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Maturity-Onset Diabetes of the Young Type 10


Presentation

  • An Atypical Presentation of Diabetes: Maturity Onset Diabetes of the Young (MODY) We present a lean 36 year old We present a lean 36 year old male presenting with impaired fasting glucose since his early twenties being treated with diet alone, with further[professional.diabetes.org]
  • She did not present with typical diabetes mellitus (DM) symptoms, however she had a three generations family history positive for insulin-dependent DM. She presented with a good clinical condition.[omicsonline.org]
  • We present the cases of three children and their families diagnosed with MODY over the past two years.[jdmdonline.biomedcentral.com]
  • Acronym MODY10 Synonyms MODY-10 MODY type 10 Keywords Disclaimer Any medical or genetic information present in this entry is provided for research, educational and informational purposes only.[uniprot.org]
  • My Taber's Cyclopedic Medical Dictionary, on the other hand, defines MODY as type 2 DM that presents during childhood or adolescence. I can't find an entry for it in the ICD-9 index.[aapc.com]
Intestinal Atresia
  • atresia, and gallbladder aplasia or hypoplasia, with or without tracheoesophageal fistula, Martinez-Frias syndrome, Mitchell-Riley syndrome AR 10 31 Non-coding variants covered by MODY Panel Gene Genomic location HG19 HGVS RefSeq RS-number ABCC8 Chr11[blueprintgenetics.com]
Oligomenorrhea
  • Women with oligomenorrhea and polycystic ovaries show a high incidence of ovulation failure that is linked to insulin resistance and related metabolic features.[omicsonline.org]
Onset around Puberty
  • around puberty (MODY 5) Insulin resistance wide clinical spectrum Insulin resistance without obesity /- Urogenital/pancreatic anomalies Insulin dependence /- Pancreatic exocrine failure /- Developmental delay/Learning difficulties The Southern District[jdmdonline.biomedcentral.com]

Treatment

  • Transplantation 765 65 Treatment of Diabetes in the Hospitalized Patient 1103 66 The Diabetic Foot Strategies for Treatment 1111 BIOLOGY OF THE COMPLICATIONS OF DIABETES 793 67 Vascular Disease of the Lower Extremities 1123 Cameron M Akbari and Frank[books.google.com]
  • Chapter 9 Metformin 107 Chapter 10 AlphaGlucosidase Inhibitors 121 Chapter 11 Thiazolidinediones 135 Chapter 12 Treatment Strategies for Type 2 Diabetes Based on Incretin Action 159 Chapter 13 Pramlintide Acetate in the Treatment of Type 2 Diabetes 171[books.google.com]
  • Due to positive effect of metformin treatment on the regulation of menstrual cycles and still high level of C-peptide we decided to continue with the treatment.[omicsonline.org]
  • Therefore, controversy exists as to whether such “treatment-exempt” patients should be reassessed for treatment later in life.[mja.com.au]
  • You don’t need any treatment for glucokinase MODY.[diabetes.org.uk]

Prognosis

  • Genetic testing is relevant for treatment and prognosis in MODY patients.[thieme-connect.com]
  • [br]Following the confirmation of the diagnosis we were able to stop insulin therapy and to proceed with dietary advice alone and reassure the patient regarding the prognosis of his disease.[professional.diabetes.org]
  • Making a specific diagnosis of MODY can have important implications for the guidance of appropriate treatment, prognosis and genetic counselling.[jdmdonline.biomedcentral.com]
  • Subsequent analysis confirmed that GCK-MODY does indeed have a good long term prognosis, mainly because the metabolic defect is mild.[diapedia.org]
  • Credit: University of Western Australia Distinguishing monogenic diabetes in the young – that is, a rare type of diabetes caused by a single genetic mutation relatively early in life—from other types of diabetes, is crucial for appropriate therapies, prognosis[medicalxpress.com]

