Keratoendotheliitis Fugax Hereditaria (KEFH) is a rare genetic disorder that affects the corneal endothelium, which is the innermost layer of the cornea. This article will explore the causes, symptoms, and treatments of KEFH.
KEFH is a rare genetic disorder that affects the corneal endothelium. The corneal endothelium is a single layer of cells that line the inner surface of the cornea, which is the transparent front part of the eye. The corneal endothelium helps to maintain the clarity of the cornea by regulating the amount of fluid that enters and leaves it.
KEFH is caused by a genetic mutation that affects the function of the corneal endothelial cells, leading to recurrent episodes of corneal edema. It is an autosomal dominant genetic disorder, which means that it is caused by a mutation in a single copy of the gene inherited from one parent. The exact gene involved in KEFH is not yet known.
The symptoms of KEFH are characterized by recurrent episodes of corneal edema, which is the swelling of the cornea due to the accumulation of fluid. These episodes can last from a few hours to a few days and usually resolve spontaneously. The symptoms of KEFH may include:
There is currently no cure for KEFH, but the symptoms can be managed with various treatment options. The treatment options for KEFH include:
Since KEFH is a genetic disorder, there is no surefire way to prevent it. However, genetic counseling and testing can be useful for individuals with a family history of the disorder who are planning to have children.
KEFH is a rare genetic disorder that affects the corneal endothelium and leads to recurrent episodes of corneal edema. The symptoms of KEFH can be managed with various treatment options, including hypertonic saline drops, miotics, and corneal transplant. Genetic counseling and testing may be useful for individuals with a family history of KEFH who are planning to have children.
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