Maternally inherited diabetes and deafness (MIDD): Diagnosis and management

Naing, A.; Kenchaiah, Manohar; Krishnan, Binu; Mir, Farheen; Charnley, Amanda; Egan, Catherine; Bano, Gul

doi:10.1016/j.jdiacomp.2014.03.006

Cited by 57 publications

(39 citation statements)

References 23 publications

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“…Microvascular complications out of keeping with duration of diabetes are often observed. Nearly 46% of MIDD cases progress to require insulin within 10 years of treatment [17]. MIDD can be distinguished from MODY by the presence of maternal transmission in conjunction with hearing impairment or macular dystrophy.…”

Section: Monogenic Diabetesmentioning

confidence: 99%

Diabetes mellitus, a complex and heterogeneous disease, and the role of insulin resistance as a determinant of diabetic kidney disease

Karalliedde

Gnudi

2014

Nephrol. Dial. Transplant.

121

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A B S T R AC TDiabetes mellitus (DM) is increasingly recognized as a heterogeneous condition. The individualization of care and treatment necessitates an understanding of the individual patient's pathophysiology of DM that underpins their DM classification and clinical presentation. Classical type-2 diabetes mellitus is due to a combination of insulin resistance and an insulin secretory defect. Type-1 diabetes is characterized by a nearabsolute deficiency of insulin secretion. More recently, advances in genetics and a better appreciation of the atypical features of DM has resulted in more categories of diabetes. In the context of kidney disease, patients with DM and microalbuminuria are more insulin resistant, and insulin resistance may be a pathway that results in accelerated progression of diabetic kidney disease. This review summarizes the updated classification of DM, including more rarer categories and their associated renal manifestations that need to be considered in patients who present with atypical features. The benefits and limitations of the tests utilized to make a diagnosis of DM are discussed. We also review the putative pathways and mechanisms by which insulin resistance drives the progression of diabetic kidney disease.

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Section: Monogenic Diabetesmentioning

confidence: 99%

Diabetes mellitus, a complex and heterogeneous disease, and the role of insulin resistance as a determinant of diabetic kidney disease

Karalliedde

Gnudi

2014

Nephrol. Dial. Transplant.

121

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“…In fact, the treatment with PIO for three months improved the glucose tolerance and the insulin sensitivity, as evaluated by the ITT. Biguanide is another potent insulin sensitizer, but we did not use it in this case because of the risk of lactic acidosis (4,6,7,25). The precise mechanism underlying the insulin-sensitizing effects of PIO in this case remains unclear.…”

Section: Discussionmentioning

confidence: 99%

“…A reduced level of insulin secretion has been considered to be a major feature of mitochondrial diabetes (2)(3)(4)(5). The 3243 A>G mutation in mtDNA reduces the level of ATP generation because of the disruption of the electron transport system (ETS), and the altered ATP to ADP ratio may result in impaired insulin secretion, which can lead to beta-cell apoptosis (6)(7)(8). A decreased insulin sensitivity is also observed in mitochondrial diabetes (9,10).…”

Section: Introductionmentioning

confidence: 99%

Treatment of Mitochondrial Diabetes with a Peroxisome Proliferator-activated Receptor (PPAR)-gamma Agonist

et al. 2016

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The 3243 A>G mutation in mitochondrial DNA is the most common cause of monogenic diabetes mellitus in Japan. A 45-year-old woman with mitochondrial diabetes and significant insulin resistance presented with hypoadiponectinemia despite a normal amount of visceral fat. Three months of treatment with pioglitazone (PIO) improved her blood glucose profile and response to the 75-g oral glucose tolerance test. These changes were accompanied by the amelioration of her insulin resistance and the impairment of early-phase insulin secretion. Her serum adiponectin levels increased to the normal range. In this case of mitochondrial diabetes, PIO was effective for glycemic control.

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“…Maternally inherited diabetes and deafness (MIDD) is a mitochondrial disorder causing a syndromic form of diabetes accompanied by sensorineural hearing loss and some cases include renal problems, pigmentary retinopathy, ptosis, myopathy, cardiomyopathy and/or neuro-psychiatric symptoms (OMIM: 520000) [27,28]. Mutations in MT-TL1, MT-TK or MT-TE mitochondrial genes coding for mtRNAs, which participate in the protein production in mitochondria and impair their functioning had been linked in MIDD [29].…”

Section: Mitochondrial-linked Shlmentioning

confidence: 99%

Genetics and Acquired Hearing Loss

Alkowari¹,

Guarch²

2019

Geriatric Medicine and Gerontology

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Hearing loss (HL) is a worldwide disease with substantial economic costs for the public health. Around 466 million people have disabling hearing loss and the WHO estimated that by 2050 over 900 million people will suffer hearing loss. Several factors including infections, noise-exposure, ototoxic medications or genetic disorders could cause hearing impairment. Hearing devices such as cochlear implants and aids are the current therapies. Although the prevalence of hearing loss is very high, alternative treatments as pharmaceutical agents are currently insufficient. Within the past years, increased knowledge on hearing loss etiology and physiopathology opened new opportunities for future research towards hearing loss treatment. Here we aim to review current bibliography on genetics factors involved in hearing loss.

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Maternally inherited diabetes and deafness (MIDD): Diagnosis and management

Cited by 57 publications

References 23 publications

Diabetes mellitus, a complex and heterogeneous disease, and the role of insulin resistance as a determinant of diabetic kidney disease

Diabetes mellitus, a complex and heterogeneous disease, and the role of insulin resistance as a determinant of diabetic kidney disease

Treatment of Mitochondrial Diabetes with a Peroxisome Proliferator-activated Receptor (PPAR)-gamma Agonist

Genetics and Acquired Hearing Loss

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