MACROSOMIA ADIPOSA CONGENITA A NEW DYSENDOCRINE SYNDROME OF FAMILIAL OCCURANCE

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Abstract
A new dysendocrine syndrome is described and discussed. On account of its clinical aspect it is called Macrosomia adiposa congenita. It was met with in the children of 2 sisters with menstrual disorders. Among 9 children, born at full term, 7 were maeroso-mians; and 5 of these died within the 1st year of life. Autopsy (1 case) showed adenomata in the suprarenal cortex and accumulation of eosinophiles in the thymus. Macrosomia adiposa congenita comes probably within the obese type of premature development (Guthrie and Emery), depending upon a hyperfunction of the suprarenal cortex (Apert); it is in a class by itself, however, through its lack of sexual abnormalities and hirsutes. Most likely macrosomia[long dash]as well as the suprarenal syndromes on the whole[long dash]is in fact a pluriglandular syndrome. From another point of view, macrosomia adiposa congenita may be regarded as a lethal, hereditable abnormality, whose appearance may be explained as a phenomenon of transformative heredity or heteropheny (Siemens).

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