Ciroza Flashcards

1
Q

Alte cauze de ciroza

A

Ciroza biliara secundara = CBS
Colangita biliara primitiva = CBP
Hemocromatoza ereditara
Boala Wilson
Deficit de alfa1-antitripsina (A1AT)
FC
Glicogenozele
Galactozemia
Idiopatica (criptogenica)
Sindromul Budd-Chiari
Boala veno-ocluziva
Congestia venoasa hepatica
Medicamente - metotrexat (MTX)
Hepatita autoimuna
Alte virusuri

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2
Q

Consecintele afectarii cronice hepatice

A

Inflamatie
Necroza
Angiogeneza
Depunere de matrice extracelulara

! Toate duc la fibroza

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3
Q

Investigatii pentru evaluarea severitatii cirozei

A

Functia hepatica - albumina serica, PT
Biochimie hepatica - transaminaze, FA
Electroliti - Na
Creatinina
Biomarkeri - ELF (fibroza hepatica)

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4
Q

Gradul fibrozei - scorul ELF (enhanced liver fibrosis)

A
  • < 7.7 = absenta/usoara
  • 7.7-9.8 = moderata
  • > 9.8 = severa
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5
Q

Investigatii pentru a determina tipul cirozei

A

Markeri virali
Autoanticorpi serici
Ig serice
Fe, feritina, CTLF
Cu, ceruloplasmina
Alfa1-antitripsina
Markeri genetici

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6
Q

Imagistica in ciroza

A

Ecografie abdominala
Fibroscan = elastografie tranzitorie unidimensionala
CT
IRM
Endoscopie

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7
Q

Ce poate identifica IHC pe fragmentul de biopsie hepatica?

A

Ducte biliare
Structuri angiogenice
Virusuri
Markeri oncogeni

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8
Q

Coloratii utilizate pe fragmentele de biopsie hepatica

A

H&E
Giemsa
Rosu picrosirius

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9
Q

Coloratie pentru colagen

A

Rosu picrosirius
* Pentru evaluarea morfometrica a fibrozei (cuantifica colagenul)

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10
Q

Factori de prognostic nefavorabil in ciroza - analize

A
  • Alb < 28 g/L (2.8 g/dL)
  • Na < 125 mmoli/L
  • PT > 6s peste N
  • Cr > 130 mcmoli/L (1.5 mg/dL)
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11
Q

Factori de prognostic nefavorabil in ciroza - clinica

A

Icter persistent
hTA persistenta
Ficat mic
Ascita
Hemoragie din varice
Complicatii neuropsihiatrice
Esecul terapiei
Etiologia

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12
Q

Ce parametri se iau in considerare la calcularea clasei Child?

A

Ascita
Encefalopatia
Bilirubina
Albumina
PT

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13
Q

Pe ce parametri se concentreaza clasa Child si scorul MELD?

A

Bilirubina
Creatinina
INR

! Sunt predictori ai mortalitatii la cei cu necesar de transplant hepatic

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14
Q

Caracteristici ACLF

A

Insuficienta organica
Rata mare de mortalitate
Aparitia la varste tinere
Etiologie alcoolica
Nivel mare de inflamatie sistemica
Prevalenta mai mare a anumitor factori precipitanti (infectii bacteriene, exces de alcool)

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15
Q

Dispozitive de asistare a ficatului

A

Dispozitive biologice care contin hepatocite
Dispozitived de detoxifiere
Sisteme artificiale de sustinere a ficatului

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16
Q

Cauzele afectarii functiei de bariera intestinala in bolile hepatice avansate

A

Modificarea motilitatii intestinale
Cresterea permeabilitatii intestinale
Suprimarea functiilor imunologice intestinale

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17
Q

Indicatii transplant hepatic

A
  1. Boli hepatice acute - IHA de orice cauza
  2. Boli hepatice cronice
  3. Colangita biliara primitiva - prurit intratabil/bilirubina > 100
  4. Hepatita B cronica
  5. Hepatita C cronica
  6. Hepatita autoimuna
  7. Boala hepatica alcoolica
  8. Tulburari metabolice primare - Wilson, hemocromatoza ereditara, deficit de A1AT
  9. Ciroza NASH
  10. Altele - CSP, polichistoza hepatica, oxaluria primara
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18
Q