Etiology

  • References Owen KR, Stride A, Ellard S, Hattersley AT: Etiological investigation of diabetes in young adults presenting with apparent type 2 diabetes.[care.diabetesjournals.org]
  • However, diagnosis allows appropriate individualized care, depending on the genetic etiology, and allows prognostication in family members.[dovepress.com]
  • At least 13 maturity onset diabetes of the young (MODY) subtypes with distinct genetic etiologies have been identified till now.[cyberleninka.ru]
  • Acrorenal field defect, ectodermal dysplasia, and lipoatrophic diabetes Agenesis of cerebellum Atypical diabetes mellitus Congenital hypoplasia of pancreas Diabetes mellitus associated with genetic syndrome Diabetes mellitus associated with hormonal etiology[icdlist.com]
  • Etiology Monogenic diabetes is a form of diabetes caused by a mutation in one of several genes. It is often misdiagnosed as DM1 or DM2. Accurate diagnosis is essential for determining appropriate treatment.[uspharmacist.com]

Epidemiology

  • The concept and the epidemiology of diabetic nephropathy have changed in tecent years. J Clin Med. 2015;4:1207-16. [ Links ] 5. Gregg EW, Li Y, Wang J, Burrows NR, Ali MK, Rolka D, et al.[scielo.isciii.es]
  • Pruhova, S., Ek, J., Lebl, J., Sumnik, Z., Saudek, F., Andel, M., Pedersen, O. and Hansen, T. (2003) Genetic epidemiology of MODY in the Czech Republic: New mutations in the MODY genes HNF 4α, GCK and HNF 1α. Diabetologia, 46, 291-295.[file.scirp.org]
  • Epidemiology [ edit ] According to data from Saxony, Germany, MODY was responsible for 2.4% of diabetes incidence in children younger than 15 years. [21] History [ edit ] The term MODY dates back to 1964, when diabetes mellitus was considered to have[en.wikipedia.org]
  • Epidemiology The increasing prevalence of diabetes worldwide has led to a situation where approximately 360 million people had diabetes in 2011, of whom more than 95% would have had type 2 diabetes.[patient.info]
  • When these genes are altered, beta cells may exhibit impaired growth, differentiation, and renewal. 1 Epidemiology Monogenic diabetes is an autosomal-dominant disease.[uspharmacist.com]
Sex distribution
Age distribution

Pathophysiology

  • Diabetes 52 : 1128 –1136, 2003 Fajans SS, Bell GI, Polonsky KS: Molecular mechanisms and clinical pathophysiology of maturity-onset diabetes of the young.[care.diabetesjournals.org]
  • Pathophysiology [ edit ] The recognised forms of MODY are all due to ineffective insulin production or release by pancreatic beta cells. Several of the defects are mutations of transcription factor genes.[en.wikipedia.org]
  • ., Bell, G.I. and Polonsky, K.S. (2001) Molecular mechanisms and clinical pathophysiology of maturity onset diabetes of the young. New England Journal of Medicine, 345, 971-980.[file.scirp.org]
  • New Zealand (4) School of Biological Sciences, University of Auckland, Auckland, New Zealand (5) Department of Endocrinology, Dunedin Public Hospital, Dunedin, New Zealand References Fajans SS, Bell GI, Polonsky KS: Molecular mechanisms and clinical pathophysiology[jdmdonline.biomedcentral.com]
  • References Fajans SS, Bell GI, Polonsky KS (2001) Molecular mechanisms and clinical pathophysiology of maturity-onset diabetes of the young. N Engl J Med 345: 971-980.[omicsonline.org]

Prevention

  • Complications are rare in cases of MODY 2 as compared to MODY 3, where aggresive treatment is required in the long-term to prevent the occurence of diabetic vascular complications.[professional.diabetes.org]
  • Vaccination may have a future role in the prevention of type 1 diabetes. [ 13 ] Type 2: see the separate Prevention of Type 2 Diabetes article.[patient.info]
  • Blood samples need to be taken on ice and processed immediately to prevent degradation by blood peptidases, which limits testing to a hospital setting with on-site laboratory facilities .[diapedia.org]
  • "Research is urgently needed to characterise the families of young patients with diabetes , as well as effective methods for screening for and preventing disease," they noted.[medicalxpress.com]

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