| 词条 | Epithelial basement membrane dystrophy |
| 释义 |
Epithelial basement membrane dystrophy (EBMD), also known as map-dot-fingerprint dystrophy and Cogans's microcystic dystrophy, is a disorder of the eye that can cause pain and dryness. It is sometimes included in the group of corneal dystrophies.[1] It diverges from the formal definition of corneal dystrophy in being in most cases non-familial. It also has a fluctuating course, while for a typical corneal dystrophy the course is progressive. When it is considered part of this group, it is the most common type of corneal dystrophy.[2] Signs and symptomsPatients may complain of severe problems with dry eyes, or with visual obscurations.[3] It can also be asymptomatic, and only discovered because of subtle lines and marks seen during an eye exam.[2] EBMD is a bilateral anterior corneal dystrophy characterized by grayish epithelial fingerprint lines, geographic map-like lines, and dots (or microcysts) on slit-lamp examination. Findings are variable and can change with time. While the disorder is usually asymptomatic, up to 10% of patients may have recurrent corneal erosions, usually beginning after age 30; conversely, 50% of patients presenting with idiopathic recurrent erosions have evidence of this dystrophy.[4] PathophysiologyIn some families autosomal dominant inheritance and point mutations in the TGFBI gene encoding keratoepithelin have been identified,[5] but according to the International Committee for Classification of Corneal Diseases (IC3D)[6] the available data still does not merit a confident inclusion of EBMD in the group of corneal dystrophies. In view of this, the more accurate designation of the disease is possibly not dystrophy but corneal degeneration.[7] The main pathological feature of the disease is thickened, multilaminar and disfigured basement membrane of corneal epithelium. The change in the structure affects the epithelium, some cells of which may become entrapped in the rugged membrane and fail to migrate to the surface where they should undergo desquamation. TreatmentPhototherapeutic keratectomy (PTK) done by an ophthalmologist can restore and preserve useful visual function for a significant period of time in patients with anterior corneal dystrophies including EBMD.[4]if the erosion is not clearing with medication the ophthalmologist can prick it with a needle multiple times to try and stick it back down (no pain). Alternately, they will scrape all the skin left on your eyeball off so that it reforms from scratch. See also
References1. ^{{Citation|author=Online Mendelian Inheritance in Man |title=#121820: Corneal dystrophy, epithelial basement membrane; EBMD |url=http://www.omim.org/entry/121820 |postscript=. }}{{dead link|date=December 2016 |bot=InternetArchiveBot |fix-attempted=yes }} {{Human corneal dystrophy}}2. ^1 {{Cite book|title = Dry Eye: A Practical Approach|url = https://books.google.com/books?id=92O4BgAAQBAJ|publisher = Springer|date = 2015-02-18|isbn = 9783662441060|language = en|first = Colin|last = Chan|pages = 111–112}} 3. ^{{cite web |url=https://www.reviewofoptometry.com/article/when-should-you-treat-ebmd-with-ptk |title=When Should You Treat EBMD with PTK? |author= John R. Martinelli, O.D. |date=22 March 2010 |website= Review of Optometry |access-date=16 March 2017}} 4. ^1 {{OMIM|121820}} 5. ^{{cite journal |vauthors=Boutboul S, Black GC, Moore JE, Sinton J, Menasche M, Munier FL, Laroche L, Abitbol M, Schorderet DF |title=A subset of patients with epithelial basement membrane corneal dystrophy have mutations in TGFBI/BIGH3 |journal=Hum. Mutat. |volume=27 |issue=6 |pages=553–7 |date=June 2006 |pmid=16652336 |doi=10.1002/humu.20331}} 6. ^{{cite journal |vauthors=Weiss JS, Møller HU, Lisch W, Kinoshita S, Aldave AJ, Belin MW, Kivelä T, Busin M, Munier FL, Seitz B, Sutphin J, Bredrup C, Mannis MJ, Rapuano CJ, Van Rij G, Kim EK, Klintworth GK |title=The IC3D classification of the corneal dystrophies |journal=Cornea |volume=27 |issue= Suppl 2 |pages=S1–83 |date=December 2008 |pmid=19337156 |pmc=2866169 |doi=10.1097/ICO.0b013e31817780fb |url=http://meta.wkhealth.com/pt/pt-core/template-journal/lwwgateway/media/landingpage.htm?issn=0277-3740&volume=27&issue=&spage=S1}} 7. ^{{cite web|author=David Verdier|url=http://emedicine.medscape.com/article/1193945-overview |title=Map-dot-fingerprint Dystrophy|publisher=eMedicine}} 1 : Disorders of sclera and cornea |
| 随便看 |
|
开放百科全书收录14589846条英语、德语、日语等多语种百科知识，基本涵盖了大多数领域的百科知识，是一部内容自由、开放的电子版国际百科全书。