ABSTRACT
Deficiency of fructose-1,6-diphosphatase (FDP) (fructose1,6-bisphosphatase) was first recognized in 1970 by Baker and Winegrad [1], in a girl with hypoglycemia and metabolic acidosis. A sibling had died of a similar illness. In subsequent reports in 1971 by Baerlocher, Gitzelmann and colleagues [2]
and by Hulsmann and Fernandez [3], there were multiple affected siblings of consanguineous matings.
