The pancreatic phenotypes include transient neonatal diabetes (6q24, ABCC8, KCNJ11, INS, HNF1B, SLC2A2, ZFP57 subtypes), in which the diabetes resolves (n=219), permanent diabetes responding to sulfonylurea treatment (KCNJ11 and ABCC8, n=299), permanent insulin-treated diabetes (INS, GCK, EIF2AK3, FOXP3, GLIS3, NEUROD1, NEUROG3, NKX2-2, MNX1, IER3IP1, RFX6, and some cases with GATA6, GATA4 and PDX1 mutations, n=265), and developmental disorders of the exocrine pancreas (GATA6, PTF1A, PDX1 and GATA4) requiring pancreatic enzyme replacement in addition to insulin treatment (n=50). The gene discussed is NKX2-2; the disease is diabetes mellitus.