovascularization (CNV). Methods: A year-old woman showed extensive macular atrophy at the posterior pole associated with disciform scar in the right eye and fibrotic juxtafoveal CNV in the left eye.

Results: The patient underwent a complete ophthalmological examination including. Corneal dystrophies were commonly subdivided depending on its specific location within the cornea into anterior, stromal, or posterior according to the layer of the cornea affected by the dystrophy.

In the ICD3 classification was published. and has classified disease into four groups as follows: Epithelial and subepithelial dystrophies.

MACULAR DYSTROPHIES 1. HEREDITARY MACULAR DYSTROPHIES MODERATOR- DR VIVEKANAND J 2. Anatomical landmarks The macula is a round area at the posterior pole, lying inside the temporal vascular arcades. It measures between 5 and 6 mm in diameter, and subserves the central 15–20° of the visual field.

KEARNS AND HOLLENHORST 1 introduced the term bull's-eye maculopathy (BEM) in to describe the characteristic clinical appearance of chloroquine retinopathy.

InDeutman 2 used the term to describe similar lesions in patients with inherited retinal dystrophies initially characterized by a central red spot surrounded by a ring of atrophic pigment epithelium or pigment epithelial mottling.

Fig. Color fundus photograph from the right eye of a patient with an advanced stage of diffuse choroidal dystrophy shows diffuse retinal pigment epithelium and choroidal atrophy.

Both the posterior pole and the retinal periphery are involved to varying degrees. A: Posterior pole view of the right eye of a year-old Caucasian male illustrating macrocystic macular changes in the characteristic spoke-wheel schisis pattern of X-linked retinoschisis, due in.

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Methods: Detailed ophthalmic and genetic testing of the patient and his father were s: We report on a year-old male patient with advanced chorioretinal dystrophy at the posterior pole extending into the peripheral retina.

His sister and his father were similarly affected with nyctalopia and decreased visual acuity, although. Corneal dystrophies are a group of rare genetic eye disorders.

With corneal dystrophies, abnormal material builds up in the cornea (the clear, front window of the eye). Most corneal dystrophies affect both eyes. They progress slowly and run in families. The cornea has five layers: Epithelium: the outermost, protective layer of the cornea.

Macular dystrophies affect the posterior pole, resulting in impaired central vision but unaffected peripheral vision. are rare conditions that are identical to each other and characterized by drusen in the posterior pole of the eye, Genetic testing for retinal dystrophies and dysfunctions: benefits, dilemmas and solutions.

Clin. In the United States, cataracts affect more than 24 million adults over 40 years (National Eye Institute, ). According to the American Academy of Ophthalmology (), cataract surgery has a substantial beneficial impact on visual function and on quality of life.2 days ago  Onset of symptoms (3 to 50 years of age) and best-corrected visual acuity (–) were highly variable.

Ophthalmoscopic and retinal imaging defined five phenotypes. Phenotype I presented with single or confluent yellow lesions at the posterior pole and midperiphery, serous retinal detachment, and intraretinal cystoid spaces.1 day ago  A dvances in retinal imaging modalities allow us to visualize posterior segment findings to a previously unmet degree.

The clinical utility of these imaging techniques is constantly expanding, with new developments in machinery allowing for improvements in patient care.