alexa Maternally Inherited Diabetes and Deafness (MIDD) with Undetectable CPeptide Level and Cerebellar Atrophy
ISSN: 2165-7920

Journal of Clinical Case Reports
Open Access

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Case Report

Maternally Inherited Diabetes and Deafness (MIDD) with Undetectable CPeptide Level and Cerebellar Atrophy

Yoshifumi Saisho* and Jun Inaishi
Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan
Corresponding Author : Yoshifumi Saisho
Department of Internal Medicine
Keio University School of Medicine
35 Shinanomachi, Shinjuku-ku
Tokyo 160-8582, Japan
Tel: +81-3-3353-1211 (x62383)
Fax: +81-3-3359-2745
E-mail: [email protected]
Received May 06, 2014; Accepted May 26, 2014; Published May 28, 2014
Citation: Saisho Y and Inaishi J (2014) Maternally Inherited Diabetes and Deafness (MIDD) with Undetectable C-Peptide Level and Cerebellar Atrophy. J Clin Case Rep 4:366 doi:10.4172/2165-7920.1000366
Copyright: © 2014 Saisho Y et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
 

Abstract

Maternally inherited diabetes and deafness (MIDD), also called mitochondrial diabetes mellitus, is a rare form of diabetes that comprises 0.5-2.8% of the diabetic population. Most cases of MIDD are associated with a point mutation in the mitochondrial DNA (mtDNA) at position 3243 of the leucine tRNA gene (A3243G) . Patients with MIDD are characterized by 1) young onset of diabetes, 2) absence of obesity, 3) neurosensory hearing loss, 4) maternal family history of diabetes and 5) progressive insulin secretory defect. In most cases of MIDD, diabetes is non-insulin dependent at onset, but progresses to require insulin therapy thereafter. However, to our knowledge, few cases with MIDD show complete loss of C-peptide level during the course of the disease

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