Cirrhosis of liverCirrhosis of liver

Dr Aye Myint KhineDr Aye Myint Khine

At any ageAt any age Prolonged morbidityProlonged morbidity Younger adultsYounger adults Premature deathPremature death

AetiologyAetiology

Any causes of chronic hepatitisAny causes of chronic hepatitis

AlcoholAlcohol

PBCPBC

PSCPSC

Secondary biliary cirrhosisSecondary biliary cirrhosis

HaemochromatosisHaemochromatosis

Wilson’s diseaseWilson’s disease

Alpha 1 antitrypsin deficiencyAlpha 1 antitrypsin deficiency

Cystic fibrosisCystic fibrosis

Histological typeHistological type

Micronodular cirrhosis –small nodules Micronodular cirrhosis –small nodules about 1mmabout 1mm

Macronodular cirrhosis-larger nodulesMacronodular cirrhosis-larger nodules

Clinical featuresClinical features

AsymptomaticAsymptomatic

mild hepatomegalymild hepatomegaly

Non-specific GI symptomsNon-specific GI symptoms

JaundiceJaundice

Mild haemolysisMild haemolysis

Circulatory changesCirculatory changes

Endocrine changesEndocrine changes

Clinical features continuedClinical features continued

Haemorrhagic tendencyHaemorrhagic tendency

Portal hypertensionPortal hypertension

Hepatic encephalopathyHepatic encephalopathy

Non-specific featuresNon-specific features

ComplicationsComplications

Portal hypertensionPortal hypertension Ascites Ascites Hepatic encephalopathyHepatic encephalopathy Spontaneous bacterial peritonitisSpontaneous bacterial peritonitis Renal failureRenal failure InfectionInfection Hepatocellular carcinomaHepatocellular carcinoma

AscitesAscites

Accumulation of free fluid in the peritoneal cavityAccumulation of free fluid in the peritoneal cavity Underfilling theoryUnderfilling theory Overflow theoryOverflow theory Activation of Na and water Activation of Na and water

RAARAAincreased sympathetic nervous activityincreased sympathetic nervous activityalteration of ADH secretionalteration of ADH secretionaltered activity of th kallikrein - kinin systemaltered activity of th kallikrein - kinin system

Hepatic encephalopathyHepatic encephalopathy

Neuropsychiatric syndrome caused by liver diseaseNeuropsychiatric syndrome caused by liver disease Precipitating factorsPrecipitating factors Changes in interllet,personality,emotions & conciousnessChanges in interllet,personality,emotions & conciousness Apathy Apathy Impaired concentration,confusion,convulsion& Impaired concentration,confusion,convulsion&

drowsinessdrowsiness Flapping tremorFlapping tremor

Hepatic encephalopathyHepatic encephalopathy

Constructional apraxiaConstructional apraxia Hyperreflexia & bilat ext planter responseHyperreflexia & bilat ext planter response Fetor hepaticusFetor hepaticus Cerebellar signs,parkinsonian syndromes,spastic Cerebellar signs,parkinsonian syndromes,spastic

paraparesisparaparesis dementiadementia

GradingGrading

Clinical gradingClinical grading Clinical signsClinical signs Grade1Grade1 poor concentration,slurred speech,slow poor concentration,slurred speech,slow

mentation,disordered sleep mentation,disordered sleep patternpattern

Grade 2Grade 2 drowsy but easily rousable,occasional drowsy but easily rousable,occasional aggressive behaviour,lethergicaggressive behaviour,lethergic

Grade 3Grade 3 marked confusion,drowsy,sleepy,but marked confusion,drowsy,sleepy,but

responds to pain and voice,gross responds to pain and voice,gross disorientationdisorientation

Grade 4Grade 4 unresponse to voice,may or may not unresponse to voice,may or may not respond to painful stimulirespond to painful stimuli

Spontaneous peritonitisSpontaneous peritonitis

Abdominal painAbdominal pain Rebound tendernessRebound tenderness Absent bowel soundsAbsent bowel sounds FeverFever Ascites neutrophil count >250mmAscites neutrophil count >250mm33

E .coliE .coli

Renal failureRenal failure

Kidneys normalKidneys normal Result from altered systemic blood flowResult from altered systemic blood flow Functional renal failure /hepatorenal syndromeFunctional renal failure /hepatorenal syndrome Absence if proteinuria/abnormal urinaryAbsence if proteinuria/abnormal urinary

sedimentsedimentUrine Na <10 mmol/dUrine Na <10 mmol/dUrine/plasma osmolarlity >1.5Urine/plasma osmolarlity >1.5

InvestigationsInvestigations

Blood for CPBlood for CP LFT,AST,ALT,GGTLFT,AST,ALT,GGT T&DPT&DP OSPTOSPT UreaUrea GlucoseGlucose Ultrasound abdomenUltrasound abdomen CXRCXR EEGEEG

