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CholestasisClinicalPresentation
Updated:Aug21,2015
Author:HishamNazer,MB,BCh,FRCP,,DTM&HChiefEditor:CarmenCuffari,MDmore...

PRESENTATION

History
Patientswithcholestasismaypresentclinicallyinmanydifferentwaysdependingonthedisease
process.
Inmostcases,scleralicterusisnotedbeforeanyothersignitmaybeapparentatconjugated
bilirubinlevelsaslowas2mg/dL.
Athigherlevelsofconjugatedbilirubin,darkurinemaybenotedsecondarytothefilteringof
bilirubinintotheurine.
Cutaneousjaundicemaynotbenoteduntilbilirubinlevelsreach5mg/dLorhigher.
Inpatientswithcholestasis,anothercommonpresentationisseverepruritussecondaryto
elevatedbileacids.
Athighconcentrations(5timesthereferencerange),retainedbileacidscancause
maddeningpruritusinwhichpatientsareunabletosleeporconcentrateandmayresortto
cutaneousmutilationforrelief.

Physical
Asnotedabove,thephysicalsignsofcholestasisareusuallyscleralicterusorcutaneousjaundice,or
both.Thesepatientsmayhavephysicalevidenceofscratchingorexcoriationiftheyalsohavesevere
bileacidretention.
Xanthomaslooklikesmallwhitepapulesorplaquesandareusuallyfoundonthetrunkand
diaperareaandinareasoffriction(eg,diaperline,creasesofhands,elbows,neck).
Anotherimportantphysicalfindinginpatientswithcholestasismaybeevidenceoffailureto
thrivewithalteredanthropometrics,suchasreducedheightandreducedweightforheightdue
tofatmalabsorption.

Causes
Seethelistbelow:
Obstructivecholestasis
Biliaryatresia
Congenitalbileductanomalies(choledochalcysts)
Cholelithiasis
Primarysclerosingcholangitis
Infectiouscholangitis(cholangitis)
CholangitisassociatedwithLangerhanscellhistiocytosis

Alagillesyndrome
Nonsyndromicductalpaucity
Hepatocellularcholestasis
Hepatitis(hepatitisA,hepatitisB,hepatitisC)
Alpha1antitrypsindeficiency
Inbornerrorsofbileacidsynthesis
Druginducedcholestasis
Totalparenteralnutrition(TPN)associatedcholestasis
Progressivefamilialintrahepaticcholestasis[4,5,6,7,8]
Workup
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