Another type of diabetes that occurs in children and young adults is maturity onset diabetes of the young (MODY).  MODY is a rare form of T2D, and accounts in only ~5% of all T2D cases.  MODY is caused by a mutation in a gene involved in either pancreatic development, insulin gene expression, or glucose sensing.  MODY follows an autosomal dominant pattern of inheritance. Thus, in families, affected individuals will usually carry only one copy of the mutant gene; and MODY it is frequently observed in every generation.  Unlike T1D and T2D which are both polygenic and multifactorial, MODY is directly inherited.

 

MODY is characterized by the absence of obesity, no ketosis and no evidence of beta cell autoimmunity. MODY generally presents as mild type 2 diabetes and is most often diagnosed before age 25 years. Because MODY occurs in children, it is sometimes misdiagnosed as T1D. Young women with MODY are also frequently diagnosed during pregnancy. MODY often can be managed without insulin although some individuals with MODY will need to have insulin injections at a later age.