Abstract
The author reports the history of a family of six children, of whom two, the eldest and the fifth, are normal; three died, a boy when 6 months old and two girls when 9 and 10 years old, from the same familial disease that also attacked another boy now 7 years old. The essential features of this disease are hypertrophy of both liver and spleen, chronic icterus with evidence of salts and bile-pigments in the blood and urine, retardation of physical, mental, and sexual development, slight deafness in one case and clubbing of the fingers in another. This condition is a good example of biliary cirrhosis of the liver.
After reviewing recent French observations, the author recalls the large contribution of English authors on the subject.
He then distinguishes three types of familial cirrhosis: Laennec's type with enlarged liver, the type of splenomegalic anascitic and anicteric cirrhosis, resembling Banti's syndrome, and the commonest type or biliary cirrhosis.
The author describes particular histological lesions, and when dealing with differential diagnoses, excludes dyslipoidic, polycoric, and other acquired cirrhosis in children. He then summarizes the relationships between this disease and the obstructive cirrhosis to a congenital defect of the bile-ducts, the cirrhosis in young Indians, the syndrome of hepatic lenticular degeneration, and the syndrome of cirrhosis of the liver combined with hæmangiomatosis. Finally, after recalling the close analogy of the condition with renal dwarfism, the author shows how the interpretation of these relationships may throw light on the pathogenesis of various progressive congenital diseases.