Tropical Diseases/Chapter 35
CHAPTER XXXV
INFANTILE BILIARY CIRRHOSIS
WITHIN the last four decades a peculiar disease of the liver has been noticed in children in Calcutta and, to a less extent, in other large towns of India. It is found to be more prevalent in Hindu than in Mohammedan children. Thus, in Calcutta, from 1891 to 1893 inclusive, infantile biliary cirrhosis, the name given to this disease, caused 1,748 deaths. Although the Hindu and Mohammedan populations of that city are about equal, yet as many as 1,616 of the deaths occurred in Hindus, whilst only 80 occurred among Mohammedans, the balance of the mortality being among the Eurasians and other races. The disease occurs principally in children under one year, rarely attacking those over three years. As a rule, it commences during dentition, or about the seventh or eighth month, running a fatal course in from three to eight months. In rare cases it may commence within a few days of birth. Instead of lasting several months, its progress may be much more rapid, and terminate in death in from two to three weeks.
The cause of infantile biliary cirrhosis is quite unknown. Neither alcohol, syphilis, nor malaria has anything to do with it. The children of the well-to-do are relatively more frequently attacked than those of the poor. It has also been observed that it tends to run in families, child after child of the same parents succumbing within a year or two of birth. In 400 cases Ghose had only six recoveries; in some of these recoveries the diagnosis was doubtful.
Symptoms.— Commencing insidiously, the characteristic initial enlargement of the liver may have made considerable progress before the disease is suspected. Nausea, occasional vomiting, sallowness, feverishness, constipation, anorexia, irritability of temper, thirst, and languor call attention to the child's condition. On examination the liver is found to be enormously enlarged, extending perhaps to the umbilicus or even lower. The surface of the organ is smooth; the edge, at first rounded and prominent, as the liver begins to contract becomes sharp and distinct and can be readily grasped between the fingers, the swollen organ feeling hard and resistant. Fever of a low type sets in; the sallowness deepens into profound jaundice; the stools are pale, the urine is dark; and there may be ascites, with puffiness of the feet and hands. Sooner or later, death from cholæmia ensues.
Pathological anatomy and pathology.— Surgeon-Major Gibbons, who has given an elaborate and most careful account of the pathological anatomy of this disease, concludes that it is a peculiar form of biliary cirrhosis, the consequence of the action on the liver cells of some irritant of gastric origin, which leads to degeneration of the cells in the first instance, with subsequent increase of intercellular connective tissue and, later, of the portal sheaths. The formation of new bile-ducts between the hepatic cells, which is a well-marked feature, he regards as evidence of a natural curative effort having for its object a regeneration of the liver cells.
Treatment.— Hitherto, in this disease, treatment has been of little avail. There is some ground, however, for thinking that early removal from the endemic locality, and a complete change of wet-nurse and food, might have a beneficial effect.