Dent disease

Last revised by Rohit Sharma on 18 Nov 2021

Dent disease is a very rare inherited renal disorder that is characterized by proximal tubule dysfunction.

Dent disease is X-linked recessive and thus is seen predominantly in males. There are two types 1-4:

  • type 1: caused by the CLCN5 gene mutation
  • type 2: caused by the OCRL1 gene mutation, thus there is an association with Lowe syndrome

The condition is named after Charles Enrique Dent (1911-1976) who described the first case in 1964 6.

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