In both forms, an acute episode of metabolic
decompensation may occur during a catabolic state such as an
infection (1). IVA appearing as diabetic ketoacidosis (DKA) was
reported in very few case reports (2,3,4,5,6). We considered our
case to be of interest because of the rarity of presentation of this
syndrome with DKA and also because of detection of a splicing
mutation in the IVD gene in this patient.