- Kayser-Fleischer ring
Infobox_Disease
Name = Kayser-Fleischer ring
Caption = A Kayser-Fleischer ring in a 32-year-old patient who had longstanding speech difficulties andtremor .
DiseasesDB = 30056
ICD10 = ICD10|H|18|0|h|15
ICD9 = ICD9|371.14
ICDO =
OMIM =
MedlinePlus =
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MeshNumber =Kayser-Fleischer rings (KF rings) are dark rings that appear to encircle the iris of the
eye . They are due tocopper deposition as a result of particular liver diseases. They are named after Dr Bernhard Kayser and Dr Bruno Fleischer, the German doctors who first described them in 1902 and 1903. [cite journal | author=Kayser B | title=Über einen Fall von angeborener grünlicher Verfärbung des Cornea | journal=Klin Monatsbl Augenheilk | year=1902 | volume=40 | issue=2 | pages=22–25] [cite journal | author=Fleischer B | title=Zwei weitere Fälle von grünlicher Verfärbung der Kornea | journal=Klin Monatsbl Augenheilk | year=1903 | volume=41 | issue=1 | pages=489–491] [WhoNamedIt|synd|1758] Initially thought to be due to the accumulation of silver, they were first demonstrated to contain copper in 1934. [cite journal | author=Gerlach W, Rohrschneider W | title=Besteht das Pigment des Kayser-Fleischerschen Hornhautringes aus Silber? | journal=Klin Wochenschr | year=1934 | volume=13 | pages=48–49 | doi=10.1007/BF01799043]Detection
As Kayser-Fleischer rings do not cause any symptoms, it is common for them to be identified during investigations for other medical conditions. In certain situations, they are actively sought; in that case, the early stages may be detected by
slit lamp examination before they become visible to the naked eye.cite journal |author=McDonnell G, Esmonde T |title=A homesick student |journal=Postgrad Med J |volume=75 |issue=884 |pages=375–8 |year=1999 |pmid=10435182 | url=http://pmj.bmj.com/cgi/content/full/75/884/375]Appearance
The rings, which consist of
copper deposits where thecornea meets thesclera , inDescemet's membrane , first appear as a crescent at the top of the cornea. Eventually, a second crescent forms below, at 6 o'clock, and ultimately completely encircles the cornea. [GPnotebook|-1684406263]Associations
Kayser-Fleischer rings are a sign of
Wilson's disease , which involves abnormal copper handling by the liver resulting in copper accumulation in the body and is characterised by abnormalities of thebasal ganglia of thebrain , liver cirrhosis,splenomegaly , involuntary movements,muscle rigidity , psychiatric disturbances,dystonia anddysphagia . The combination of neurological symptoms, a low bloodceruloplasmin level and KF rings is diagnostic of Wilson's disease.Other causes of KF rings are
cholestasis (obstruction of thebile duct s),primary biliary cirrhosis and "cryptogenic" cirrhosis (cirrhosis in which no cause can be identified).References
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