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INSUFICIENA HEPATIC ACUT

Definiie

Apariia encefalopatiei hepatice in mai puin de 8 saptmni de la debutul unei

hepatopatii la un pacient fr antecedente hepatice preexistente.

Insuficiena hepatic acut se poate defini i pe baza sumrii urmatorilor parametriclinici i biologici:

debut acut al afeciunii hepatice la un pacient necunoscut cu boal cronic

hepatic

coagulopatie cu T!"#$% secunde sau I&'!"#$ necorectat cu (itamina )

i"sau encefalopatie prezent dac T#*+,*-- sau I&'#*+,*- sau absentdac T!"#$% sau I&'!"#$

Etiologie-(eziTabel nr.*

Histopatologie

/in punct de (edere histopatologic se pot intlni urmtoarele aspecte:

*.Necroz hepatic (

gradul nu se coreleaz cu dez(oltarea encefalopatiei0.1ninfecii (irale apare o distribuie panacinar23n reaciile idiosincrazice ladroguri necroza este zonal2 cele mai multe cazuri se asociaz cu necrozmasi( cu tendina la confluare2 infiltratul inflamator este util in stabilireaetiologiei2 3n unele cazuri nu exist semne de regenerare pe cand 3n altesituaii exist o proliferare a structurilor ductulare.

$. Degenerescena hepatoce!ar" exist o necroz hepatocitarminim"infiltrat inflamator2poate fi cu infiltrarea gras a hepatocitelor2

apare 3n afeciunile metabolice"toxice.

Etiologie,Tabel nr.*

Tabel nr.*

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A#ecti!ni &ou,nscut"sugar mic 4opil!5 luni

In#ecii congenitaesa!$o%&n$ite(#rec'ente

6erpes (irus 746 (irus (irusulhepatitic 9 adeno(irus (irusul7pstein 9arr;790 (irusul4oxac;;? @eptospira

Ca!ze)eta%oice()o$erat#rec'ente

galactozemii tirozinemiihemocromatoza neonatal bolimitocondriale

intoleranta ereditarala fructoza 9. ilson

Into*icaii rare Amanita phaloidesAcetaminofen 6I&>alicilai 44lBanestezice

Ca!zeische)ice+hipo*ice+reper#!zie(rare

cardiopatii congenitale miocarditpostchirurgie cardiac asfixiese(er"hipo TA la nastere

>dr. 9udd 4hiari

Ate ca!ze 6epatita autoimunreCet acut de

transplant hepaticmaligniti.

Patogenie

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Insuficiena hepatic acut determin disfuncie multipl de organ 3n principalfiind afectai creierul i rinichiul. &u sunt cunoscute pe deplin procesele cedetermin inCuria hepatic dar acestea sunt dependente de echilubrul dintreurmtorii factori: se(eritatea afeciunii hepatice susceptibilitatea gazdei

capacitatea de regenerare a ficatului.

Insuficiena hepatic acut conduce la encefalopatie hepatic iar ipotezele carestau la baza apariiei acesteia sunt: ipoteza a)oniaca, ipoteza ne!roto*ineorsinergice, ipoteza #a-ior ne!rotrans)itori, ipoteza ne!rotrans)itoriinhi%itori .A/Aergici0

Tablou clinic

/ebutul poate fi 3n mai puin de cte(a ore"sptmni iar tabloul clinic este funciede etilogia afeciunii hepatice dar 3n general disfuncia hepatic asociaz:hipogice)ie coag!opatie, ence#aopatie0

Alte manifestri ce apar 3n cadrul insuficienei hepatice fulminante sunt: alterareastrii generale febr mialgii (rsturi diaree2 incetrul poate apare mai trziu maiales 3n bolile metabolice.

Se)nee $e aar) ae progresiei %oii s!nt"

hipoglicemie creterea necesarului de glucoz2

scderea ureei albuminei colesterolului

creterea T fr rspuns la administrarea de (itamin ) parenteral

icter persistent cu creterea rapid a bilirubinei 3n asociere cu scderea

progresi( a transaminazelor

accentuarea letargiei halucinaii

rar diateze hemnoragice colaps.

data instalat insuficiena hepatic icterul este accentuat iar foetor,ul hepatice(ident.

