452 PUURIGLAXDULAR DISEASES 



that the father of the first when shown the photograph of the second believed 

 momentarily that it was that of his child. Also the case of Variot and Piron- 

 neau was similar to the first two, as is seen in the photographs these authors 

 publish; it is also remarked by them, although their case is that of a girl. 

 In all cases the disturbance in development began in the earliest youth. In 

 all the inhibition of growth was very considerable, so that we could really 

 speak of dwarfism. In all there was extreme emaciation, with the skin thin 

 folded. In all were the muscles rather prominent. The osseous system 

 showed the characteristic alterations. The dimensions remained childlike. 

 The lower jaw was rather poorly developed, apparently also the upper jaw, 

 causing perhaps the aquiline nose observed in all. The long bones were 

 relatively slender, the ends of the bones thickened, the closure of the epiphyses 

 was rather premature. The genitalia remained backward in development in 

 all cases; and finally the absence of hair is common to all, affecting not 

 only the hairiness belonging to the so-called secondary sexual characters, 

 that on the lip, chin, axillae, pubis, and trunk, but also the hair on the head, 

 lashes, and eyebrows. This increased the senile appearance, which in all 

 these cases is of high degree. 



That here we are dealing with a morbid entity can in all likelihood be as- 

 sumed from the similarity of the cases and the course of the disease. On the 

 contrary the pathogenesis of this condition and the role of the ductless 

 glandular system in the same seem to me to be as yet but little clear. Variot 

 and Pironneau point out that in the case of Hastings Gilford the suprarenal 

 showed fibrous degeneration, and see in the disease picture the sequel of a 

 suprarenal insufficiency's setting-in in youth. A pert goes still further 

 and sees the cause in a damaging of the suprarenal cortex. On account 

 of the characteristic disturbances in the hairiness and in the genital sphere 

 he sees in this disease a countertype to that of tumors of the suprarenal 

 cortex. Hastings Gilford believes on the contrary that we are to ascribe 

 to the hypophysis a "growth center" of considerable importance. The 

 results of the one autopsy done on these cases up to the present are insufficient, 

 as there was no microscopical examination of the ductless glands. It was 

 mentioned only that the suprarenals were sclerosed. In the case of Ransom, 

 that does not belong with certainty to this group, the other ductless glands 

 were also apparently sclerosed. If now also the microscopical examination 

 in such cases would also furnish an extensive sclerosis of the several ductless 

 glands, it would not necessarily be shown the disease of the ductless glands 

 was primary; it would also be possible that we might be dealing with a diffuse 

 sclerotic process involving the entire body, that has also involved the ductless 

 glandular system, such a general process as we regarded above as possible 

 for hemochromatosis and which is perhaps also true for pedatrophy. 



Then we would be dealing with a secondary sclerosis of the ductless 

 glandular system, that at all events lends to progeria many of the striking 



