Infantile Cholestasis Flashcards
Cholestasis is defined as _____________ due to _____________ or to _____________ through intra or extra hepatic bile ducts
decrease in bile flow
impaired secretion by hepatocytes
obstruction of bile
Infantile cholestasis is defined as _________ occurring within the first _________ of extra uterine life
conjugated hyperbilirubinemia
90 days
Surgical jaundice is???
Any type of jaundice that is fixable with surgery
Cholestatic jaundice affects approximately 1 in _______ infants globally
• ___________ accounts for 25-35% o f infantile cholestasis globally.
•In LUTH- ________ is the commonest cause o f infantile cholestasis
2500
Biliaryatresia
Sepsis
Infantile cholestasis has ____ sex predilection
however biliary atresia shows ________ preponderance, while neonatal
hepatitis is commoner in _____.
no
female
males
Pathophysiology of infantile cholestasis
•immaturity of the _________________ in infants
•manifestations of __________________ early in life
•susceptibility of infants _________ to various ________ and _______ stimuli
hepatobiliay excretory function
inborn errors of metabolism
immature liver ; infections and toxic
Cholestasis can be classified into _______ and ________
Extra hepatic
Intra hepatic
Intra hepatic cholestasis can be divided into __________ and _________
Hepatocellular
Obstructive
Etiology o f EXTRAHEPATIC cholestasis
• Extrahepatic ________
• __________ cyst
• Bile duct _________
• Spontaneous _________ of the _______
•____________
• inspissated bile/ mucus plug
• Extrinsic ________________
biliaryatresia; Choledochal
stenosis
perforation of the bileduct
Cholelithiasis; compression of the bileduct
Etiology of intrahepatic cholestasis
________
__________
_____________ disorders
Miscellaneous
Infections
Metabolic
Endocrine
CLINICAL PRESENTATION of infantile cholestasis
Mention 6
•__________
• Xanhtoma
•________
•—————
•________ stool
• _____ urine
• Pasage of ____________
• Pruritus
• Failure to _______
• Night blindnes • Rickets
Jaundice
Hepatomegaly
splenomegaly
Acholic; dark
Bulky stool
thrive
Comparison between biliary atresia and intrahepatic hepatitis
In terms of:
Location
Birth weight
Stool
Associated anomalies
Ultrasonographic finding
Duodenal intubation
Post hepatic; intra hepatic
Normal ; pre-term
Persistently acholic; alternating normal and pale
Plenty; rare
Triangular cord sign; absent cord sign
Doesn’t Yield bile stained fluids; yields bile stained fluids