новорожденный, врожденный порок развития, врожденная гиперплазия внутрипеченочных желчных протоков, Pediatrics, RJ1-570, Gynecology and obstetrics, RG1-991

Congenital hyperplasia of the intrahepatic bile ducts refers to early fetopathy. It represents a «bizarre» proliferation of bile ducts in the portal tract with excessive development of connective tissue. A frequent complication of this defect is the attachment of secondary purulent cholangitis. When combined with small cystic kidney disease, death occurs from renal failure in the first days of life.