[Long-term results in the treatment of extrahepatic bile duct atresia]. 1985

P Schweizer

This article is intended as a contribution towards the question of long-term results to be expected when using hepato-portodigestive anastomoses in children. The paper is based on analyses of the course of the disease in 683 children, as placed at the author's disposal by paediatricians and paediatric surgeons in Europe, the United States of America, and Japan, as well as on an evaluation of 56 child patients observed by the author himself. This assessment resulted in the formulation of five conditions determining the prognosis. One can expect a 5-year survival rate of 66 per cent with good clinical, laboratory, sonographic and scintiscan data as well as histological findings, provided surgery is possible before 6 weeks after onset of cholestasis (this usually means surgery before the 8th week of life), provided also that cirrhosis has not set in at the time of surgery and that there are no additional numerical and morphological changes of the interlobular bile ducts; furthermore, if the average total diameter of the bile duct structures in the hepatic porta is at least 450 mu, and if no cholangitides occur postoperatively. If these conditions are not fulfilled, prognosis must be considered as poor. Such patients become candidates for a liver transplant, which has a 4-year survival rate of 58 per cent since introduction of the cyclosporin A-low-dose-steroid treatment. The article also discusses questions of organisation and of ethics connected with liver transplantation.

UI MeSH Term Description Entries
D007223 Infant A child between 1 and 23 months of age. Infants
D007231 Infant, Newborn An infant during the first 28 days after birth. Neonate,Newborns,Infants, Newborn,Neonates,Newborn,Newborn Infant,Newborn Infants
D007567 Jaundice, Neonatal Yellow discoloration of the SKIN; MUCOUS MEMBRANE; and SCLERA in the NEWBORN. It is a sign of NEONATAL HYPERBILIRUBINEMIA. Most cases are transient self-limiting (PHYSIOLOGICAL NEONATAL JAUNDICE) occurring in the first week of life, but some can be a sign of pathological disorders, particularly LIVER DISEASES. Icterus Gravis Neonatorum,Neonatal Jaundice,Physiological Neonatal Jaundice,Severe Jaundice in Neonate,Severe Jaundice in Newborn,Jaundice, Physiological Neonatal,Neonatal Jaundice, Physiological
D008105 Liver Cirrhosis, Biliary FIBROSIS of the hepatic parenchyma due to obstruction of BILE flow (CHOLESTASIS) in the intrahepatic or extrahepatic bile ducts (BILE DUCTS, INTRAHEPATIC; BILE DUCTS, EXTRAHEPATIC). Primary biliary cholangitis involves the destruction of small intra-hepatic bile ducts and decreased bile secretion. Secondary biliary cholangitis is produced by prolonged obstruction of large intrahepatic or extrahepatic bile ducts from a variety of causes. Biliary Cirrhosis,Biliary Cirrhosis, Primary,Biliary Cirrhosis, Secondary,Cholangitis, Chronic Nonsuppurative Destructive,Liver Cirrhosis, Obstructive,Primary Biliary Cholangitis,Biliary Cirrhosis, Primary, 1,Primary Biliary Cirrhosis,Secondary Biliary Cholangitis,Secondary Biliary Cirrhosis,Biliary Cholangitides, Primary,Biliary Cholangitis, Primary,Biliary Cholangitis, Secondary,Cholangitides, Primary Biliary,Cholangitis, Primary Biliary,Cholangitis, Secondary Biliary,Cirrhosis, Biliary,Cirrhosis, Secondary Biliary,Liver Cirrhoses, Biliary,Obstructive Liver Cirrhosis,Primary Biliary Cholangitides,Secondary Biliary Cholangitides
D011183 Postoperative Complications Pathologic processes that affect patients after a surgical procedure. They may or may not be related to the disease for which the surgery was done, and they may or may not be direct results of the surgery. Complication, Postoperative,Complications, Postoperative,Postoperative Complication
D011788 Quality of Life A generic concept reflecting concern with the modification and enhancement of life attributes, e.g., physical, political, moral, social environment as well as health and disease. HRQOL,Health-Related Quality Of Life,Life Quality,Health Related Quality Of Life
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D005500 Follow-Up Studies Studies in which individuals or populations are followed to assess the outcome of exposures, procedures, or effects of a characteristic, e.g., occurrence of disease. Followup Studies,Follow Up Studies,Follow-Up Study,Followup Study,Studies, Follow-Up,Studies, Followup,Study, Follow-Up,Study, Followup
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D001652 Bile Ducts The channels that collect and transport the bile secretion from the BILE CANALICULI, the smallest branch of the BILIARY TRACT in the LIVER, through the bile ductules, the bile ducts out the liver, and to the GALLBLADDER for storage. Bile Duct,Duct, Bile,Ducts, Bile

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