Later, the EDS nosology has used the name “spondylodysplastic EDS” for a group of three conditions, B3GALT6 deficiency (better known as spondyloepimetaphyseal dysplasia with joint laxity, Beighton type), B4GALT7 deficiency, and SLC39A13 deficiency (the original spondylo-cheiro-dysplastic type) [3]. The gene discussed is B3GALT6; the disease is hyperinsulinemic hypoglycemia, familial, 4.