title: Maternally-inherited diabetes with deafness (MIDD) and hyporeninemic hypoaldosteronism.
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.
authors.affiliation: Escola Paulista de Medicina, Universidade Federal de São Paulo, São Paulo, SP, Brazil.
authors.forename: Patricia B
authors.initials: PB
authors.lastname: Mory
authors.forename: Marcia C dos
authors.initials: MC
authors.lastname: Santos
authors.forename: Claudio E
authors.initials: CE
authors.lastname: Kater
authors.forename: Regina S
authors.initials: RS
authors.lastname: Moisés
mesh_terms: D004272:DNA, Mitochondrial / Q000235:genetics*; D003638:Deafness / Q000235:genetics*; D003924:Diabetes Mellitus, Type 2 / Q000235:genetics*; D006801:Humans; D006994:Hypoaldosteronism / Q000235:genetics*; D008297:Male; D008875:Middle Aged; D028361:Mitochondrial Diseases; D010375:Pedigree; D017354:Point Mutation / Q000235:genetics*
chemical_list: D004272:DNA, Mitochondrial