Presentation
Congenital coloboma of the choroid in which the abnormality is transmitted as irregularly dominant was present only in the mother. [ijo.in]
Essentially, he thus had already described the present-day paradigm of the clinical course from drusen to late AMD. [nature.com]
Users should cite the Phenotype Library in all publications, presentations and reports as follows: "HDR UK Phenotype Library phenotypes.healthdatagateway.org" [phenotypes.healthdatagateway.org]
Present even when both eyes are uncovered. Stroke, cerebrovascular accident. Pathologic condition. [emc-eyes.com]
Entire Body System
- Falling
Johnson and Falls (1949). The first case of this syndrome was described by v. Meekeren (1682) in a Spaniard. Since then many cases have been recorded particularly in dermatological literature. [ijo.in]
Side vision; vision elicited by stimuli falling on retinal areas distant from the macula. Pinguecula (pin-GWEK-yu-luh). Anatomic defect. [emc-eyes.com]
Skin
- Macula
Exudative senile macular retinal degeneration Junius-Kuhnt degeneration Exudative age-related macular degeneration Disciform macular degeneration Wet senile macular retinal degeneration Wet senile macular degeneration Subretinal neovascularisation of macula [findacode.com]
Junius-Kuhnt Syndrome (Kuhnt-Junius Disease; Macular Senile Disciform Degeneration [I]; Macula Lutea Juvenile Degeneration [2]) 555e47f 555e47f 555e47f 555e47f 555e47f 6 General: Onset in advanced age or in juvenile period; etiology unknown; possible [esanatos.com]
H3533 황반의 낭, 구멍 또는 거짓구멍 Macular cyst, hole, or pseudohole H3534 낭성 황반변성 Cyst of macula H3535 황반의 드루젠(퇴행성) Drusen (degenerative) of macula H3536 황반의 주름 Puckering of macula H3537 독성 황반병증 Toxic maculopathy H3539 상세불명의 황반변성(연령과 관계된) Unspecified macular degeneration [druginfo.co.kr]
The macula lutea, a portion of the retina, is the only part of the retina that can achieve very sharp focus. This is possible due to the presence of a high concentration of cone cells in the macula lutea. [zien.be]
[Junius' juvenile macular exudative retinitis, juvenile disciform degeneration of the macula; clinical and pathogenic studies]. SCUDERI G. SCUDERI G. Boll Ocul. 1953 Aug;32(8):463-88. Boll Ocul. 1953. PMID: 13105869 Undetermined Language. [pubmed.ncbi.nlm.nih.gov]
Eyes
- Visual Impairment
This in turn will be key to developing effective and safe treatments for individuals affected by progressive visual impairment caused by this disease. [ophthalmologyscience.org]
Identifying the content area for the 51‐item National Eye Institute Visual Function Questionnaire: results from focus groups with visually impaired persons. Archives of Ophthalmology 1998;116(2):227‐33. [cochranelibrary.com]
- Scotoma
Ocular: Impairment of central vision; central scotoma; atrophic macular degeneration surrounded by retinal hemorrhages, resulting in mountlike lesion; exudative and atrophic reaction with deposit in and about macula. Clinical: None. Deutman AF. [esanatos.com]
Scotoma size and reading speed in patients with subfoveal occult choroidal neovascularization in age‐related macular degeneration. Ophthalmology 2003;110(1):65‐9. Enlace al artículo Google Scholar Eyetech Study Group. [cochranelibrary.com]
- Retinal Hemorrhage
Ocular: Impairment of central vision; central scotoma; atrophic macular degeneration surrounded by retinal hemorrhages, resulting in mountlike lesion; exudative and atrophic reaction with deposit in and about macula. Clinical: None. Deutman AF. [esanatos.com]
In the active stage, findings include dilated, tortuous peripheral blood vessels, retinal hemorrhages and abnormal newly formed blood vessels (neovascularization). [emc-eyes.com]
- Central Scotoma
Ocular: Impairment of central vision; central scotoma; atrophic macular degeneration surrounded by retinal hemorrhages, resulting in mountlike lesion; exudative and atrophic reaction with deposit in and about macula. Clinical: None. Deutman AF. [esanatos.com]
- Visual Acuity Decreased
Often, though, it is not possible to improve the lost visual acuity. AMD is a chronic, progressive disorder. A course of treatment is considered successful if the visual acuity decreases less rapidly than it would without treatment. [zien.be]
Workup
Visual Field Test
- Central Scotoma
Ocular: Impairment of central vision; central scotoma; atrophic macular degeneration surrounded by retinal hemorrhages, resulting in mountlike lesion; exudative and atrophic reaction with deposit in and about macula. Clinical: None. Deutman AF. [esanatos.com]
Treatment
[New examination methods for macular disorders--application of diagnosis and treatment]. Yoshida A. Yoshida A. Nippon Ganka Gakkai Zasshi. 2000 Dec;104(12):899-942. Nippon Ganka Gakkai Zasshi. 2000. PMID: 11193944 Review. Japanese. MeSH terms [pubmed.ncbi.nlm.nih.gov]
[…] naïve Neovascular Age-related Macular Degeneration (nAMD) Patients Completed NCT01988662 Phase 4 29 Single or Combined Protocols for the Treatment of Patients With Neovascular Age-related Macular Degeneration: Compliance, Risk/Benefit and Cost/Benefit [malacards.org]
A course of treatment is considered successful if the visual acuity decreases less rapidly than it would without treatment. Lucentis, Avastin or Eylea etc. are administered in injection form in the eye. [zien.be]
Direct treatment of the entire lesion Observation MPS Subf. [cochranelibrary.com]
Etiology
Junius-Kuhnt Syndrome (Kuhnt-Junius Disease; Macular Senile Disciform Degeneration [I]; Macula Lutea Juvenile Degeneration [2]) 555e47f 555e47f 555e47f 555e47f 555e47f 6 General: Onset in advanced age or in juvenile period; etiology unknown; possible [esanatos.com]
Epidemiology
The International AMD Epidemiological Study Group. An international classification and Grading System for Age‐related maculopathy and Age‐related macular degeneration. Survey of Ophthalmology 1995;39(5):367‐74. [cochranelibrary.com]
Pathophysiology
Only those that seem relevant, either because of their widespread use or the pathophysiologic concept they indicate, will be commented on. [nature.com]
Prevention
Opacity or cloudiness of the crystalline lens, which may prevent a clear image from forming on the retina. [emc-eyes.com]
This 2RT treatment can prevent, or postpone for many years, the progression of dry AMD to wet AMD. However, the effects of the 2RT laser are not permanent. It is likely that the treatment will have to be repeated once every 6 to 12 months. [zien.be]
Financial support – Novartis, Roche/Genentech; Nonfinancial support – Heidelberg Engineering Supported in part by Research to Prevent Blindness, Inc., New York, New York (unrestricted grant to the Department of Ophthalmology & Visual Sciences, University [ophthalmologyscience.org]
Notes Funding: grant from the Wilmer Retinal Vascular Center Macular Research Fund (Baltimore, MD); unrestricted grant from the Research to Prevent Blindness (New York, NY). [cochranelibrary.com]