Branchiootorenal syndrome

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Branchiootorenal syndrome (pronounced: bran-kee-oh-oh-toh-REN-al sin-drome), also known as BOR syndrome or Melnick-Fraser syndrome, is a rare genetic disorder that typically affects the development of the ear, neck, and kidney. The term "branchiootorenal" is derived from the Greek words "branchia" (gills), "oto" (ear), and "renal" (kidney).

Etymology

The term "branchiootorenal" is derived from the Greek words "branchia" (gills), "oto" (ear), and "renal" (kidney). This reflects the areas of the body that are typically affected by the syndrome.

Symptoms

The symptoms of Branchiootorenal syndrome can vary greatly from person to person. However, common symptoms often include hearing loss, kidney abnormalities, and branchial clefts or branchial fistulas on the neck.

Causes

Branchiootorenal syndrome is caused by mutations in the EYA1, SIX1, or SIX5 genes. These genes are involved in early development, particularly of the ear, neck, and kidneys.

Diagnosis

Diagnosis of Branchiootorenal syndrome is typically based on the presence of characteristic signs and symptoms. Genetic testing can confirm the diagnosis.

Treatment

Treatment for Branchiootorenal syndrome is typically supportive and based on the signs and symptoms present in each person. This may include hearing aids for hearing loss, surgery for branchial clefts or fistulas, and treatment for kidney abnormalities.

See also

References


External links

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