Stadializarea insuficienei hepatice acuteTabel nr.$0

Tabel nr.$

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>tadiul =anifestri clinice Dlapping tremor =anifestri 77E

I,prodromalsubicteric

lentoare mentalusoar tulburri aleciclului somn,(eghe

discret minime

II,comiminent

somnolentconfuziecomportamentinadec(at"bizardezorientareoscilaii"schimbride dispoziie

uor dee(ideniat

ritm lentgeneralizat

III,stupor foarte somnolent dar poate fi trezit nurspunde la comenzi(erbale confuziemarcat delirhiperreflexies.9abins

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Transaminazele: A>T A@T cu (alori !*%%% GI"@pot aCunge pana la *%%%%

GI"@0

>cderea rapid a transaminazelor semnific: epuizarea masei hepatocitare i

insuficien hepatic terminal sau din contra recuperare hepatic e(ident cuameliorarea coagulrii si diminuarea encefalopatiei.

9ilirubina poate aCunge la (alori de $%%,*$%% micromol"@ prin catabolismul

hemului la ni(el hepatic"ca urmare a hemolizei. 4ea mai mare parte este subform conCugat i rele( disfuncia secretorie a hepatocitelor (iabile2 sprefinal bilirubina este neconCugata prin pierderea capacitii de conCugare0.

Icterul marcat este tipic pentru I6A se(er cu necroz

In intoxicaia cu aracetamol"Infecia cu ;69"bilile de metabolism,icterul estemoderat"absent.

Amoniemie crescut $,8H&0pn la peste *%% micromol"@

4reatinina crescut ca urmare a complicaiilor renale

Greea poate fi crescut prin disfuncie renal hemoragie digesti(

deshidratare sau sczut prin insuficien de producie hepatic 6ipoglicemie care este greu de corectat

/eficit al factorilor de coagulare ducnd la coagulopatie de consum

Anomalii electrolitice ce se 3nsoesc de (rsturi deshidratare

Eazele arteriale rele( alcaloz respiratorie"metabolic acidoz metabolic

6emoleucograma poate e(idenia leucocitoz 3n cadrul infeciilorbacteriene iar trombocitele pot fi sczute prin producie sczut sauconsum.

Managementul insuficienei hepatice acute: suport hepatic,pre(enirea itratarea complicaiilor, transplantul hepatic.

4riteriile de admisie 3n terapie acut sunt: encefalopatie hemoragie digesti(insuficien renal acut insuficien respiratorie infecii se(ere tulburri decoagulare hipoglicemie dificil de corectat acidoz lactic

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arametrii urmrii i ritmul monitorizrii acestora sunt Tabel nr.50

4linic T% /inor 3nor

@a $ore

@a Jore

@a *$ore

@a $Bore

araclinic F

TA F F F 6@E

D4 F F F ionogram

D' F F F F F A>T'G

>a$ F F F Greecreatinina

;4 F F F F F glicemie

diurez F F F trasaminaze

>tatusneurologic

F F F 9ilirubintotal"direct

9ilanhidric

F F F amoniemie

greutate F F F hemostaz

temperatura F F 7)E

F F 7cografieabdominal

F F 77E

=suri generale de 3ngriCire:

e(itarea sedrii/?0"utilizarea corticoterapiei

confort termictendin la hipotermie0

manipulare minim

monitorizarea D4 D' TA ;4 temperaturii statusulul neurologic

abord (enos prin 4;4,determinarea ;4 administrarea de lichide

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sond nazogastric pentru detectarea 6/> administrarea medicaiei

sond urinar pentru monitorizarea diurezei i implicit a funciei renale

antibioterapie

efectuarea de analize,biochimice: bilirubin transaminaze albumin ureeelectrolii calciu fosfor amoniemie 'A glicemie acid lactic2 hematologice,6@E T D. ; ;II2 serologii pentru hepatite cupremie ceruloplasminaautoanticorpi2 analiza urinii pentru metabolii toxici amoniac.

