sindrom icteric
TRANSCRIPT
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SINDROAMELE ICTERICE
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• Definitie:
Icterul este un sindrom caracterizat prin coloratia galbena a tegumentelor si mucoaselor datorita depunerii in tesuturi a bilirubinei
• Clinic, icterul apare daca bilirubina depaseste 2-3 mg%
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!!
XANTHOPSIAXANTHOPSIA
= o tulburare vizuala in care = o tulburare vizuala in care obiectele apar colorate in obiectele apar colorate in galben, prin modificare ocularagalben, prin modificare oculara
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Diagnostic diferentialDiagnostic diferential
hipercarotenemiehipercarotenemie
insuficienta renala cronicainsuficienta renala cronica
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hipercarotenemie primarahipercarotenemie primara (CAROTENODERMA) (CAROTENODERMA)
hipercarotenemia se poate intilni in: hipercarotenemia se poate intilni in:
- Mixedem- Mixedem- Diabet zaharat- Diabet zaharat- Insuficienta renala cronica- Insuficienta renala cronica- Eunuci- Eunuci
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! Carotenodermia se diferentiaza de icter, ! Carotenodermia se diferentiaza de icter, prin: prin:
culoarea conjunctiveiculoarea conjunctivei urina urina scaunscaun
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METABOLISMUL BILIRUBINEI
a). Sursele bilirubinei
b). Transportul bilirubinei
c). Metabolismul hepatic al bilirubinei
d). Faza intestinala
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distrugerea hematiilor imbatrinite(80 – 85%)
distrugerea celulelor eritroide imature
hemoglobina
globina
hem
bilirubin glucuronid
hem - oxigenaza
bilirubina
biliverdina
biliverdin - reductaza
glucuronil transferaza
urobilinogen
stercobilinogen
- bilirubina este produsul final al degradarii hemului
- bilirubina neconjugata este legata de albumine si transportata la ficat
- bilirubina este preluata de sinusoidele hepatice, patrunde in hepatocit si se conjuga cu acidul glucuronic, devenind hidrosolubila
- bilirubin glucuronizii netoxici sunt excretati in bila
- majoritatea glucuronizilor sunt deconjugati in intestin de flora microbiana si transformati in urobilinogen
- 20% din urobilinogen se reabsoarbe in ileon si colon, majoritatea intorcindse la ficat si o mica parte se elimina renal
- urobilinogenul ramas in intestin se transforma in stercobilina si se elimina in scaun
alte hemoproteinemioglobina, citocromi,catalaze, peroxidaze (15-20%)
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Tipuri de bilirubina
Bilirubina neconjugata (indirecta) - reactioneaza cu reactivul Van den Bergh numai dupa adaugare de alcool - liposolubila - nu se elimina renal - este toxica pentru multe celule si organe - traverseaza placenta, epiteliul intestinal, epiteliul vezicii biliare, bariera
hematoencefalica
Bilirubina conjugata (directa)
- reactioneaza direct cu reactivul Van den Bergh
- hidrosolubila
- nu are afinitate pentru grasimi
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DIAGNOSTICUL ICTERULUICuloarea tegumentelor
si a mucoaselor
flavinic
rubinic
verdinic
melanic
Bilirubinemie
indirecta
directa
mixta
Aspectul scaunului
acolic
normocrom
pleiocrom
Examenul urinii
urobilinogen
bilirubina directa
saruri biliare
prehepatic
hepatic
post hepatic
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Examinarea pielii se face la lumina zilei !Examinarea pielii se face la lumina zilei !
conjunctiva conjunctiva sub limbasub limba palatpalat buzebuze
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Prehepatic
supraproductie de bilirubina
alterarea preluarii din singe a bilirubinei si transport deficitar la nivelul hepatocitului
perturbarea conjugarii
Hepatic
distructii hepatocitare
perturbarea functiei de preluare, transport sau conjugare a bilirubinemiei
perturbarea eliminarii bilei(colestaza intrahepatica)
Posthepatic(colestataza extrahepatica)
perturbarea eliminarii bilei
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Clasificarea icterelor
• Icter cu predominenta bilirubinei indirecte
• Icter cu predominenta bilirubinei directe
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CLASIFICAREA ICTERELOR
I. Exces de productie a bilirubinei (icter hemolitic)
IIa. Defect hepatic de captare a bilirubinei
IIb. Defect hepatic de conjugare a bilirubinei
IIc. Defect de excretie hepatica a bilirubinei
III. Defect de excretie biliara a bilirubinei (icter obstructiv)
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ICTERUL HEMOLITIC
• apare in conditii de hemoliza patologica• bilirubina indirecta este mai mare de 1 mg/dL, dar rareori depaseste 6 mg/dL (icter moderat)• intensitatea icterului variaza in raport cu numarul de eritrocite distruse
CLINIC• icter si paloare, fara prurit, fara bradicardie• splenomegalie• posibil convulsii si tulburari respiratorii• posibil litiaza biliara
hemoliza compensata: hemoliza fara anemiehemoliza acuta intravascularahemoliza extravasculara
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ICTERUL HEMOLITIC
Cauze de hemoliza:
1. intracorpusculare - anomalii de membrana eritrocitara(microsferocitoza,ovalocitoza,..) - anomalii de incarcare enzimatica eritrocitara (deficit de G6PD,deficit de PK,deficit de hexochinaza) - anomalii in structura hemoglobinei(drepanocitoza- Hb S, talasemie)
2.Hemoliza de cauza extracorpusculara: - imunologica-izoAtc(hemoliza posttransfuzionala),autoAtc - agresiune oxidanta - agresiune mecanica - agresiune infectioasa - agresiune termica - toxice(venin de sarpe..)