ManagementManagement

General managementGeneral management

Specific treatmentsSpecific treatments

Treatment of complications of cirrhosisTreatment of complications of cirrhosis

Orthotopic liver transplantationOrthotopic liver transplantation

General managementGeneral management

Good nutritionGood nutrition

Avoid protein excessAvoid protein excess

Low salt dietLow salt diet

Alcohol absteinenceAlcohol absteinence

Avoid NSAID and sedatives & opiatesAvoid NSAID and sedatives & opiates

Cholestyramine for pruritusCholestyramine for pruritus

Specific treatmentsSpecific treatments

Alpha interferon with ribavirin improve liver biochemistry Alpha interferon with ribavirin improve liver biochemistry

& may retard development of HCC in HCV induced & may retard development of HCC in HCV induced

cirrhosiscirrhosis

Little benefit of UDCA in PBCLittle benefit of UDCA in PBC

Penicillinmine for Wilson’s diseasePenicillinmine for Wilson’s disease

Venesection for haemochromatosisVenesection for haemochromatosis

Management of complicationsManagement of complications

AscitesAscites

low salt dietlow salt diet 40mmol/d -20mmmol/d40mmol/d -20mmmol/d

avoid NSAID avoid NSAID

fluid restrictionfluid restriction 0.5-1 l/24 hr 0.5-1 l/24 hr

-spironolactone 100mg/24 hr orally-spironolactone 100mg/24 hr orally

increase dose every 48 hr to 400mg/24hrincrease dose every 48 hr to 400mg/24hr

daily weight chart weight loss <1/2 kg/daydaily weight chart weight loss <1/2 kg/day

-frusemide 160mg/d-frusemide 160mg/d

-therapeutic paracentesis+ commitant albumin infusion-therapeutic paracentesis+ commitant albumin infusion

(6-8l fluid removed) (6-8l fluid removed)

Management of complications continuedManagement of complications continued

Spontaneous bacterial peritonitisSpontaneous bacterial peritonitis

-I.V cefuroxine1.5 g 8hrly +-I.V cefuroxine1.5 g 8hrly +

metronidazole 500mg 8hrlymetronidazole 500mg 8hrly

-Prophylaxis ; ciprofloxacin 250mg orally-Prophylaxis ; ciprofloxacin 250mg orally

cotrimoxazole 960mg weekdays onlycotrimoxazole 960mg weekdays only

Management of complications continuedManagement of complications continued

Hepatic encephalopathyHepatic encephalopathy

Reduce protein intakeReduce protein intake

High calorie dietHigh calorie diet

Lactulose 15-30ml tdsLactulose 15-30ml tds

Neomycin 1-4 g 4-6hrlyNeomycin 1-4 g 4-6hrly

Hepatorenal syndromeHepatorenal syndrome

18% of cirrhosis patients18% of cirrhosis patients

IV albumin with/without haemodialysisIV albumin with/without haemodialysis

Indications for liver transplantationIndications for liver transplantation

cholestatic forms of cirrhosischolestatic forms of cirrhosis

PBC PBC

Alcoholic cirrhosisAlcoholic cirrhosis

cirrhosis due to hepatitis Ccirrhosis due to hepatitis C

Alpha1Antitrypsin deficiency Alpha1Antitrypsin deficiency

HaemochromatosisHaemochromatosis

Signs of liver insuffiency pointing to the need for Signs of liver insuffiency pointing to the need for liver transplantliver transplant

Sustained or increased jaundiceSustained or increased jaundice TB >100 Umol/lTB >100 Umol/l Ascites Ascites Hepatic encephalopathy not responding to medical Hepatic encephalopathy not responding to medical

therapytherapy hypoalbuminaemia <30g/lhypoalbuminaemia <30g/l Fatigue and lethargy affecting the quality of lifeFatigue and lethargy affecting the quality of life Intractable itichingIntractable itiching Recurrent variceal bleedingRecurrent variceal bleeding

Contraindications Contraindications

SepsisSepsis

AIDSAIDS

Extrahepatic malignancyExtrahepatic malignancy

Active alcohol and other substance abuseActive alcohol and other substance abuse

Marked cardiorespiratory dysfunctionMarked cardiorespiratory dysfunction

PrognosisPrognosis

Overall prognosis is poorOverall prognosis is poor

25% survive 5 years from diagnosis25% survive 5 years from diagnosis

If liver function is good,If liver function is good,

50% survive for 5 years50% survive for 5 years

25% upto 10 years25% upto 10 years

Poor prognostic factorsPoor prognostic factors

Deteriorating liver functionDeteriorating liver function

Falling albuminFalling albumin

Serum albumin <30g/lSerum albumin <30g/l

Marked hyponatremia<120mg%Marked hyponatremia<120mg%

Prolonged PTProlonged PT

Portal hypertensionPortal hypertension

Prolonged elevation of portal venous pressure( normal 2-Prolonged elevation of portal venous pressure( normal 2-5mmHg)5mmHg)