>uport nutriional

cantitatea de lichide administrat este funcie de diurez preKena sau

nu a edemuluzi cerebral ni(elul sodiului din snge dar in general estenecesar restricia de lichide2

caloriile se administreaz sub form de glucoz *%,*+L dac exist un

necesar crescut de glucoz se (a administra pe 4;40

este necesar restricia proteic:%8,*g")gc"zi parenteral2

electroliii se (or administra: &a#*mmol")gc"zi )#5,J mmol")gc"zi

este necesar asigurarea perfuziei renale prin administrarea de

Durosemid: *,5 mg")gc la inter(al de J ore sau plasm proasptcongelat. In caz de oligurie"anurie este necesar hemofiltrarea"dializa.

Co)picaiie -i pre'enirea acestora

*. 6ipoglicemia MB%%mg"@0 apare la maCoritatea copiilor i poate contribui ladeteriorarea neurologic i disfuncia multipl de organ. Dactorii

precipitani ai hipoglicemiei sunt: insuficiena produciei hepaticehiperinsulinemia creterea utilizrii glucozei ca urmare a infeciilorbacteriene.

7ste necesar monitorizarea la $,B ore a glucozei i administrarea i( de glucoz*%,+%L dextroz0.

$. 4oagulopatia i manifestrile hemoragice.

Tulburrile hemostazei apar ca urmare a insuficienei produciei hepatice afactorilor de coagulare I II ; ;II H0 ce duce la alungirea T si a TT de

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asemenea se produce consecuti( scderii numrului"funciei trombocitelor saucoagulrii intra(asculare

7(aluarea se(eritii funcie de I,Tabel nr. B

Dorma IL din (aloarea normal0

uoar N%,-%

medie +%,J-

se(er *%,B-

letal M*%

=edicamentele utilizate sunt: Ditomenadiona,%$ mg")gc"zi maxim *% mg"zi 5zile snge proaspt *%,$% ml")gc la J ore inter(al crioprecipitat. >ngerareacti(mas trombocitar $G"*% ) TrM+%%%%"mm50 plasm proaspt congelat

*%,*+ ml")gc rD;IIa J%,*$% microg")gc i( la $,5 ore *%,$% microg")gc"h0

5. rofilaxia 6/>: doze mari de antagoniti,6$: 'anitidin,*,5 mg")gcH5inhibitori ai pompei de protoni:meprezol,*%,$% mg")g"zi >ucralfat*+

mg")gcHB"zi

B. 7ncefalopatia hepatic necesit: restricie proteic e(itareasedati(elor"barbituricelor &eomicin: +% mg")gc"zi la J ore po @actuloz,*+,5%ml la J ore ulterior doza se aCusteaz pentru a produce $,5 scaune moi"zi clismae(acuatorie intubare 3n std. III"I; al encefalopatiei tratamentul prompt alcomplicaiilor ce cresc amoniogeneza: 6/> tulburrile hidroelectrolitice iacidobazice hipoglicemia

+. 4ombaterea edemului cerebral apare de obicei 3n stadiile II,I;0:poziionarea bolna(ului,ridicarea trunchiului la 5% grade hiper(entilaie moderatrestricie de fluide controlul hiponatremiei =anitol:%+g")gc la B,J orehemofiltrare.

J. Tulburrile hidroelectrolitice i acidobazice

,mai frec(ent este hiponatremia ce apare prin scderea excreiei de ap cretereaA/6 tulburri ale pompei de &a,) administrarea excesi( de soluii hipotone2

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,hipernatremia,apare prin administrarea i( de fluide ce conin &a folosireaexcesi( a @actulozei"=anitolului

,hipoe urmreste meninerea unei diureze!%+ml")gc"h. /ac se instaleaz I'A este necesar hemodializa

*. Ascita apare prin hipertensiune portal acut colaps lobular. &ecesitcorecia presiunii oncotice cu albumin i corectarea tulburrilorhidroelectrolitice.