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ICTERUL HEMOLITIC PARACLINIC• bilirubinemie indirecta > 1 mg/dL• anemie normocroma, micro- sau macrocitara, hiperegenerativa• dozarea de LDH si haptoglobina-teste majore de hemoliza (LDH crescut cu haptoglobina redusa-90% hemoliza)• frotiu sanguin: elemente comune ale hemolizei si elemente caracteristice fiecarei cauze de hemoliza• reticulocite crescute• medulograma: hiperplazie eritroida cu indice de eritroblastoza 40-50%• sideremie normala sau crescuta
DIAGNOSTIC DIFERENTIAL
1. icter prin hiperproductia bilirubinei precoce (anemia Biermer )
2. deficit de metabolizare a bilirubinei indirecte
(icterul fiziologic al nou- nascutului, ictere familiale)
3. ictere medicamentoase
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ICTERE PRIN DEFICIT DE CAPTARE HEPATICA
SINDROMUL GILBERT < 12% din populatie(icterul familial nehemolitic, colemia familiala)
Transmitere autosomal dominanta Are mecanism dublu: deficit de captare si deficit de conjugare hepatica
Clinic: icter cronic de intensitate moderata aparut in copilarie sau adolescenta
mai ales la barbati ficatul si splina sunt in limite normale icterul se accentueaza dupa diete hipocalorice sau hipolipidice
bilirubina indirecta crescuta ~ 3 mg/dL teste functionale hepatice normale
diagnosticul este de excludere
evolutie benigna Nu exista tratament specific. Se poate administra fenobarbital
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ICTERE PRIN DEFICIT DE GLUCURONOCONJUGARE
I. SINDROMUL CRIGLER-NAJJAR (icter nehemolitic congenital) Deficit de UDP-GT
Tipul ITransmitere autozomal recesivabilirubinemia indirecta > 17- 48 mg/dLmanifestari neurologiceevolutie spre exitus
Tipul IITransmitere autosomal dominantabilirubinemia indirecta > 5- 23 mg/dLmanifestari neurologice tratament: fenobarbital
In ambele cazuri ficatul si splina sunt in limite normale
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ICTERE PRIN DEFICIT DE GLUCURONOCONJUGARE
II . ICTERUL FIZIOLOGIC AL NOU- NASCUTULUI datorat imaturitatii functionale a UDP-GT
apare in zilele 3-5 postpartum si persista cca.5 zile
are intensitate moderata
III. SINDROMUL LUCEY- DRISCOLL este o prelungire a icterului fiziologic al nou-nascutului se datoreaza excretiei prin laptele matern a pregnadiolului care inhiba
UDP-GT rareori bilirubinemia indirecta depaseste 5 mg/dL
IV. ICTERUL MEDICAMENTOS prin medicamnete care inhiba UDP-GT (novobiocin, rifampicina…. )
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ICTERE PRIN DEFICIT DE EXCRETIE HEPATOCITARA Se caracterizeza prin hiperbilirubinemie mixta, cu predominenta formei directe
1. SINDROMUL DUBIN-JOHNSONtransmitere autozomal recesivadeficit de excretie a anionilor organici CLINIC: icter de intensitate moderata, uneori prurit, hepatomegalie moderata, dureroasa, rareori splenomegalie
probele de colestaza sunt normalebiopsie hepatica-pigment brun lizozomalcolecistografie negativaevolutie cronica, in puseenu necesita tratament
2. SINDROMUL ROTOR
afectiune rara care se deosebeste de sindromul Dubin-Johnson prin:- colecistografia i.v. este pozitiva- lipsa pigmentului brun hepatocitar
prognostic bunnu necesita tratament
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ICTERE PRIN DEFICIT DE EXCRETIE HEPATOCITARA
3. Hepatitele acute si cronice si ciroza hepatica se insotesc de hiperbilirubinemie mixta
4. Icterul recurent din sarcina apare mai ales in trimestrul III si dispare la 7- 14 zile postpartum bilirubina directa crescuta pana la 6 mg/dL, fosfataza alcalina posibil crescuta
5. Icterul postoperator
apare dupa operatii extinse
Cauze: - crestera productiei de bilirubina
- afectarea functiei hepatocitare
- obstructia cailor biliare extrahepatice
Apare in zilele 1-10 postoperator si se rezolva spontan in general
Cresc: fosfataza alcalina, 5 nucleotidaza, transaminazele
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ICTERE PRIN DEFICIT DE EXCRETIE HEPATOCITARA
