“…Clinical manifestations are seen chiefly in the pediatric age group. As a cause of death among children under 15 years, it outranks such conditions as poliomyelitis, rheumatic fever, and diabetes. 1 The condition, first described by Fanconi, et al.,2 in 1936, was brought to recognition as a specific disease entity in 1938 by several investigators,3,4 most notably Andersen.5 At that time, pancreatic insufficiency was considered the basic defect and was responsible for the name given to the disease.5'6 This is a misleading term, however, for it is a pathological condition now known to involve many other organs, particularly the lungs, paranasal sinuses, sweat glands, liver and rectum.…”