>12mmHg>12mmHg

CausesCauses

Extrahepatic post sinusoidalExtrahepatic post sinusoidal

Budd-Chiai syndromeBudd-Chiai syndrome

Intrahepatic post sinusoidalIntrahepatic post sinusoidal

Venoocclusive diseaseVenoocclusive disease

SinusoidalSinusoidal

CirrhosisCirrhosis

Cystic liver diseaseCystic liver disease

Partial nodular transformation of liverPartial nodular transformation of liver

Metastatic malignant diseaseMetastatic malignant disease

Causes continuedCauses continued

Intrahepatic pre-sinusoidalIntrahepatic pre-sinusoidal

SchistosomiasisSchistosomiasis

sarcoidosissarcoidosis

congenital hepatic fibrosiscongenital hepatic fibrosis

Vinyl chlorideVinyl chloride

DrugsDrugs Extrahepatic pre-sinusoidalExtrahepatic pre-sinusoidal

Portal vein thrombosisPortal vein thrombosis

Abdominal traumaAbdominal trauma

Malignant diseaseof pancreas and liverMalignant diseaseof pancreas and liver

pancreatitispancreatitis

congenitalcongenital

Clinical featuresClinical features

Portal venous congestionPortal venous congestion Collateral formationCollateral formation SplenomegalySplenomegaly HypersplenismHypersplenism

thrombocytopenia 100x 10thrombocytopenia 100x 1099/l/l

leucopenia leucopenia

AnaemiaAnaemia

Collateral vessels on ant abd wall around umblicusCollateral vessels on ant abd wall around umblicus

Oesophageal varicesOesophageal varices

Rectal varicesRectal varices

Fetal hepaticusFetal hepaticus

InvestigationsInvestigations

Barium swallow x rayBarium swallow x ray

USS abdomen USS abdomen

splenomegaly splenomegaly

liver disease liver disease

portal vein thrombosisportal vein thrombosis

Endoscopy collateral vesselsEndoscopy collateral vessels

Portal venographyPortal venography

ComplicationsComplications

Variceal bleeding (oesophageal,gastric,other (rare)Variceal bleeding (oesophageal,gastric,other (rare)

Congestive gastropathyCongestive gastropathy

HypersplenismHypersplenism

AscitesAscites

Renal failureRenal failure

Hepatic encephalopathyHepatic encephalopathy

Variceal bleedingVariceal bleeding

Oesophageal bleeding within 3-5 cm fr oesophagogastric Oesophageal bleeding within 3-5 cm fr oesophagogastric

junctionjunction

gastric varicesgastric varices

Size of varicesSize of varices

High portal prHigh portal pr

Liver failureLiver failure

DrugsDrugs

Management of acute variceal bleedingManagement of acute variceal bleeding

To restore circulationTo restore circulation Reduction of portal pressureReduction of portal pressure Local measuresLocal measures

Reduction of portal pressureReduction of portal pressure

Less important >sclerotherapy or bandingLess important >sclerotherapy or banding Pharmacological measuresPharmacological measures

vasopressin I/Vvasopressin I/V 0.4 U/min x 24 hrs0.4 U/min x 24 hrs

0.2 U/min x 24 hrs0.2 U/min x 24 hrs

TerlipressionTerlipression 2mg iv 6hrly 2mg iv 6hrly

1mg iv 6hrly x 24 hrs1mg iv 6hrly x 24 hrs

OtreotideOtreotide 50ug iv stat & hrly infusion50ug iv stat & hrly infusion

TIPSS & shunt surgery TIPSS & shunt surgery

-high mortality-high mortality

Local measuresLocal measures

Sclerotherapy or bandingSclerotherapy or banding

daignostic endoscopydaignostic endoscopy

stop bleeding in 80% of patientsstop bleeding in 80% of patients

can be repeated if bleeding recurscan be repeated if bleeding recurs Balloon tamponadeBalloon tamponade

