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Maturity-onset diabetes of the young panel (MODY)
Maturity-onset diabetes of the young panel (MODY) [27 genes]: It includes all genes associated with MODY (AKT2, BLK, CEL, GCK, HNF1A, HNF1B, HNF4A, INS, KCNJ11, KLF11, NEUROD1, PAX4, PDX1, PTF1A, RFX6, APPL1) and other early-onset forms of diabetes (EIF2AK3, FOXP3, GATA6, GLIS3, IER3IP1, INSIG2, INSR, NEUROG3, SLC2A2, TBC1D4 WFS1).
Adequate coverage for structural variants or CNVs (copy number variants).
| AKT2 | BLK | CEL | GCK | HNF1A | HNF1B | HNF4A | INS | KCNJ11 | KLF11 |
| NEUROD1 | PAX4 | PDX1 | PTF1A | RFX6 | APPL1 | EIF2AK3 | FOXP3 | GATA6 | GLIS3 |
| IER3IP1 | INSIG2 | INSR | NEUROG3 | SLC2A2 | TBC1D4 | WFS1 |
Maturity-onset diabetes of the young panel (MODY) [27 genes]: It includes all genes associated with MODY (AKT2, BLK, CEL, GCK, HNF1A, HNF1B, HNF4A, INS, KCNJ11, KLF11, NEUROD1, PAX4, PDX1, PTF1A, RFX6, APPL1) and other early-onset forms of diabetes (EIF2AK3, FOXP3, GATA6, GLIS3, IER3IP1, INSIG2, INSR, NEUROG3, SLC2A2, TBC1D4 WFS1).
Adequate coverage for structural variants or CNVs (copy number variants).
Patients with early-onset diabetes (age generally <35 years) presenting with:
- Atypical characteristics for type 1 diabetes mellitus including absence of pancreatic islet autoantibodies, evidence for endogen insulin production beyond the “honeymoon period”, measurable C-peptide in the presence of hyperglycemia, low insulin treatment requirements, absence of ketoacidosis in the absence of insulin treatment.
- Atypical characteristics for diabetes mellitus type 2 including the following: onset of diabetes before age 45, absence of significant obesity, absence of acanthosis nigricans, normal triglyceride levels, and/or normal or elevated HDL-C.
- Mild, stable fasting hyperglycemia not progressing or not noticeably responding to pharmacological therapy.
- Extreme sensitivity to sulfonylureas.
- Extrapancreatic features (renal, hepatic, gastrointestinal).
- Personal or family history of neonatal diabetes or neonatal hypoglycemia.
- Family history of diabetes consistent with a dominant autosomal inheritance pattern, contrasting with diabetes types 1 and 2.
- Monogenetic diabetes. A guideline for NZ healthcare practitioners. Available on the web site at: http://www.nzssd.org.nz/education/2013%20Monogenic_diabetes_card_with_forms_18%20Dec%20copy.pdf
- IDF Clinical Practice Guidelines. International Diabetes Federation. Available on the web site at: http://www.idf.org/sites/default/files/Diabetes-in-Childhood-and-Adolescence-Guidelines.pdf
- American Diabetes Association. Standards of Medical Care in Diabetesd2016. Available on the web site at http://care.diabetesjournals.org/content/suppl/2015/12/21/39.Supplement_1.DC2/2016-Standards-of-Care.pdf