methylmalonic aciduria due to methylmalonyl-CoA mutase deficiency vitamin B12-unresponsive methylmalonic acidemia type mut0 ICD10:E71.1 MONDO:0017360 Orphanet:289916 SCTID:237945003 UMLS:CN203025 Vitamin B12-unresponsive methylmalonic acidemia type mut0 is an inborn error of metabolism characterized by recurrent ketoacidotic comas or transient vomiting, dehydration, hypotonia and intellectual deficit, which does not respond to administration of vitamin B12. complete deficiency of methylmalonyl-CoA mutase vitamin B12-unresponsive methylmalonic aciduria type mut0