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New PDF release: Liver Disorders in Childhood. Postgraduate Paediatrics

By Alex P. Mowat

ISBN-10: 0407001638

ISBN-13: 9780407001633

This ebook presents an account of contemporary paediatric hepatology

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Additional info for Liver Disorders in Childhood. Postgraduate Paediatrics Series

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1 Causes of Conjugated Hyperbilirubinaemia in Infancy Structural defects Bile duct abnormalities Polycystic disease Vascular lesions Biliary atresia Spontaneous perforation of bile duct Choledochal cyst Bile duct stenosis Biliary hypoplasia syndromes Choledocholithiasis Cholangiolitis Veno-occlusive disease Poor perfusion syndromes Haem angioendothelioma Lymphatic defects Chromosomal abnormalities Metabolic defects Infections Post-haemolytic disorders Toxic or deficiency disorders Intravenous nutrition Drugs Familial syndromes Neonatal hepatic necrosis Idiopathic precise nature of the pigment which gives the liver its characteristic greenish-black appearance is controversial.

28 UNCONJUGATED HYPERBILIRUBINAEMIA Clinical features Jaundice appears soon after birth, the bilirubin rapidly rising to 20mg/ dl (340 jumol/litre), is poorly controlled by phototherapy in the first few days and exchange transfusion is usually necessary. Only with continuous phototherapy is it possible to keep the serum bilirubin below 340 jumol/litre. Investigations fail to show haemolysis or other aggravating factors of neonatal jaundice but a history of parental consanguinity and instances of similar severe icterus in other family members may be found.

There is at present no direct evidence to implicate ineffective hepatic perfusion or diminished transport across the hepatocellular membranes as a factor in physiologic jaundice. PATHOLOGICAL NEONATAL UNCONJUGATED HYPERBILIRUBINAEMIA Pathological neonatal unconjugated hyperbilirubinaemia indicates that a disease process or processes may be present aggravating or accentuating physiological neonatal unconjugated hyperbilirubinaemia, and in clinical practice is considered to exist when serum bilirubin concen­ tration exceeds that found in normal healthy infants.

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Liver Disorders in Childhood. Postgraduate Paediatrics Series by Alex P. Mowat

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