Presentation of two clinical cases:

The first case was a newborn with a diagnosis of transient DMN, in whom hyperglycemia was an accidental finding. A mutation was identified in one gene which encodes some subunities of the ATP-sensitive K channel (KATP). This patient had a good response to glibenclamide. The second newborn developed a transient NMD secondary to a 6q24 mutation, but this patient also presented aortic coarctation, urinary tract and brain malformations. The hyperglycemia was treated with insulin.

Conclusions:

These two cases illustrate the variability that can occur in DMN, both with different evolution and therapeutic management, as well as the need of molecular genetics for diagnosis and treatment.