FACTORS ALTERING CONCENTRATION OF BLOOD LIPIDS 453 



the disease is usually absent in pathological states other than diabetes 

 in which hyperlipemia exists. However, Ahrens and Kunkel 621 reported 

 the occurrence of xanthomas in eighteen cases of primary biliary cirrhosis. 

 The presence of xanthomas coincided with an elevation of total serum 

 lipids; they tended to disappear when the total serum lipid decreased. 

 Xanthomatosis may, however, occur without concomitant hypercholester- 

 olemia. One such case reported by Rosenthal and Braunisch 622 was im- 

 proved by treatment with adrenalin. Diabetic xanthomatosis responds to 

 treatment with insulin. 622-625 According to one report, the xanthomatosis 

 regressed with the improvement of the diabetes, and was intensified when 

 the diabetes became more severe. 626 A similar relationship has been re- 

 ported as existing between diabetes on the one hand and both xanthomato- 

 sis and lipemia retinalis on the other hand. 627 



Another allied condition called essential xanthomatosis, or essential chol- 

 esterolemia, which occurs independently of diabetes, involves the forma- 

 tion of nodules in various locations in the body. These are composed 

 largely of cholesterol. Blood cholesterol has been found to be at a level 

 of 400 to 500% of that of normal subjects, but the other blood lipids are 

 not proportionately increased. It has been suggested that this condition is 

 related to a congenital inability to excrete cholesterol. Although some 

 cases may respond to a cholesterol-free diet, Sperry and Schick 628 were un- 

 able to note any improvement as a result of this dietary measure. Ac- 

 cording to Chanutin and Ludewig, 629 the free cholesterol, phospholipids, 

 and total lipids in the blood were increased in xanthomatosis, while choles- 

 terol ester and neutral fats were reduced. Schick and Sperry 630 reported 

 that a patient with essential xanthomatosis whom they had observed over 

 a fifteen-year period, during thirteen years of which he had lived on a diet 

 containing negligible amounts of cholesterol, exhibited hypercholesterol- 

 emia throughout the interval. These workers emphasize the familial 

 nature of essential xanthomatosis, and support the concept that inherited 

 hypercholesterolemia is the primary disturbance in this disease. 



621 E. H. Ahrens, Jr., and H. G. Kunkel, J. Clin. Invest., 28, 1565-1574 (1949). 



622 F. Rosenthal and R. Braunisch, Z. klin. Med., 92, 429-441 (1921). 



623 A. Chauffard, P. Brodin, and R. Yavanovitch, Bull. mem. soc. med. hop. Paris 

 [3], 48, 1573-1582(1924). 



624 W. H. Gordon and M. S. Feldman, /. Michigan Stale Med. Soc., 23, 231-233 (1924). 

 626 T. H. McGavack and H. C. Shepardson, Ann. Internal Med., 7, 582-604 (1933). 



626 J. A. Buchanan and J. C. Indelicato, Am. J. Med. Sci., 195, 50-53 (1938). 



627 A. C. Curtis, J. M. Sheldon, and H. C. Eckstein, Am. J. Med. Sci., 186, 548-556 

 (1933). 



628 W. M. Sperry and B. Schick, Am. J. Diseases Children, 51, 1372-1384 (1936). 



629 A. Chanutin and S. Ludewig, /. Lab. Clin. Med., 22, 903-911 (1937). 



630 B. Schick and W. M. Sperry, Am. J. Diseases Children, 77, 164-174 (1949). 



