494 PATHOLOGICAL PIGMENTATION 



strictors. (7) Reduce motor and sensory irritability. (8) Act on 

 the higher cerebral centers, causing coma, stupor, and death. Sel- 

 lards^^ found that injection of bile salts into guinea pigs causes ulcer- 

 ation and hemorrhage in the stomach. 



It is difficult to decide how much of the profound intoxication that 

 is sometimes present in icterus ("cholemia" and "icterus gravis") 

 to ascribe to the reabsorbed bile, for frequently there is an accompany- 

 ing infection, and even if there is no infection the impairment of liver 

 function by the obstruction of bile outflow must also be reckoned 

 with. The liver is not only the great destroyer of toxic substances 

 absorbed from the alimentary canal, but it is also an important seat 

 of nitrogenous metabolism, interference with which may lead to ac- 

 cumulation of many toxic nitrogenous substances in the blood. ^^ The 

 long duration of severe icterus in some cases of occlusion of the bile- 

 ducts, with relatively shght evidences of intoxication, would seem to 

 indicate, however, that on the whole the bile is not so much respon- 

 sible for the intoxication observed in icterus as are the associated 

 conditions. On the other hand, in not a few instances it has been 

 observed that escape of large quantities of bile into the peritoneal 

 cavity may be followed by symptoms similar to those of icterus gravis; 

 in these cases only the bile can be held responsible for the intoxica- 

 tion. «" 



Dissociated Jaundice*^' is the existence of cither bile salts or bile pigment sep- 

 arately in the blood. This may be produced either by the bile salts being ex- 

 creted by the kidney, leaving only the less diffusible pigment in the blood, or by 

 separate escape of bile salts from the liver into the blood. Also in true hem- 

 olytic icterus we may have bile pigments present in the blood without bile salts. 



Congenital Hemolytic Icterus.'^- — This term describes a condition characterized 

 by a chronic, non-obstructive jaundice, without evident intoxication. A similar 

 condition is also observed developing in adults, without familial tendencies. The 

 congenital form usually shows familial character, but isolated congenital cases do 

 occur. It is the result of active hemolysis, apparently taking place chiefly in the 

 spleen, and leading to an icterus without evident participation of the liver. The 

 cause of the hemolysis is entirely unknown, although there is a marked fragility of 

 the erythrocytes evidenced by reduction of their resistance to hypotonic solutions, 

 and it results in a moderate anemia, with excretion of much uroliilin in both stools 

 and urine; the blood contains biliru])in which is not excreted in the urine. The 

 jaundice is usuall3^ unaccompanied by evidence of cholemia, icteric pruritus or 

 hemophilia. The spleen is greatly enlarged and improvement has generally fol- 

 lowed splenectomy but the exact relation of the spleen to the disease is not known." 

 The frequent occurrence of gall stones in this condition may be the result of hyper- 

 cholesterolemia from hemolysis. 



The metabolism of a case*^' showed loss of nitrogen, calcium, magnesivnn and 

 iron, and a much increased uric acid excretion. These conditions may improve 

 after operation.^^ 



"^ Arch. Int. Med., 1909 (4), 502. 



'« See Bickel, E.xper. Untersuch. iiber der Pathol, der Cholaemie, Wiesbaden 

 1900. 



«»See Ehrhardt, Arch. klin. Chir., 1901 (64), 314. 



0' Hoover and Hlankenhorn, Arch. Int. Med., 1916 HS), 289. 



" See Richards and Johnson, Jour. Amer. Med. .Vssoc, 1913 (61), 1586. 



•3 See series of articles by Pearce el al., in Jour. Exp. Med., on Relation of 

 Spleen to Blood Destruction. 



6< McKelvv and Rosenbloom, Arch. Int. Med., 1915 (15), 227. 



« Goldschmidt, Pepper and Pearce, Arch. Int. Med., 1915 (16), 437. 



