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These cases are important to efficiently and accurately diagnose, given the clinical implications of syndromic features, cost-effective treatment regimen, and the potential impact on multiple family members. Proper recognition of the clinical manifestations, family history, and cost-effective lab and genetic testing provide the diagnosis. All patients must undergo a thorough history, physical examination, multigenerational family history, lab evaluation (glycated hemoglobin A1c [HbA1c], glutamic acid decarboxylase antibodies [GADA], islet antigen 2 antibodies [IA-2A], and zinc transporter 8 [ZnT8] antibodies). The presence of clinical features with 3 (or more) negative antibodies may be indicative of MODY-monogenic diabetes, and is followed by genetic testing. Molecular genetic testing should be performed before attempting specific treatments in most cases. Additional testing that is helpful in determining the risk of MODY-monogenic diabetes is the MODY clinical risk calculator (&amp;gt;25% post-test probability in patients not treated with insulin within 6 months of diagnosis should trigger genetic testing) and 2-hour postprandial (after largest meal of day) urinary C-peptide to creatinine ratio (with a \u22650.2 nmol\/mmol to distinguish HNF1A- or 4A-MODY from type 1 diabetes). Treatment, as well as monitoring for microvascular and macrovascular complications, is determined by the specific variant that is identified. In addition to the diagnostic approach, this article will highlight recent therapeutic advancements when patients no longer respond to first-line therapy (historically sulfonylurea treatment in many variants).<\/jats:p><\/jats:sec><jats:sec><jats:title>Learning Objectives<\/jats:title><jats:p>Upon completion of this educational activity, participants should be able to:<\/jats:p><\/jats:sec><jats:sec><jats:title>Target Audience<\/jats:title><jats:p>This continuing medical education activity should be of substantial interest to endocrinologists and all health care professionals who care for people with diabetes mellitus.<\/jats:p><\/jats:sec>","DOI":"10.1210\/clinem\/dgaa710","type":"journal-article","created":{"date-parts":[[2020,10,9]],"date-time":"2020-10-09T10:27:41Z","timestamp":1602239261000},"page":"237-250","source":"Crossref","is-referenced-by-count":146,"title":["Approach to the Patient with MODY-Monogenic Diabetes"],"prefix":"10.1210","volume":"106","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4964-7716","authenticated-orcid":false,"given":"David T","family":"Broome","sequence":"first","affiliation":[{"name":"Department of Endocrinology, Diabetes & Metabolism, Cleveland Clinic Foundation, Cleveland, Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3897-4551","authenticated-orcid":false,"given":"Kevin M","family":"Pantalone","sequence":"additional","affiliation":[{"name":"Department of Endocrinology, Diabetes & Metabolism, Cleveland Clinic Foundation, Cleveland, Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8152-4046","authenticated-orcid":false,"given":"Sangeeta R","family":"Kashyap","sequence":"additional","affiliation":[{"name":"Department of Endocrinology, Diabetes & Metabolism, Cleveland Clinic Foundation, Cleveland, Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2208-3607","authenticated-orcid":false,"given":"Louis H","family":"Philipson","sequence":"additional","affiliation":[{"name":"Kovler Diabetes Center, Departments of Medicine and Pediatrics, University of Chicago, Chicago, Illinois"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"80","published-online":{"date-parts":[[2020,10,9]]},"reference":[{"key":"2021031115032637700_CIT0001","author":"MODY Probability Calculator \/\/ Diabetes Genes"},{"issue":"2","key":"2021031115032637700_CIT0002","doi-asserted-by":"crossref","first-page":"286","DOI":"10.2337\/dc10-1293","article-title":"Urinary C-peptide creatinine ratio is a practical outpatient tool for identifying hepatocyte nuclear factor 1-{alpha}\/hepatocyte nuclear factor 4-{alpha} maturity-onset diabetes of the young from long-duration type 1 diabetes","volume":"34","author":"Besser","year":"2011","journal-title":"Diabetes 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