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1. BY: DR. K.S.K JUSU
Department of internal medicine
Makeni school of clinical sciences
Chronic Liver Disease

2. Chronic Liver Disease
 Definition:
 A spectrum of disorders characterized by ongoing
chronic liver damage and a potential to progress
to cirrhosis or end stage liver disease
 A duration of 6 months is used as cut off in cases
of hepatitis B and C but not used in other causes
 Irreversible liver damage may occur before
symptoms of liver damage appear

3. Chronic active hepatitis Chronic persistent hepatitis
Severe form of chronic disease Milder form of chronic disease
Slow but relentlessly
progressive disease
Does not lead to progressive
liver damage despite
continuing mild inflammation
Comparison between Chronic active hepatitis
and chronic persistent hepatitis

4. Cirrhosis
 Caused by chronic liver diseases
 Characterized by diffuse hepatic cell injury
(hepatocellular necrosis) causing inflammation,
widespread fibrosis and nodular regeneration
 Result of abnormal reconstruction and loss of
normal lobular architecture

5. Cirrhosis

6. Normal Histology of Liver
Portal triad:
1. Branch of
hepatic artery
2. Branch of portal
vein
3. Bile ductule
4. Lymphatic
vessels

7. Liver Histology in Cirrhosis

8. Liver Histology in Cirrhosis

9. Cirrhosis
 Types:
1. Compensated
2. Decompensated

10.  Compensated cirrhosis:
 Disease is silent
 Hepatosplenomegaly
 Abnormal liver function tests

11.  Decompensated cirrhosis:
 Jaundice
 G I bleed
 Ascitis and /or
 Hepatic encephalopathy

12. Causes of cirrhosis in children
1. Hepatitis B and C
2. Autoimmune hepatitis
3. Inherited diseases
a) Glycogen storage
disease
b) Tyrosinemia
c) Wilson disease
d) α1-antitrypsin
deficiency
e) Cystic fibrosis
4. Bile duct diseases
a) Biliary atresia
b) Sclerosing cholangitis
c) Congenital hepatic fibrosis
d) Choledochal cysts
5. Drugs and toxins
a) Isoniazid
b) Methotrexate
c) Excess vitamin A
6. Fatty liver disease
url link: http://www.chp.edu/our-services/transplant/liver/education/liver-disease-states/cirrhosis

13. Clinical features
 Characteristics of liver in cirrhosis:
Liver may be firm to hard nodular or
have irregular margins
 Differential left lobe enlargement in
CLD
Small, non palpable shrunken liver in
post necrotic cirrhosis

14. Manifestations of CLD
 These include spider angiomata, palmer
erethema, clubbing, leuconychia, muscle
wasting, delayed puberty and gynecomastia
 Testicular atrophy and parotid enlargement are
not present in children

15. Portal hypertension
 Defined as an increase of blood
pressure in the portal venous bed
beyond the physiological values
 (Normal portal pressure is 5 to
10 mm Hg or 7 to 14 cm H 2 O)
 In children it is usually caused by an
increase of resistance to the blood flow
from the mesenteric venous circulation
through or to the liver
 Increase of resistance results from

16. Causes of Portal Hypertension:
 Depends on site of obstruction:
 Prehepatic (the most frequent type in
children, 60–75 %)
 Intrahepatic (secondary to hepatocellular
injury leading to cirrhosis e.g., biliary atresia)
 Post-hepatic (e.g., Budd-Chiari syndrome,
rare in children)

17. Clinical features of Portal
hypertension
Splenomegaly
Ascitis
Tortuous veins over abdominal
wall i.e. caput medusa
Esophageal varices
with/or without gastric varices
seen on endoscopy

18.  Hepatic encephalopathy:
 Neuropsychiatric abnormalities in
patients with liver dysfunction
 Features:
 asterixia
 constructional apraxia
 altered sensorium

19.  Apraxia:
 Inability to perform tasks or movements
even though they understand the task, are
willing to complete it, and have the physical
ability to perform the movements
 Constructional apraxia:
 Inability or difficulty to build, assemble, or
draw objects

