paediatric jaundice Flashcards
define jaundice
: yellowish discoloration of the skin, sclerae, and mucous membranes due to the deposition of bilirubin
define cholestasis
any condition affecting bile formation or secretion or leading to biliary obstruction within the liver or in the biliary ducts between the liver and the duodenum
cause of unconjugated hyperbillirubinemia
1.Increased hemoglobin breakdown
-hemolysis
#G6PD
#SICKLE CELL
#HEMOLYTIC DISEASE OF NEWBORN
Impaired hepatic uptake of bilirubin
-DRUGS
#rifampin, #probenecid, #sulfonamides
Defective conjugation of bilirubin
-hyperbilirubinemia
#Gilbert’s syndrome and Crigler-Najjar syndrome
-neonatal jaundice(immature hepatocytes + hyperbilli)
-liver diseases #wilson's disease #hepatitis #cirrhosis
causes of Conjugated hyperbilirubinemia
1)Decreased excretion/reuptake of bilirubin (Dubin-Johnson syndrome)
2]Intrahepatic cholestasis
- Liver disease (e.g., hepatitis or cirrhosis)
- Primary biliary cholangitis (autoimmune destruction of the intralobular bile ducts)
3)Extrahepatic cholestasis (biliary obstruction)
-Choledocholithiasis(gallstones)
-Tumors
#pancreatic head cancer, #gallbladder cancer
-Inflammatory processes #primary sclerosing cholangitis,
pathophys of jaundice
elevated serum bilirubin, caused by prehepatic, intrahepatic, or posthepatic defects
UC/indirect hyperbilli
- increased hemoglibin bkdwn
- defective hepatic uptake
- defective hepatic conjugation
sx of jaundiced
yellow eyes, skin, nails
Pale, clay-colored (acholic) stool 4
(bile containing the direct bilirubin cannot reach the colon due to cholestasis, this may lead to discolored stools. Alternatively, the direct bilirubin may be excreted via the urine, making it dark.)
Darkening of urine(increased urobillin in urine)
Pruritus (accumulated bile acids irritate peripheral nerves in the skin)
Fat malabsorption (lack of bile acid in duodenum results in reduced of lipids.)
(steatorrhea, weight loss)
dg
LIVER FUNCTION TESTS 1)hyperbillirubinemia induced cholestasis signs ↑ Alkaline phosphatase (ALP) ↑ Gamma glutamyltransferase (γ-GT) ↑ Bilirubin
2)liver ez
↑ ALAT&ASAT
3)coagulation study
IMAGING
US: dx types of cholestasis
ERCP for further imaging( pancreas)
dg of PREHEPATIC
jaundidce
dark stool elevated indirect normal dirrect normal/dark urine billirubin increased urobilinogen other markers anemia
dg of INTRAHEPATICjaundidce
Pale, clay-colored (rarely dark) stool
↑indirect & ↑direct bilirubin
↑bilirubin in urine (dark urine)
Normal or ↑urobilinogen
other useful
markers
↑Transaminases
↑ Cholestatic enzymes
EXTRA HEPATIC JAUNDICE DG
pale stool
normal indirect
very high direct
very dark urine d/2 increased direct bilirubin and urobillin
increased cholestatic enzymes
dx of jaundice in newborn
deposition of carotene in the skin (carotenoderma) following excessive consumption of fruits and vegetables rich in carotene, such as carrots, sweet potatoes, kale, and oranges or multivitamin supplements. In contrast to jaundice, it does not lead to scleral icterus.
rx
Management of the underlying condition Cirrhosis Hepatitis B, hepatitis C Alcoholic liver disease Primary biliary cholangitis, primary sclerosing cholangitis Inherited hyperbilirubinemia Pancreatic cancer, cholangiocarcinoma Cholelithiasis and cholecystitis Surgical excision of biliary cysts is the preferred treatment. Cholecystectomy is not necessary unless the gallbladder contains
complications of jaundice
Hepatomegaly: Chronic biliary obstruction leading to backflow of bile may result in inflammation.
Risk of kernicterus in newborns with neonatal jaundice