6MulLlple causes of subfulmlnanL and

fulmlnanL hepaLlc fallure

W lulmlnanL or subfulmlnanL llver fallure
W compllcaLed by encephalopaLhy
W ln many cases by deaLh ls seen Lo be a syndrome LhaL may resulL
from numerous causes
W 90 of all cases vlral hepaLlLls druglnduced hepaLlLls and
hepaLlLls due Lo varlous Lypes of polsonlngs
W ConslderaLlon of Lhe posslblllLy of Lhese less common eLlologles by
Lhe cllnlclan ls of conslderable lmporLance slnce some lncludlng
masslve mallgnanL lnvolvemenL (such as leukemla) or acuLe
fulmlnanL Wllsons dlsease may respond Lo speclflc LreaLmenL
measures
W hepaLlc LransplanLaLlon proves to be opp/icob/e in lnl of many
eLlologlc dlagnosls may conLlnue Lo have lmporLanL LherapeuLlc
lndlcaLlons ln aL leasL some cases wlLh Lhls syndrome
) uescrlbe Lhe paLhology of clrrhosls due Lo chronlc
vlral hepaLlLls and recognlze LhaL Lhe use of Lhe Lerm
posLnecroLlc clrrhosls" ls cllnlcally mlsleadlng
W Macroscoplc
W Lhe llver ls lnlLlally enlarged buL wlLh progresslon of Lhe dlsease lL becomes
smaller
W surface ls lrregular
W Lhe conslsLency ls flrm
W color ls ofLen yellow
W uependlng on Lhe slze of Lhe nodules Lhere are Lhree macroscoplc Lypes
W In m|cronodu|ar form (Laennecs c|rrhos|s or porta| c|rrhos|s) regeneraLlng
nodules are under 3 mm
W ln macronodu|ar c|rrhos|s (postoectotlc clttbosls) tbe ooJoles ote lotqet tboo
J mm
W ln m|xed c|rrhos|s conslsLs ln a varleLy of nodules wlLh dlfferenL slzes
W clrrhosls ls deflned by its potho/oqico/ feotures on microscopy (1) Lhe presence of
regeneraLlng nodules of hepaLocyLes Lhe paLLern of flbrosls seen can depend upon
Lhe underlylng lnsulL LhaL led Lo clrrhosls flbrosls can also prollferaLe even lf Lhe
underlylng process LhaL caused lL has resolved or ceased 1he flbrosls ln clrrhosls
can lead Lo desLrucLlon of oLher normal Llssues ln Lhe llver lncludlng Lheslnusolds
Lhe space of ulsse and oLher vascular sLrucLures whlch leads Lo alLered reslsLance
Lo blood flow ln Lhe llver and porLal hyperLenslon
13
chronic hepotitis 8 there is infi/trotion of the /iver porenchymo with /ymphocytes
) Classlfy Lhe varlous caLegorles of llver dlsease
assoclaLed wlLh drug and Loxln exposures and llsL some
of Lhe more lmporLanL agenLs responslble for Lhese
W @ox|c |n[ury to the ||ver
Drug |nduced
Industr|a| and env|ronmenta| tox|n
A|coho||c
ostoperat|ve hepat|c dysfunct|on
W Infect|ous agents and paras|tes
nepat|t|s v|rus
8acter|a| and other pathogen|c agents
aras|tes
W Immune d|sorder
Auto|mmune
r|mary b|||ary c|rrhos|s
W @umors
r|mary ma||gnant tumors
Metastat|c ma||gnant tumors
8en|gn hepat|c tumors
@umor||ke |es|on
W Inher|ted
W||sons d|sease
nemochromatos|s
Inborn errors
A|pha1ant|tryps|n def|c|ency
Cyst
9) uescrlbe Lhe paLhology of Lhe llver assoclaLed wlLh
clrculaLory dlsLurbances
W nepat|c ve|n (nV) thrombos|s causes asc|tes hepatomega|y and severe congest|on of
the ||ver (8uddCh|ar| syndrome 8CS) Severe hepaLlc flbrosls develops ln Lhls syndrome wlLh a
varleLy of hlsLologlcal paLLerns Some llvers have a paLLern ofclrrhosls ln whlch Lhere is fibrous
bridqinq between nvs ond porto/ trocts {venoporto/ cirrhosis) Lher llvers have a paLLern of
reversedlobulaLlonclrrhosls (venocenLrlc clrrhosls) ln whlch flbrous brldglng beLween Pvs and
porLal LracLs ls mlnlmal 1he prevalence and paLhogenesls of Lhese forms of clrrhosls and Lhe effecL
of porLal veln (v) Lhrombosls ln Lhls dlsease have noL been sLudled We examlned 13 resecLed
llvers from paLlenLs wlLh 8CS Lo deLermlne Lhe dlsLrlbuLlon of vascular obsLrucLlon and Lhe
characLer of Lhe parenchymal response Slx llvers had venoporLal clrrhosls and all of Lhese had
severe v obllLeraLlon caused by Lhrombosls 1hree llvers had venocenLrlc clrrhosls and had
normal medlum and large vs 1he remalnlng slx llvers had mlxed venocenLrlc/veno
porLal clrrhosls and had moderaLe v obllLeraLlon 1he nodules ln venocenLrlc clrrhoslshad
evldence of an unusual clrculaLlon wlLh small arLerles supplylng a mldzonal venous plexus LhaL
appeared Lo draln reLrogradely lnLo paLenL small vs nlne llvers had large regeneraLlve nodules
hlsLologlcally slmllar Lo focal nodular hyperplasla v Lhrombosls ls a frequenL occurrence ln 8CS
1he correlaLlon beLween v Lhrombosls and Lhe paLLern ofclrrhosls suggesLs a role for v
obllLeraLlon ln Lhe genesls of venoporLal brldglng flbrosls ln Lhls dlsease and posslbly ln oLher
dlseases leadlng Lo clrrhosls