From OMIM
Permanent neonatal diabetes mellitus-1 (PNDM1) is a rare autosomal recessive disorder characterized by severe hyperglycemia which requires insulin treatment soon after birth. The disorder results from a complete lack of glucokinase; total absence of basal insulin release was observed as well (Njolstad et al., 2001). PNDM is distinct from transient neonatal diabetes mellitus (TNDM; see 601410) and childhood-onset autoimmune diabetes mellitus type I (IDDM; 222100). Genetic Heterogeneity of Permanent Neonatal Diabetes Mellitus PNDM2 (618856) is caused by heterozygous mutation in the KCNJ11 (600937) gene on chromosome 11p15.1. PNDM3 (618857) is caused by heterozygous or homozygous mutation in the ABCC8 (600509) gene on chromosome 11p15.1. PNDM4 (618858) is caused by heterozygous or homozygous mutation in the INS (176730) gene on chromosome 11p15.5. Pancreatic agenesis, which results in exocrine pancreatic deficiency as well as permanent neonatal-onset diabetes mellitus, can be caused by mutation in the PDX1 gene (600733). Pancreatic agenesis associated with cerebellar agenesis (609069) can be caused by mutation in the PTF1A gene (607194). Pancreatic agenesis associated with congenital cardiac defects (600001) can be caused by mutation in the GATA6 gene (601656).  http://www.omim.org/entry/606176