CONGENITAL HEMOLYTIC ICTERUS 489 



sible for the iiitoxieation 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 -i^ is the oxistcneo of cillicr l)ilc salts or l)ilo pi<j;ment sep- 

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

 creted by the kidney, leaving only the le^s dillusible piprinent in the blood, or by 

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

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



CONGENITAL HEMOLYTIC ICTERUS 2a 



This term describes a cimdition charat'teri/ed by a chronic, non-obstructive 

 jaundice, without evident intoxication. A similar condition is also obser\ed de- 

 veloping in adults, without familial tendencies. The congenital form usually 

 shows familial character, but isolated congenital cases do occur. It is the re- 

 sult of active hemolysis, apparently taking place chiefly in the spleen, and lead- 

 ing to an icterus without evident participation of the liver. Tlie cause of the 

 hemolysis is entirely unknown, althougli 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 urobilin in l)oth stools 

 and urine; the blood contains bilirubin wliich is not excreted in the urine. The 

 jaundice is usually unaccompanied by evidences of cholemia. icteric pruritis or 

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

 lowed splenectomy but the exact relation 'of the spleen to tlic disease is not 

 known. 2b The frequent occurrence of gall stones in this condition may be the re- 

 sult of hypercholesterolemia from hemolysis. 



The metabolism of a case 2c showed loss of nitrogen, calcium, maenesium and 

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

 after operation. 2d 



The Pigmentation in Icterus. — Living tissues have but a slight 

 tendency to take up bile-pigments, much of the tissue-staining ob- 

 served at autopsy being due to postmortem imbibition from the blood 

 and lymph. Quincke ^ found that after .subcutaneous injection of 

 bilirubin only the connective tissue, both cells and intercellular fibrils, 

 becomes diffusely colored ; later, it fades out of the cells, leaving only 

 the fibrils stained. ]\Iuscle-cells, fat-cells, and vessel-walls take up the 

 pigment only after their death. If the jaundice continues for a long 

 time, the subcutaneous deposits of bilirubin may undergo a slow oxida- 

 tion, the color changing to an olive or to a dirty grayish green. The 

 pigment in the connective tis,sues is at first in solution, but may be de- 

 posited in a granular form after a considerable amount has accumu- 

 lated. Bile pigments and bile salts may both be present in consider- 

 able amounts in the blood and not pass through the kidneys, and also 



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

 2e Hoover and Blankenhorn. Arch. Int. Med.. 1010 (18), 289. 

 2a See Richards and Johnson, Jour. Amer. Med. Assoc., 191.3 (61), 1586. 

 2b See series of articles by Pearce ct al. in Jour. Exp. ^led., on Relation of 

 Spleen to Blood Destruction. 



2c IMcKelvy and Rosenbloom, Arch. Int. Med.. IOI.t (In), 227. 



2d Goldschmidt, Pepper and Pearce, Arch. Int. Med., 191.5 (16), 437. 



3Virchow's Arch., 1884 (95), 125. 



