Cirrhosis Flashcards
What is cirrhosis?
Liver damage characterised by diffuse distortion of basic architecture and replacement with scar tissue and formation of regenerative nodules.
What are the common causes of cirrhosis?
- Fatty liver (alcohol, metabolic syndrome)
- Chronic viral hepatitis: Hep B +/- D, Hep C
What are the less common causes of cirrhosis?
1) Primary biliary cirrhosis
2) AI hepatitis
3) Haemochromatosis
4) Chronic Hepatic venous congestion: cardiac cirrhosis, Budd Chiari syndrome.
6) Wilson’s disease
7) Drugs (e.g. methotrexate)
8) a1 anti-trypsin deficiency
10) Glycogen storage disease
What initiates liver fibrosis?
Stellate cells, activated by cytokines and ROS
What occurs in liver fibrosis?
In the space of Disse, normal matrix is replaced by collagens (predominately 1 and 3), and fibronectin.
Subendothelial fibrosis -> loss of endothelial fenestration -> impaired function.
What are the characteristic features of liver cirrhosis?
Regenerating nodules separated by fibrous septa with loss of the normal lobular architecture within the nodules.
What is micronodular cirrhosis?
Regenerating nodules
What is macronodular cirrhosis?
Regenerating nodules >3mm
What are the first tests to order in suspected cirrhosis?
LFTs (deranged) GGT (elevated) Serum Albumin (reduced) Serum Sodium (reduced) APTT (prolonged) Platelet count (reduced) Hep B/C Abs
What are the best indicators of liver function?
Serum albumin and prothrombin time.
What causes hyponatremia of patients with cirrhosis?
Low sodium indicates severe liver disease due to a defect in free water clearance or to excess diuretic therapy.
What imaging utilised to assess/diagnose cirrhosis? Outline expected features.
US: liver surface nodularity, changes to liver size, ascites, splenomegaly, increased diameter of portal vein.
CT: hepatosplenomegaly, ascites, collateral circulation (varices), liver surface texture.
What imaging can be used to detect HCC?
Arterial phase contrast CTA
How is cirrhosis classified?
- Compensated: findings consistent with cirrhosis present but hepatic synthetic function preserved with no evidence of complications of portal HTN.
- Decompensated: complications of liver dysfunction with decreased synthesis and portal HTN.
What are the RFx for cirrhosis?
Alcohol abuse, IVDU, unprotected sex, obesity, blood transfusion.
What are the key diagnostic features of cirrhosis on Hx and Ex?
- Presence of RFx
- Abdo distension
- Jaundice and pruritus
- Coffee ground vomitus and maelena
- Hand and nail features (leukonychia, palmar erythema, spider angiomata)
- Facial features (jaundice, telangiectasia)
How is cirrhosis severity scored?
Child Pugh and MELD
What is the Child Pugh scoring system?
CP A, B or C based on:
Ascites, hepatic encephalopathy, serum bilirubin, albumin, APTT and INR.
A = well compensated –> B = decompensated
How is cirrhosis managed?
- Treatment of underlying chronic liver disease and prevention of superimposed hepatic insult
- Monitor for complications
- Sodium restriction and diuretic therapy for ascites
- 2nd = liver transplant
What are the common causes of death in cirrhotic patients?
- Renal failure (hepatorenal syndrome)
- Sepsis
- GI bleed
- HCC
What are the stages of cirrhosis?
- S1: compensated and asymptomatic. Can last 10-20y w/ norm life expectancy
- S2: onset first decompensation (typically development of ascites, variceal bleeding, encephalopathy).
Complications of cirrhosis?
VARICES
- Varices
- Anaemia
- Renal failure
- Infection
- Coagulopathy
- Encephalopathy
- Sepsis
What are the haematology complications of cirrhosis?
- Pancytopenia (from hypersplenism): first platelets > WBC >Hb.
- Decreased clotting factor synthesis = prolonged INR, bruising and bleeding.
What proportion of patients with cirrhosis develop varices / vatical haemorrhage?
- 50% cirrhotics will develop varies
- ~30% develop haemorrhage (mortality then = 30%+)
What is the strongest predictor of variceal development in cirrhosis?
hepatic venous pressure gradient >10mmHg.
Management of oesophageal varies?
Resuscitation, ABx, vasoactive drugs + endoscopic band ligation or sclerotherapy, TIPS.
What are the classifications of renal failure in cirrhosis?
- Pre renal (usually over diuresis)
- Acute tubular necrosis
- Hepatorenal syndrome
What are the types of hepatorenal syndrome?
- Type I: sudden and acute renal failure (rapid doubling of Cr over 2/52)
- Type II: gradual increase in Cr with worsening liver function (Cr doubling over years)
When is hepatorenal syndrome likely to develop?
- overdiuresis or dehydration (e.g. V/Diarrhoea)
- GI bleed
- Sepsis
Treatment for type I hepatorenal syndrome?
-Octreotide + midodrine + albumin
Increases renal blood flow by increasing systemic vascular resistance.
Definitive Rx = liver transplant.
What is the presumed aetiology of hepatopulmonary syndrome?
?VQ mismatch, intrapulmonary shunting and limitation of O2 diffusion. Relates to failure of damaged liver to clear circulating pulmonary vasodilators vs production of vasodilating substance by liver.
What is the clinical triad of hepatopulmonary syndrome?
- Liver disease
- Increase A-a gradient on RA
- Evidence of intrapulmonary vascular abnormalities
What is platypnea?
Increase in dyspnoea when upright, improved with recumbency.
What is orthodeoxia?
Desaturation in upright position, improved with recumbency.
What are the clinical features of hepatopulmonary syndrome?
- Dyspnoea, platypnea, orthodeoxia.
- Hyperdynamic circulation with CO>7L/min at rest.
- Decreased pulmonary and systemic resistance (intrapulmonary shunting)
How is hepatopulmonary syndrome diagnosed?
Contrast enhanced echo:
inject air bubbles into peripheral vein; air bubbles appear in left ventricle after 3rd heartbeat (normal = no air bubbles; VSD = air bubbles
Rx hepatopulmonary syndrome?
Only proven treatment is transplant.
