Literature DB >> 29846255

Monogenic diabetes: the impact of making the right diagnosis.

Anastasia G Harris1, Lisa R Letourneau, Siri Atma W Greeley.   

Abstract

PURPOSE OF REVIEW: Monogenic forms of diabetes have received increased attention and genetic testing is more widely available; however, many patients are still misdiagnosed as having type 1 (T1D) or type 2 diabetes. This review will address updates to monogenic diabetes prevalence, identification, treatment, and genetic testing. RECENT
FINDINGS: The creation of a T1D genetic risk score and the use of noninvasive urinary C-peptide creatinine ratios have provided new tools to aid in the discrimination of possible monogenic diabetes from likely T1D. Early, high-dose sulfonylurea treatment in infants with a KCNJ11 or ABCC8 mutation continues to be well tolerated and effective. As the field moves towards more comprehensive genetic testing methods, there is an increased opportunity to identify novel genetic causes. Genetic testing results continue to allow for personalized treatment but should provide patient information at an appropriate health literacy level.
SUMMARY: Although there have been clinical and genetic advances in monogenic diabetes, patients are still misdiagnosed. Improved insurance coverage of genetic testing is needed. The majority of data on monogenic diabetes has been collected from Caucasian populations, therefore, research studies should endeavor to include broader ethnic and racial diversity to provide comprehensive information for all populations.

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Year:  2018        PMID: 29846255      PMCID: PMC6077987          DOI: 10.1097/MOP.0000000000000643

Source DB:  PubMed          Journal:  Curr Opin Pediatr        ISSN: 1040-8703            Impact factor:   2.856


  73 in total

1.  Hypoglycemia in sulfonylurea-treated KCNJ11-neonatal diabetes: Mild-moderate symptomatic episodes occur infrequently but none involving unconsciousness or seizures.

Authors:  Monica S Lanning; David Carmody; Łukasz Szczerbiński; Lisa R Letourneau; Rochelle N Naylor; Siri Atma W Greeley
Journal:  Pediatr Diabetes       Date:  2017-12-05       Impact factor: 4.866

2.  FOXP3 mutations causing early-onset insulin-requiring diabetes but without other features of immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome.

Authors:  Jessica L Hwang; Soo-Young Park; Honggang Ye; May Sanyoura; Ashley N Pastore; David Carmody; Daniela Del Gaudio; Janna F Wilson; Craig L Hanis; Xiaoming Liu; Gil Atzmon; Benjamin Glaser; Louis H Philipson; Siri Atma W Greeley
Journal:  Pediatr Diabetes       Date:  2017-11-29       Impact factor: 4.866

Review 3.  Insulin therapy in neonatal diabetes mellitus: a review of the literature.

Authors:  Ivana Rabbone; Fabrizio Barbetti; Raffaella Gentilella; Gilberto Mossetto; Riccardo Bonfanti; Claudio Maffeis; Dario Iafusco; Elvira Piccinno
Journal:  Diabetes Res Clin Pract       Date:  2017-04-13       Impact factor: 5.602

4.  Isomers of the TCF1 gene encoding hepatocyte nuclear factor-1 alpha show differential expression in the pancreas and define the relationship between mutation position and clinical phenotype in monogenic diabetes.

Authors:  Lorna W Harries; Sian Ellard; Amanda Stride; Noel G Morgan; Andrew T Hattersley
Journal:  Hum Mol Genet       Date:  2006-06-07       Impact factor: 6.150

5.  A genetic diagnosis of maturity-onset diabetes of the young (MODY): experiences of patients and family members.

Authors:  A R Bosma; T Rigter; S S Weinreich; M C Cornel; L Henneman
Journal:  Diabet Med       Date:  2015-03-28       Impact factor: 4.359

6.  Activating mutations in the gene encoding the ATP-sensitive potassium-channel subunit Kir6.2 and permanent neonatal diabetes.

Authors:  Anna L Gloyn; Ewan R Pearson; Jennifer F Antcliff; Peter Proks; G Jan Bruining; Annabelle S Slingerland; Neville Howard; Shubha Srinivasan; José M C L Silva; Janne Molnes; Emma L Edghill; Timothy M Frayling; I Karen Temple; Deborah Mackay; Julian P H Shield; Zdenek Sumnik; Adrian van Rhijn; Jerry K H Wales; Penelope Clark; Shaun Gorman; Javier Aisenberg; Sian Ellard; Pål R Njølstad; Frances M Ashcroft; Andrew T Hattersley
Journal:  N Engl J Med       Date:  2004-04-29       Impact factor: 91.245

7.  Mutant proinsulin proteins associated with neonatal diabetes are retained in the endoplasmic reticulum and not efficiently secreted.

Authors:  Soo-Young Park; Honggang Ye; Donald F Steiner; Graeme I Bell
Journal:  Biochem Biophys Res Commun       Date:  2009-12-23       Impact factor: 3.575

8.  Activating mutations in the ABCC8 gene in neonatal diabetes mellitus.

Authors:  Andrey P Babenko; Michel Polak; Hélène Cavé; Kanetee Busiah; Paul Czernichow; Raphael Scharfmann; Joseph Bryan; Lydia Aguilar-Bryan; Martine Vaxillaire; Philippe Froguel
Journal:  N Engl J Med       Date:  2006-08-03       Impact factor: 91.245

9.  The effect of early, comprehensive genomic testing on clinical care in neonatal diabetes: an international cohort study.

Authors:  Elisa De Franco; Sarah E Flanagan; Jayne A L Houghton; Hana Lango Allen; Deborah J G Mackay; I Karen Temple; Sian Ellard; Andrew T Hattersley
Journal:  Lancet       Date:  2015-07-28       Impact factor: 79.321

10.  Type 1 Diabetes Genetic Risk Score: A Novel Tool to Discriminate Monogenic and Type 1 Diabetes.

Authors:  M N Weedon; A T Hattersley; K A Patel; R A Oram; S E Flanagan; E De Franco; K Colclough; M Shepherd; S Ellard
Journal:  Diabetes       Date:  2016-04-05       Impact factor: 9.461

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  3 in total

1.  Maturity-Onset Diabetes of the Young Identified Among Algerian Probands with Early-Onset Diabetes.

Authors:  Faiza Bouldjennet; Anette P Gjesing; Malha Azzouz; Samir Ait Abderrahman; Amina El Guecier; Said Ali; Brahim Oudjit; Farida Mennadi-Lacete; Lyèce Yargui; Aissa Boudiba; Ahcène Chibane; Chafia Touil-Boukoffa; Torben Hansen; Rachida Raache
Journal:  Diabetes Metab Syndr Obes       Date:  2020-12-08       Impact factor: 3.168

Review 2.  Applications of iPSC-derived beta cells from patients with diabetes.

Authors:  Kristina G Maxwell; Jeffrey R Millman
Journal:  Cell Rep Med       Date:  2021-04-20

3.  NEUROD1 mutation in an Italian patient with maturity onset diabetes of the young 6: a case report.

Authors:  Lucia Brodosi; Bianca Baracco; Vilma Mantovani; Loris Pironi
Journal:  BMC Endocr Disord       Date:  2021-10-15       Impact factor: 2.763

  3 in total

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