Contraindicatii absolute transplant hepatic

A

Sepsis activ (alt teritoriu decat arborele hepatobiliar)
Cancere extrahepatice
Metastaze hepatice (exceptie neuroendocrine)
Lipsa angajamentului pshiologic al pacientului

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19
Q

Pregatirea pentru interventia de transplant

A

Confirmare diagnostic
Imagistica ecografica + sectionala
Studiu radiologic al circulatiei hepatice arteriale + al arborelui biliar
Evaluarea starii cardiorespiratorii + renale

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20
Q

Durata conservarii ficatului pentru transplant

A

20h

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21
Q

Durata operatiei de transplant hepatic

A

8h

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22
Q

Terapii imunosupresoare post-transplant hepatic

A

Tacrolimus +/- AZT/MMF
Sirolimus
Ciclosporina microemulsificata
Steroizi

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23
Q

Rejet acut/celular de grefa - aspect HP

A

Infiltrat pleomorf portal - dominat de eozinofile
Leziuni de cai biliare
Endotelita vaselor de sange

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24
Q

Supravietuire dupa transplant hepatic electiv

A

90% - 1 an
70-85% - 5 ani

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25
Boli cu recurenta ridicata post-transplant hepatic
CHC Ciroza VHC CSP
26
Prevalenta CBP
7.5/100.000
27
Denumire alternativa CBP
Colangita cronica distructiva non-supurativa
28
Clinica CBP
Prurit - cel mai precoce simptom Fatigabilitate - frecvent invalidanta Icter Hepatomegalie Xantelasma pigmentara palpebrala Depuneri de colesterol in pliurile tegumentare ale mainilor
29
Manifestari autoimune in CBP
Sjogren Tioridita Sclerodermie
30
Alte manifestari asociate cu CBP
ATR GNM Boala celiaca Pneumonie interstitiala
31
Investigatii CBP
Anticorpi mitocondriali - AMA M2 > 1:160 (pot fi prezenti si altii nespecifici = ANA, ASMA) FA crescuta - adesea singura anomalie Cho crescut IgM foarte mare Ecografie - modificari difuze de arhitectura hepatica Biopsie hepatica
32
Aspect biopsie in CBP
Modificari timpurii (majoritatea in zona 1) * Infiltrat portal limfoplasmocitar * Granuloame (40% cazuri) Modificari tardive * Deteriorarea si rarefierea ductelor biliare mici * Proliferare ductulara * Fibroza tractelor portale * Ciroza
33
In ce boli se observa granuloame hepatice pe biopsie?
CBP Reactii medicamentoase Sarcoidoza TB Bruceloza Schistosomiaza Parazitoze - strongiloidoza
34
Caracteristici colangita autoimuna
Histologie de CBP Serologie de hepatita autoimuna Raspunde la steroizi + AZT
35
Tratament CBP
Acid ursodezoxicolic (10-15 mg/kg) - in boala precoce; alternativa = acid obeticholic Steroizii - preferabil nu Suplimentare vitamine liposolubile Bifosfonati - pentru osteoporoza Colestiramina (da indigestie) - pentru prurit Alte medicamente pentru prurit * Rifampicina * Naloxona * Naltrexona Pruritul intratabil se amelioreaza cu plasmafereza/MARS (sistem molecular recirculant absorbant) Transplant hepatic - esecul terapiei medicale
36
Complicatii CBP
Complicatiile cirozei Osteoporoza Osteomalacie - mai rar Polineuropatie - mai rar
37
Efecte benefice AB + BB neselective in ciroza
Blocheaza translocatia bacteriana Reduc presiunea portala Reduc hemoragia variceala Reduc PBS
38
Ce inseamna ciroza in stadiu final?
Chid C MELD 20+ UKELD 49+
39
Mortalitate donatori LDH
1:200 -> 1:400
40
Cauze CSP secundara
