Chronic Liver Disease
Prensentation
Sir, this patient has decompensated chronic liver disease with portal hypertension,
splenomegaly and ascites.
My findings include:
Presence of an enlarged spleen that is palpable 3cm from the left costal margin. It is
non-tender, firm in consistency, smooth surface, regular edge, notch border with no
splenic rub. I am unable to get above this mass. The liver is not enlarged with a span
of 12 cm in the right mid-clavicular line. The kidneys are not ballotable. There is
presence of ascites with shifting dullness and this is not associated with tenderness.
He is deeply jaundice and bruising noted on the ULs and LLs with presence of
stigmata of CLD including leukonychia, clubbing, palmar erythema, spider naevi and
gynaecomastia with loss of axillary hair. There is also presence of bilateral edema.
Complications:
He is cooperative with the examination with no flapping tremor to suggest hepatic
encephalopathy.
There are no enlarged Cx LNs and patient is not cachexic looking. There is also no
conjunctival pallor noted.
Aetiology:
I did not find any parotidomegaly, dupytren’s contracture, tattoos, surgical scars or
thrombosed veins.
Treatment:
I did not notice any abdominal tap marks but patient has sinus bradycardia, indicating
use of beta-blockers.
I would like to complete my examination by looking at the patient’s temperature chart
for fever and a rectal examination for hard impacted stools or malena.
In summary, this patient has decompensated chronic liver disease with portal
hypertension, splenomegaly and ascites. There is presence of bruising, leukonychia,
jaundice with no evidence of hepatic encephalopathy.
A. The most likely etiology for this gentleman is
1. Chronic ethanol ingestion due to presence of parotidomegaly but no dupytren’s
contracture; presence of hepatomegaly
2. Chronic hepatitis B infection as patient has tattoos.
3. Chronic hepatitis C infection as I note an abdominal surgical scar with possibility
of transfusion in the past
B. In the local context, the most likely underlying etiology is chronic ethanol
ingestion, chronic hepatitis B and C.
C. The most likely aetiology is chronic ethanol ingestion as I notice that this patient
has presence of parotidomegaly. In view that there is also a hard irregular liver that is
palpable, it raises the possibility of an underlying mitotic lesion of the liver.
D. The most likely aetiology is
1. Primary biliary cirrhosis as she is a middle-aged lady with evidence of CLD with
pruritus, xanthelasma and generalised pigmentation.
2. Hemochromatosis as he is a middle-aged gentleman with slate-grey appearance
with presence of diabetic dermopathy. I would like to complete the examination by
examining the CVS for CMP, urine dipstick for glycosuria and for small testes
secondary to pituitary dysfunction.
3. Wilson’s disease as the patient has a short stature associated with Kayser Fleisher
rings of the eyes and tremor and chorea of the affecting the left upper limb.
4. Haemolytic anaemia (Thalassemia major/intermedius, Hereditary spherocytosis) as
the patient has a short stature associated with hyperpigmentation and thalassemic
facies with frontal bossing, flat nasal bridge and maxillary hyperplasia. I would like to
complete the examination by examining the CVS for CMP, urine dipstick for
glycosuria and for small testes secondary to pituitary dysfunction.
Questions
What is cirrhosis of the liver?
 Defined pathologically
 Diffuse liver abnormality
 Fibrosis and abnormal regenerating nodules
What are the causes of liver cirrhosis?
 Chronic ethanol ingestion
 Viral hepatitis – B and C
 In UK, the risk for hep C is blood transfusion before Sept 1991 or blood
products before 1986
 Cardiac failure
 Others
 Autoimmune chronic active hepatitis (female)
 Primary biliary cirrhosis (female)
 Primary sclerosing cholangitis
 Haemochromatosis (male)
 Hemolytic disease
 Wilson’s disease
 Alpha 1 AT deficiency
 Galactosemia
 Type 4 glycogen storage disease
 Budd-Chiari (in malignancy- PRV or intraabdominal, AI, OCPs, IBD and
PNH)
 Drugs – MTX (Look for RA or Psoriasis; Bx before starting MTX and bx
every 1.5g accumulated dose), amiodarone, isoniazid, methyldopa (MAMI)
 Cryptogenic
What are the complications of cirrhosis? (5)
 Portal hypertension
 Ascites – Tense ascites, SBP
 Splenomegaly – thrombocytopenia



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 Varices
Hepatorenal syndrome
 Dx
 Cr Clr <40
 Absence of other causes for renal impairment
 Absence of Cr improvement, proteinuria (0.5g/d), hematuria (<50/hpf) and
urinary Na <10
 Type 1 = rapidly deterioration in renal fn ie doubling of serum Cr in < 2wks to
>221 umol/l
 Type 2 = stable or slowly progressive that does not mean criteria for type 1
Hepatic encephalopathy
 Stages
 1 – depression, euphoria, sleep disturbance, slurred speech; may have
asterixis, normal EEG
 2 – lethargy, moderate confusion; asterixis present; abnormal EEG
 3 – marked confusion, arousable; asterixis present; abnormal EEG
 4 – coma; abnormal EEG
Coagulopathy – low platelets and reduced clotting factors
HCC
How do you stage cirrhosis of the liver?