6. ICTERELE MEDICAMENTOASE steroizi anabolizanti estrogeni tranchilizante (clorpropamida) antitiroidiene hipoglicemiante orale eritromicina tuberculostatice
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COLESTAZA
sindrom clinic si biochimic determinat de un obstacol in calea fluxului biliar cauze: intrahepatice extrahepatice
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Colestaza cronica
hiperpigmentare
excoriatii / leziuni de grataj
diateza hemoragica
xantelasme, xantoame
osteoporoza
Intrahepatica
prodrom viral
IH HP
Hepatita virala
Drog/ alcool
Boala hepatica cronica
Extrahepatica
durere biliara/ pancreatica
febra
vezica biliara palpabila
litiaza colecist/coledoc
cancer cap pancreas
altele
fosfataza alcalina
5´nucleotidaza
transaminaze
CT
ERCP
Biopsie…
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Ictere prin colestaza intrahepatica
Cauze: - hepatite acute virale - hepatita alcoolica - steatoza hepatica nealcoolica - ciroza biliara primitiva - colestaza medicamentoasa sau toxica - stari septice - neoplazii - nutritia parenterala totala - transplantul de organ - leucemia cu celule paroase - boli infiltrative hepatice
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ICTERE PRIN OBSTRUCTIE BILIARA EXTRAHEPATICA
ETIOLOGIE
Neoplasm (cai biliare, cap de pancreas)Litiaza coledoculuiStenoza coledocului (oddita scleroasa, coledocita, papilita, stenoza postoperatorie)Pancreatita cronicaCompresiune coledociana extrinsecaParaziti intestinali migrati in coledoc COMPLICATII
infectioasehemoragicemalabsorbtieciroza biliara secundara
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Sindromul icteric – urgenta medicala
sindrom HELLP
icter postnatal
hemoliza intravasculara
colangita ascendenta
insuficienta hepatica fulminanta
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ETAPELE DE DIAGNOSTIC ALE UNUI SINDROM ICTERIC
• Etapa clinica - anamneza - examinarea tegumentelor si mucoaselor - examenul urinii si scaunului - examenul abdomenului
• Investigatii - singe - ecografie abdominala, CT, - ERCP
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ANAMNEZA
Ce modificari ale urinii si scaunului a avut?
A avut in ultimele 2-3 luni interventii chirurgicale/ stomatologice?
A calatorit in ultimul timp?
A facut baie intr-un riu?
Consuma cronic alcool?
Ce medicamente a luat in ultimul timp?
A mai avut modificari de culoare ale conjunctivelor, urinii si scaunului?
Sunt in familie sau printre prieteni persoane ce au avut icter?
varstaprofesie si obiceiuridurere abdominala, scadere ponderala, prurit, greata si varsaturidebut: brusc, progresiv, cu febra
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hemoliza
hematom
afectiune
hepato - celularaobstructie biliara extrahepatica
Icter mediu + urina neinchisa la culoare = sindrom Gilbert
Hiperbilirubinemie directa necolestatica = sindrom Dubin Johnson
Flavin
Orange
Rubin
Verdin
Melas
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ICTER EXEMPLIFICARI
icter precedat de greata, varsaturi
hepatita virala litiaza biliara (Murphy)
urina inchisa la culoare + HP + IH boala hepatica
durere
icter
scadere ponderala cancer cap pancreas
diabet zaharat recent instalat
semn Courvoisier
icter
cachexie metastaze hepatice
ficat modificat clinic
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Icter + splenomegalie hemoliza cronica
Icter + adenopatie
acut mononucleoza infectioasa
cronic limfom
Icter + adenopatie + splenomegalie
leucemie limfatica cronica
limfom
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Algoritm diagnostic in sindromul icteric
istoric
examen clinic
teste laborator
fosfataza alcalina si transaminaze valori crescute
NU
DA
hemoliza
boli ereditare
boala hepatica
teste laborator
CT
ecografie
biopsie hepaticaecografie
cai biliare dilatate
(litiaza, pancreas, colangita)
cai biliare nedilatate (ERCP, CT)
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