HIV Cryptosporidioza Consum de ketamina
41
Cauze de CBS
Stenoze de CBP Calculi biliari Colangita sclerozanta
42
Moduri de evidentiere arbore biliar
Ecografie MRCP ERCP - uneori Colangiografie transhepatica percutana - evidentierea CBIH cand abordarea endoscopica este dificila
43
Factori care influenteaza cursul bolii in HCE
Genul Aportul alimentar de Fe Hepatotoxicele asociate (mai ales alcool) Genotipuri
44
Triada clasica in HCE
Hiperpigmentare cutanata Hmegalie DZ Doar in cazuri de supraincarcare importanta de Fe
45
Utilitatea biopsiei hepatice in HCE
Stabilirea dimensiunii lezarii tisulare Evaluarea fierului tisular Masurarea concentratiei hepatice de Fe
46
Ce concentratie hepatica de Fe sugereaza HC?
> 180 mcmoli/g de greutate uscata a fragmentului biopsiat
47
Ce inseamna supraincarcare hepatica cu Fe relevanta clinic?
> 60 mcmoli/g de ficat
48
Manifestari neurologice in boala Wilson
Dizartrie Tremor Miscari involuntare Dementa
49
RA grave la penicilamina
Modificari ale pielii Eruptii cutanate Leziuni renale Leucopenie
50
Complicatii si efecte ale cirozei
HTP Hemoragie GI Ascita EPS CHC Bacteriemii, infectii IR Sindrom hepatopulmonar
51
Cauze de HTP
Prehepatica - tromboza v porta Posthepatica * ICD (rar) * Pericardita constrictiva * Obstructie VCI Hepatica a) Presinusoidala * Schistosomiaza * Sarcoidoza * CBP b) Sinusoidala * Ciroza (ex. alcoolica) * Transformare nodulara partiala * FHC c) Postsinusoidala * BVO * Budd-Chiari
52
Principalele locuri de aparitie a colateralelor port-sistemice
JEG Rect Vena renala stanga Retroperitoneu Diafragm Perete abdominal anterior (vena ombilicala)
53
Cauze de HTP extrahepatica prin tromboza venei porte
Anomalii venoase portale congenitale Sepsis neonatal de vena ombilicala Afectiuni protrombotice mostenite * Factor V Leiden * Boli mieloproliferative +/- mutatii JAK2
54
Cauze intrahepatice de HTP
Ciroza - cea mai frecventa Schistosomiaza Alte cauze * FHC * Hiperplazia nodulara regenerativa * Transformarea nodulara partiala
55
Complicatii tamponament cu balon
Pneumonie de aspiratie Ulceratia mucoasei Ruptura esofagiana
56
Avantaje stent Danis
Nu afecteaza deglutitia Nu poate fi indepartat de pacientii necooperanti Permite investigatiile post-endoscopice
57
Complicatii bandare varice esofagiene
Ulceratii esofagiene Mediastinita Stenoze (mai rar)
58
Factori precipitanti ascita
Continuarea consumului excesiv de alcool Infectie/sepsis CHC Tromboza de vene splanhnice
59
Cauze de gradient albumina ser/ascita > 11 g/L
HTP - ciroza Sindrom Budd-Chiari BVO Obstructia fluxului hepatic de iesire Insuficienta tricuspidiana (IT) ICD Pericardita constrictiva
60
Cauze de gradient albumina ser-ascita < 11 g/L
Carcinomatoza peritoneala TB peritoneala Pancreatita Sindrom nefrotic
61
Cauza de ascita serocitrina
Cancer - cea mai frecventa Ciroza Infectii * TB * Perforatie intraabdominala cu orice bacterie (E. coli de ex) * Spontan (ciroza - PBS) Sindrom Budd-Chiari - proteine multe in lichid Pancreatita CRONICA ICC Pericardita constrictiva Hipoproteinemie (sindrom nefrotic) Sindrom Meigs (tumora ovariana)
62
Cauze de ascita chiloasa
Ciroza Obstructia canalului limfatic principal - carcinoame - prezenti chilomicroni in lichid
63
Cauze de ascita hemoragica
Cancer SEU rupta Traumatism abdominal Pancreatita ACUTA
64
Dezavantaje diuretice de ansa
HipoNa HipoK Depletie volemica
65
Bacterii frecvent implicate in PBS
E. coli Klebsiella Enterococi