 Child-Pugh staging
o Consists of 5 parameters with score ranging from 5 to 15
o Prognosticate
o 5 parameters ( 2 clinical and 3 Ix)
o Bilirubin (<34, 34-50, >50 umol/l)
o INR (<1.7, 1.7-2.3, >2.3)
o Albumin (>35, 28-35, <28)
o Ascites (mild, moderate, severe)
o Encephalopathy (absent, I and II, III and IV)
o A – 5-6 pts (1 year 100%, 2 year 85%)
o B – 7-9 (1 year 80%, 2 year 60%)
o C – 10-15 (1 year 45%, 2 year 35%)
How would you investigate? (Note the STEM STATEMENT) (5)
 Confirming the dx
o Abdominal USS or CT
 Establishing the aetiology
o Hep markers, CAGE questionnaire, liver Bx in selected cases
 Prognosticate
o LFT – Albumin, bilirubin
o INR
 Complications
o Endoscopy of the upper GIT
o Mitotic change – USS and AFP
o Evaluation of renal function – urea, electrolytes and Cr
o Evaluation of ascitic fluid
 Cell count
 Ascites albumin (SAAG)
 Gram stain and C/S

 Others – AFB smear and c/s, cytology
Evaluation for liver transplant
o 5 year survival rate for cirrhosis with ascites is 30-40% vs 70-80% for post
liver transplant
o MELD score (Model for End Stage liver disease which has bilirubin,
creatinine and INR)
o Consider for those with refractory ascites, SBP or HRS
When should an abdominal paracentesis be done for a patient with cirrhosis and
ascites?
 Newly diagnosed to r/o SBP
 Symptomatic – fever, abdominal pain, encephalopathy, GI bleed
How would you manage? (4)
 Education and counselling
o Stop drinking alcohol, regular follow up
 Manage the underlying disease
o Hepatitis B
 General measures (stop alcohol, hep A vaccination)
 Lifelong surveillance for HCC with USS and AFP
 Antiviral for
 Immune clearance phase( HBeAg +, ALT raised)
 Reactivation phase ( HBe Ag -, ALT raised, HBV DNA
raised)
 IFN alpha (SE : influenza-like; neutropenia and
thrombocytopenia; neuropsychiatric and unmasking AI disease)
 Lamivudine (well tolerated but YMDD mutant)
o Hepatitis C
 At risk are IVDAs and transfusion pre 1989 (Singapore) or
pretransfusion Sept 1991 or blood pdts before 1986 (UK)
 General measures
 Surveillance (HCC and screen for HIV)
 Indications
 HCV RNA levels (>50 IU/ml)
 Raised ALT
 Bx showing fibrosis and inflammation
 Treatment
 Peg interferon
 Ribavirin
o Alcoholic liver disease
 >21u/wk in males and >14u/wk in females
 100% of normal liver develops fatty liver
 35% develop alcoholic hepatitis
 20% develop cirrhosis
 40% of alcoholic hepatitis develop cirrhosis
 Maddrey’s discrimination function
 PT x Bil x 4.6
 >32 = severe



Treat with corticosteroids or total enteral nutrition (2030 kcal/kg/day)
o Others (see notes below)
Manage the complications
o Hepatic encepholpathy
 Treat precipitants (see below)
 Prevent
 Low protein diet
 Lactulose
o Hepatorenal syndrome
 Treatment with
 Noradrenaline infusion, telipressin or midodrine with
octreotide plus
 Albumin infusion (1g/kg on D1 then 20-40g/day)
 For 5-15 days
 Prevention (in patient with cirrhosis and ascites)
 IV albumin
 NB that hemodialysis does not help in this condition
o Ascites (see ascites)
o Upper GI bleed
 Secure VS
 Urgent endoscopy
 Operative
 Prevention
 Propanolol to reduce HR by 25% or to 55-60 bpm
 Variceal banding
o HCC
Definitive treatment
o Liver transplant
o MARS (Molecular adsorbent Recirculating system)dialysis as an
interim measure before liver transplant
What are the factors precipitating decompensation?
