DDOST-CDG is a form of congenital disorders of N-linked glycosylation characterized by failure to thrive, developmental delay, hypotonia, strabismus and hepatic dysfunction. The disease is caused by mutations in the gene DDOST (1p36.1). The disease is DDOST-congenital disorder of glycosylation (MONDO_0013789). Also known as: CDG syndrome type Ir, CDG-Ir, CDG1R, DDOST-CDG, carbohydrate deficient glycoprotein syndrome type Ir, congenital disorder of glycosylation type 1r, congenital disorder of glycosylation type Ir.