NEONATAL CHOLESTASIS Flashcards
DEFN of neonatal cholestsis
Iñcreased conjugat3d bilirubin beyond 14 days of life if :
Total serum bilirubin is < 5 mg /dl ; ⬆️conj bil >= 1 mg / dl
TSB > 5 mg / dl; ⬆️ conj bil >20%
Markers of cholestasis 🌟NAG
5’- nucleotidase
ALP
GGT ( 4X in Intrahepatic causes , 10X extrahepatic causes)
USG feature of neonatal cholestasis
Triangular cord sign - hyperechoic areas corresponding to fibrous or atretic portion of bike duct
EHBA finding in radionucleotide study
HIDA SCAN - DEcreased excretion of tracer
Best investigation for neonatal cholestasis
Liver biopsy
PFIC
Progressive familial Intrahepatic cholestasis
Impaired bile salt secretion
2 types :
Type 1 - AT8B1 GENE (BYLERS DISEASE )
TYPE 2 - ABCB2 GENE ( ⬇️GGT )
BYLERS disease
AT8B1 GENE DEFECT
TYPE 1 PFIC
Basm syndrome
Biliary atresia
cArdiac defects
Spleenic malformations
Situs inversus
PolySpleenism
Malrotation of gut
Triangular facies
Allagille syndrome
Broad forehead
Widely spaced eyes
Underdeveloped mandible
Long straight nose
Alagille syndrome why ?
Autosomal dominant disease
Arterio hepatic dysplasia
Paucity of Intrahepatic bile duct
Gene - JAGGED 1 / NOTCH 2
Alagille syndrome clinical features
Jaundice
Facies
Eyes
Cardiac
Pruritis
Tendon xanthoma
Eye features in alagille syndrome
Microcornea
Posterior embryotoxon
Schwalbe’s ring
Heart defects in alagille
Peripheral pulmonary stenosis
TOF
VSD
Surgical mgt of neonatal cholestasis
Kasai ‘ s procedure
Hepatojejunostomy
Temporary treatment
To be done as soon as possible after birth
Mgt for fat soluble vitamin deficiency in neonatal cholestasis
Vit A: oral 10-25000 IU /day
D: oral 800IU / day
E: oral 25-200iu / kg / day
K; inj , 2-5 mg every 4 weeks