 Infection – SBP, pneumonia, UTI
 GI bleed
 Constipation
 Diuretics and electrolyte imbalance
 Diarrhea and vomiting
 Sedatives
 Surgery
What are the nail changes of hypoalbuminaemia?
 Leukonychia, ie nail bed opacify indicating an albumin level <30g/dL; affecting
the thumb and index nails bilaterally initailly
 Muehrcke’s lines – transverse white lines
What are the causes of palmar erythema?
 CLD
 RA, thyrotoxicosis and polycythaemia
 Pregnancy, normal finding
What are the causes of anaemia in cirrhotic patients?
 Anaemia of chronic disease
 Fe deficiency from GI bleed
 Hemolysis from hypersplenism
 Folate and B12 from poor nutrition
How many spider naevi should be present to be considered as significant?
 More than 5
When examining a patient with signs of chronic liver disease, think of:
Primary biliary cirrhosis
 Clinical
 Female middle age
 CLD with pruritus, xanthelesma, generalised pigmentation,
hepatosplenomegaly
 Stages
 Asymmptomtic with normal LFTs (positive Abs)
 Asymptomatic with abnormal LFTs
 Symptomatic – lethary and pruritus
 Decompensated
 Commonly associated with sicca syndrome, arthralgia, Raynauds,
Sclerodactyly and Thyroid disease
 Ix
 Raised ALP, Anti-Mitochondrial Ab – M2 Ab, IgM
 Lipids
 Other tests for CLD
 Histology – Granulomatous cholangitis
 Mx
 Symptomatic
 Urosdeoxycholic acid
 Cholestyramine
 Fat soluble vitamins
 Immunosuppression – Cyclosporin, steroids, AZA, MTX, tacrolimus,
colchicines
 Liver transplant
Hemochromatosis
 Clinical
 Male
 Slate-grey appearance, hepatomegaly
 Affects
 Liver – cirrhosis and cancer
 Pancrease – DM
 Heart failure (CMP)
 Pituitary dysfunction
 Pseudogout
 Therefore requests
 Urine dipstick, CVS examination and testicular examination
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

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Autosomal recessive, HLA-A3, Ch 6 – HFE gene, increased Fe absorption with
tissue deposition,
Ix
 Raised ferrritin, transferrin saturation and liver Bx
Mx
 Non-pharmological
 Avoid alcohol
 Avoid shellfish as they are susceptible to Vibrio vulnificus
 Venesection
Dy/Dx of generalised pigmentation
 Liver – hemochromatosis in males and PBC in females
 Addison’s
 Uremia
 Chronic debilitating conditions eg malignancy
 Chronic haemolytic anaemia
Wilson’s disease
 Clinical
 Short stature
 Eyes
 KF rings - greenish yellow to golden brown pigmentation of the limbus of
the cornea due to deposition of Cu in Descemet’s membrane at 12 and 6
o’clock position. Also occurs in PBC and cryptogenic cirrhosis
 Sunflower cataract
 Extrapyrimidal
 Tremor and chorea
 Presents as difficulty writing and speaking in school
 Pseudogout
 Penicillamine complications
 Myasthenic – ptosis
 Lupus – malar rash, small hand arthritis
 Urinalysis for glycosuria from proximal RTA
 Autosomal recessive, Ch 13, increased Cu absorption and tissue deposition
 Ix
 Low serum ceruloplasmin, increased 24H urinary Cu
 Liver Bx – increased Cu deposition
 Mx
 Penicillamine
Ulcerative Colitis
 Clinical
o Skin – erythema nodosum, pyoderma gangrenosum
o Joint arthropathy – LL arthritis, AS, sacroilitis
o Aphthous ulcers
o Ocular – iritis, uveitis and episcleritis
o CLD – Cirrhosis, chronic active hepatitis, fatty liver PSC,
Cholangiocarcinoma, metastatic colorectal cancer, amyloid