HEREDITY IN THE INFANTILE TYPE OF GAUCHER'S DISEASE

Abstract

The chronic type of kerasin storage disease has been known since 1882, when Gaucher¹ published his paper on "the primary epithelioma" of the spleen. This condition was found by Schlagenhaufer² in 1907 to be a systemic disease of the reticuloendothelial system, and the acute or infantile type of the disease was differentiated from the chronic or adult type in 1927 by Oberling and Woringer.⁸ The term "acute Gaucher's disease" was coined by Rowland⁴ in 1928 in order to distinguish it from the more chronic type in larger children and adults which was previously known. The acute or infantile type is, according to Rowland,⁵ a rare form of the disease with clinical and pathologic features somewhat different from those of the chronic type. It is characterized by striking familial tendency, by the development of nervous symptoms at the age of 5 to 6 months due to