Maternally-inherited diabetes with deafness (MIDD) and hyporeninemic hypoaldosteronism

Arq Bras Endocrinol Metabol. 2012 Nov;56(8):574-7. doi: 10.1590/s0004-27302012000800019.

Abstract

Maternally-inherited diabetes with deafness (MIDD) is a rare form of monogenic diabetes that results, in most cases, from an A-to-G transition at position 3243 of mitochondrial DNA (m.3243A>G) in the mitochondrial-encoded tRNA leucine (UUA/G) gene. As the name suggests, this condition is characterized by maternally-inherited diabetes and bilateral neurosensory hearing impairment. A characteristic of mitochondrial cytopathies is the progressive multisystemic involvement with the development of more symptoms during the course of the disease. We report here the case of a patient with MIDD who developed hyporeninemic hypoaldosteronism.

Publication types

  • Case Reports

MeSH terms

  • DNA, Mitochondrial / genetics*
  • Deafness / genetics*
  • Diabetes Mellitus, Type 2 / genetics*
  • Humans
  • Hypoaldosteronism / genetics*
  • Male
  • Middle Aged
  • Mitochondrial Diseases
  • Pedigree
  • Point Mutation / genetics*

Substances

  • DNA, Mitochondrial

Supplementary concepts

  • Noninsulin-dependent diabetes mellitus with deafness