Sengstaken-BlakemoreSengstaken-Blakemore

Minnesota tubeMinnesota tube

Oesophageal transectionOesophageal transection

stapling gun stapling gun

spleenectomyspleenectomy

Prevention of recurrent bleedingPrevention of recurrent bleeding

SclerotherapySclerotherapy

sclerosing agentssclerosing agents

1-2 weekly1-2 weekly

S/E feverS/E fever

Transient chest pain/abdoTransient chest pain/abdo

Transient dysphagia Transient dysphagia

oesophageal perforationoesophageal perforation

Low mortalityLow mortality

Prevention of recurrent bleedingPrevention of recurrent bleeding

BandingBanding

occluded with tight rubber bandoccluded with tight rubber band

More effective More effective

Fewer S/E Fewer S/E

Prevention of recurrent bleedingPrevention of recurrent bleeding

TIPSSTIPSS

Stent placed b/t portal vein &hepatic vein in the liverStent placed b/t portal vein &hepatic vein in the liver

under radiological contol under radiological contol

t/h Internal jugular veint/h Internal jugular vein

Prior angiographyPrior angiography

FFPFFP

Antibiotic coverAntibiotic cover

S/ES/E Shunt narrowing/occlusionShunt narrowing/occlusion

Hepatic encephalopathyHepatic encephalopathy

Portasystemic shunt surgeryPortasystemic shunt surgery

High mortalityHigh mortality

Non-selective shuntNon-selective shunt

Postop liver failurePostop liver failure

Hepatic encephalopathyHepatic encephalopathy

More selective shuntMore selective shunt

less post hepatic encephalopathyless post hepatic encephalopathy

Preserved for patients in whom other treatments have Preserved for patients in whom other treatments have

not been successfulnot been successful

PropranololPropranolol

80-160mg/day,80-160mg/day,

Not widely used:Poor complianceNot widely used:Poor compliance

use for primary preventionuse for primary prevention

Child-pugh grading and risk of variceal bleedingChild-pugh grading and risk of variceal bleeding

pointspoints 1 1 22 33

Bilirubin(Bilirubin(µmol/l)µmol/l) <34<34 34-5134-51 >51>51

Albumin(g/l)Albumin(g/l) >3.5>3.5 2.8-3.52.8-3.5 <2.8<2.8

PT(seconds>normal)PT(seconds>normal) 1-31-3 4-64-6 >6>6

AscitesAscites NoneNone slightslight moderatemoderate

EncephalopathyEncephalopathy NoneNone 1-21-2 3-43-4

Points ≥ 8 Points ≥ 8 risk risk

Congestive gastropathyCongestive gastropathy

EndoscopyEndoscopy

multiple areas of punctate erythemamultiple areas of punctate erythema

distally GITdistally GIT

erosion –bleedingerosion –bleeding

IDAIDA

TreatmentTreatment

propranololpropranolol

TIPSSTIPSS

MCQ MCQ

1.Clinical features of cirrhosis of the liver includes1.Clinical features of cirrhosis of the liver includes

(a)(a) HepatomegalyHepatomegaly

(b)(b) JaundiceJaundice

(c)(c) Palmer erythemaPalmer erythema

(d)(d) AmenorrhoeaAmenorrhoea

(e)(e) Low grade feverLow grade fever

MCQMCQ

2.Complications of cirrhosis are2.Complications of cirrhosis are

(a)(a) Portal hypertensionPortal hypertension

(b)(b) Ascites Ascites

(c)(c) Hepatic encephalopathyHepatic encephalopathy

(d)(d) Renal failureRenal failure

(e)(e) BacteraemiaBacteraemia

MCQMCQ

3.Poor prognostic factors includes3.Poor prognostic factors includes

(a)(a) HypoalbuminaemiaHypoalbuminaemia

(b)(b) HypernatraemiaHypernatraemia

(c)(c) Increasing total bilirubinIncreasing total bilirubin

(d)(d) Prolonged prothrombin timeProlonged prothrombin time

(e)(e) Cirrhosis due to haemochromatosisCirrhosis due to haemochromatosis

MSQMSQ

What are the common causes of cirrhosisWhat are the common causes of cirrhosis

of liver? How would you manage a case of of liver? How would you manage a case of

COL with hepatic encephalopathy?COL with hepatic encephalopathy?

MCQMCQ

1.Clinical features of portal hypertension are1.Clinical features of portal hypertension are

(a)(a) SplenomegalySplenomegaly

(b)(b) HypersplenismHypersplenism

(c)(c) JaundiceJaundice

(d)(d) Fetor hepaticusFetor hepaticus

(e)(e) Spider naeviSpider naevi

MCQMCQ

2. Following drugs can be used in acute 2. Following drugs can be used in acute variceal bleedingvariceal bleeding

(a)(a) VasopressinVasopressin

(b)(b) NSAIDNSAID

(c)(c) TerlipressinTerlipressin

(d)(d) Octreotide Octreotide

(e)(e) Beta blocker Beta blocker

MSQMSQ

Describe management of acute GI Describe management of acute GI bleeding due to rupture oesophageal bleeding due to rupture oesophageal varices.What preventive measures would varices.What preventive measures would you give to prevent recurrent bleeding?you give to prevent recurrent bleeding?