浙江大学医学院：《儿科学》婴儿胆汁淤积症 Infantile cholestasis

Infantile cholestasis 浙江大学医学院附属儿童医院 江米足

Infantile cholestasis 浙江大学医学院附属儿童医院 江米足

Neonatal jaundice Neonatal jaundice is one of the most common conditions needing medical attention in newborn babies About 60% of term and 80% of preterm babies develop jaundice in the first week of life, and about 10% of breast fed babies are still jaundiced at age i month Neonatal jaundice is generally harmless but high concentrations of unconjugated bilirubin may occasionally cause kernicterus (permanent brain damage)

Neonatal jaundice ◼ Neonatal jaundice is one of the most common conditions needing medical attention in newborn babies. ◼ About 60% of term and 80% of preterm babies develop jaundice in the first week of life, and about 10% of breast fed babies are still jaundiced at age 1 month. ◼ Neonatal jaundice is generally harmless, but high concentrations of unconjugated bilirubin may occasionally cause kernicterus (permanent brain damage)

Physiologic jaundice Jaundice becomes visible on the 2nd-3rd day usually peaking between the 2nd and 4th days at 5-6mg/d and decreasing to below 2 mg/dl between the 5th and /th days of life 6-7 of full-term infants have indirect bilirubin levels 212.9 mg/dl and less than 3% have levels≥15mg/dl Indirect bilirubin levels in full-term infants decline to adult levels(1mg/dl) by 10-14 days of life

Physiologic jaundice ◼ Jaundice becomes visible on the 2nd-3rd day, usually peaking between the 2nd and 4th days at 5-6mg/dl and decreasing to below 2 mg/dl between the 5th and 7th days of life. ◼ 6-7% of full-term infants have indirect bilirubin levels ≥12.9 mg/dl and less than 3% have levels ≥ 15 mg/dl. ◼ Indirect bilirubin levels in full-term infants decline to adult levels (1mg/dl) by 10-14 days of life

In contrast to physiological unconjugated hyperbilirubinaemia which requires careful monitoring but is common and usually benign, the presence of significant conjugated bilirubin always indicates pathology

◼ In contrast to physiological unconjugated hyperbilirubinaemia, which requires careful monitoring but is common and usually benign, the presence of significant conjugated bilirubin always indicates pathology

Pathologic jaundice It appears in the 1st 24-36hr of life Serum bilirubin is rising at a rate faster than 5 mg/d/24hr. Serum bilirubin is 2 12 mg/dl in full-term or 10 14 mg/dl in preterm infants Jaundice persists after 10-14 days of life Direct-reacting bilirubin is 22 mg/dl at any time Among other factors suggesting a nonphysiologic cause of jaundice

Pathologic jaundice ◼ It appears in the 1st 24-36hr of life ◼ Serum bilirubin is rising at a rate faster than 5 mg/dl/24hr. ◼ Serum bilirubin is ≥ 12 mg/dl in full-term or 10- 14 mg/dl in preterm infants. ◼ Jaundice persists after 10-14 days of life. ◼ Direct-reacting bilirubin is ≥ 2 mg/dl at any time. ◼ Among other factors suggesting a nonphysiologic cause of jaundice

Cholestasis An alternative or concomitant response to injury caused by extrahepatic or intrahepatic obstruction to bile flow Accumulation in serum of substances normally excreted in bile such as direc-reacting bilirubin choesterol bile acid trace elements occurs

Cholestasis ◼ An alternative or concomitant response to injury caused by extrahepatic or intrahepatic obstruction to bile flow. ◼ Accumulation in serum of substances normally excreted in bile such as ◼ direc-reacting bilirubin ◼ choesterol ◼ bile acid ◼ trace elements occurs

Neonatal cholestasis a Neonatal cholestasis is defined a prolonged elevation of serum levels of conjugated bilirubin beyond the first 14 days of life Jaundice that appears after 2 wk of age progress after this time, or does not resolve at this time should be evaluated and a direct bilirubin level determined

Neonatal cholestasis ◼ Neonatal cholestasis is defined a sprolonged elevation of serum levels of conjugated bilirubin beyond the first 14 days of life. ◼ Jaundice that appears after 2 wk of age, progress after this time, or does not resolve at this time should be evaluated and a direct bilirubin level determined

Neonatal cholestasis May be due to infectious genetic metabolic, or undefined abnormalities mechanical obstuction of bile flow functional impairment of hepatic excretory function and bile secretion May be divided into extrahepatic and intrahepatic disease

Neonatal cholestasis ◼ May be due to infectious, genetic, metabolic, or undefined abnormalities ◼ mechanical obstuction of bile flow ◼ functional impairment of hepatic excretory function and bile secretion. ◼ May be divided into extrahepatic and intrahepatic disease

Neonatal cholestasis Neonatal cholestasis Intrahepatic disease Extrahepatic disease(bile duct injury or Hepatocyte injury Bile duct injury obstruction) Metabolic Viral Idiopathic Intrahepatic Extrahepatic disease neonatal disease bile duct biliary hepatitis hypoplasia atresia or paucity

Neonatal cholestasis Neonatal cholestasis Intrahepatic disease Extrahepatic disease (bile duct injury or obstruction) Extrahepatic biliary atresia Hepatocyte injury Bile duct injury Intrahepatic bile duct hypoplasia or paucity Metabolic disease Viral disease Idiophathic neonatal hepatitis

Extrahepatic disorders Biliary atresia sclerosing cholangitis Bile duct stenosis Choledochal cyst Choledochal-pancreaticoductal junction Anomaly Spontaneous perforation of the bile duct Mass(neoplasia, stone) Bile/mucous plug

Extrahepatic disorders ◼ Biliary atresia ◼ sclerosing cholangitis ◼ Bile duct stenosis ◼ Choledochal cyst ◼ Choledochal-pancreaticoductal junction Anomaly ◼ Spontaneous perforation of the bile duct ◼ Mass (neoplasia, stone) ◼ Bile/mucous plug