$. 4omplicaiile cardio(asculare i pulmonare:

,in hipotensiunea arteriala determinat de hemoragie bacteriemie0 este necesarrefacerea (olemiei i administrarea de ageni presori /opamina"/obutamina0

6ipotensiunea arterial asociat cu (asodilataie periferic i acidoz metabolicsunt indicatori de moarte iminentOO

, 7A necesit administrarea de $ *%%L (entilaie mecanic

**. ancreatita necesit repaus digesti( e(itarea laparotomiei

*$. Infeciile respiratorii urinare de cateter0 necesit inter(enie prompt prinantibioterapie agresi( se e(it aminoglicozidele a(nd 3n (edere efectul lornefrotoxic0

Transpant! hepatic ortotopic

Indicaii: stadiul II"I; de encefalopatie boli ire(ersibile:b. ilson hepatita 9 4tirozinemia tip I hepatita toxic de cauz nedeterminat icter prelungit!N zile

anterior debutului encefalopatiei T mult alungit!+% sec0 hipoglicemie sauacidoz metabolica se(er

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4ontraindicaii: ischemie cerebral insuficien renal hemoragie digesti(septicemie"peritonit

actori de prognostic se!er: encefalopatie std. III"I; icter prelungit cubilirubin indirect!*+ mgL, I&'!B, T!J% sec necorectabil dup administrarea(itaminei ), TT!$% secunde fat de martor hipoglicemie se(er acidozmetabolic se(er albumin seric M$+gL, ascit refractar la diureticecolesterolemieM*%% mgL.

"ibliografie:

*. >Puires '6 Qr >hneider 9@ 9ucu(alas Q et al. Acute li(er failure inchildren: the first 5B8 patients in the pediatric acute li(er failure studKgroup. Q ediatr $%%J2 *B8:J+$.

$. >Puires '6 Qr. Acute li(er failure in children. >emin @i(er /is $%%82$8:*+5.

5. >Puires '6 Alonso 7=. Acute li(er failure in children. In: @i(er /isease in4hildren Bth ed >uchK DQ >o. Acute @i(er Dailure >tudKEroup. 4rit 4are =ed $%%N2 5+:$B-8.

8. )amat )unde > ;os = et al. In(asi(e intracranial pressure monitoring isa useful adCunct in the management of se(ere hepatic encephalopathKassociated Rith pediatric acute li(er failure. ediatr 4rit 4are =ed $%*$2*5:e55.

-. >haRcross /@ endon QA. The neurological manifestations of acute li(erfailure. &eurochem Int $%*$2 J%:JJ$.

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*%.6adzi & 6eight > 9all > et al. 7(olution in the management of acuteli(er failure,associated aplastic anaemia in children: a single centreexperience. Q 6epatol $%%82 B8:J8.

**.>chmidt @7 /alhoff ). >erum phosphate is an earlK predictor of outcome

in se(ere acetaminophen,induced hepatotoxicitK. 6epatologK $%%$2 5J:J+-.

*$.>Puires '6 /haRan A Alonso 7 et al. Intra(enous &,acetKlcKsteine inpediatric patients Rith nonacetaminophen acute li(er failure: a placebo,controlled clinical trial. 6epatologK $%*52 +N:*+B$.

*5.>undaram ; >hneider 9@ /haRan A et al. )ingUs 4ollege 6ospital4riteria for non,acetaminophen induced acute li(er failure in aninternational cohort of children. Q ediatr $%*52 *J$:5*-.

*B.@u 9 ?hang > &aruppl0:B$BA.

*+.>teRart Q/ 6or(ath ' 9aruffini 7 et al. olKmerase V gene @Edetermines the ris< of sodium (alproate,induced li(er toxicitK. 6epatologK$%*%2 +$:*N-*.

*J.@ee > >orini(asan et al. ImmunosuppressionRithdraRal after auxiliarK li(er transplantation for acute li(er failure.Transplant roc $%%+2 5N:*N$%.

*8.>oltKs )A >oto,EutiWrrez A &agaKa = et al. 9arriers to the successfultreatment of li(er disease bK hepatocKte transplantation. Q 6epatol $%*%2+5:NJ-.

*-.sacharopoulos 6T =oRat A /a(ies = et al. Dulminant hepatic failure

in childhood: an analKsis of 5* cases. Arch /is 4hild *-8%2 ++:$+$.$%.>undaram >> Alonso 7= &ar