20. Evaluation of CLD
to determine etiology
to access degree of liver dysfunction
and presence of complication

21. Grading of liver damage
 CHILD score (or Child-Pugh score) for
grading of liver damage
 PELD score (Pediatric end stage liver
disease)

22. Child-Pugh score
 Marker of global liver function in a patient with
cirrhosis and may help in selection of patients
appropriate for resection, particularly in the setting of
hepatocellular carcinoma
 Child-Pugh Classification:
 The Child-Pugh classification is a universal scoring
system of the degree of liver failure in patients with
cirrhosis
 Child-Pugh class (A, B, or C) has been used as a
predictive index for operative mortality rate in adult
patients undergoing portosystemic shunting
procedures

23. Prognosis of CLD
Child-Pugh score
 Used to assess prognosis in chronic liver
disease and cirrhosis
 Score considers 5 factors:
 3 synthetic functions (total bilirubin, serum albumin,
and INR)
 2 based on clinical assessment (degree of ascites
and degree of hepatic encephalopathy)

24. Factor 1 point 2 points 3 points
Total bilirubin
(μmol/L)
<34 34-50 >50
Serum albumin
(g/L)
>35 28-35 <28
PT INR <1.7 1.71-2.30 >2.30
Ascites None Mild Moderate to
Severe
Hepatic
encephalopathy
None Grade I-II (or
suppressed with
medication)
Grade III-IV (or
refractory)
Child-Pugh score

25. Nutrition failure in CLD
 Clinical recognition as:
Growth failure
Loss of muscle mass
Delayed motor development
Signs of fat-soluble or essential fatty
acid deficiencies
Presence of ascites, edema and
organomegaly

26. Class A Class B Class C
Total points 5-6 7-9 10-15
1-year
survival
100% 80% 45%
©
Child-Pugh score

27. Hepatorenal syndrome
Functional renal impairment occur
which are reversible after liver
transplant
Progressive renal insufficiency
occur in absence of other known
causes (e.g. prerenal, nephrotoxic
drugs) of renal failure in patients
with severe liver disease

28. Ascitis
 Accumulation of fluid within the peritonial cavity
 Causes:
 Cirrhosis and portal hypertension
 Budd-Chiari syndrome
 Other causes:
 Nephrotic syndrome
 Protein loosiing enteropathy
 Tubercular ascitis
 Constrictive pericarditis
 Cardiac failure
 Chylous ascitis: lymphatic obstruction, thoracic
duct injury

29. Ascitis with liver disease
Develops in liver failure
Indicates advanced disease
Represents a state of excess total
body sodium and water

30. Ascitis with liver disease
Management
 Mobilization of ascitic fluid
 Negative sodium balance
 Diuretics
 Spironolactone + frusemide)
 Sodium restricted diet
 Therapeutic abdominocentesis with albumin
infusion
 Prevention and treatment of infection
 Surgical and endovascular shunts
 Liver transplantation

31. Spontaneous bacterial peritonitis
 Cirrhotic with ascitis have increased susceptibility to
infections
 Spontaneous bacterial peritonitis is most frequent
and most severe
 Bacterial infection of ascitic fluid without any intra-
abdominal, surgically treatable source of infection
 Results from bacterial translocation

32. Causes of chronic liver disease
in children

33. Viral
1.Cytomegalovirus
2.Rubella
3.Herpes simplex
4.Hepatitis B
5.Delta hepatitis
6.Enteroviruses
Idiopathic:
1.Neonaal hepatitis
2.Familial intrahepatic chlolestasis
Bacterial
1.Syphilis
Biliary tree abnormalities:
1.Extrahepatic biliary atresia
2.Arteriohepatic dysplasia
3.Intrahepatic duct paucity
4.Choledochal cyst
Metabolic/ genetic
1.α1-antitrypsin deficiency
2.Galactosemia
3.Fructosemia
4.Tyrosinosis
5.Glycogen storage disease type 3, 4
6.Niemann-Pick disease
7.Wolman disease
Vascular:
1.Congestive cardiac failure
2.Constrictive pericarditis
3.Veno-occlusive disease
4.Budd-Chiari syndrome
Toxic:
1.Parenteral nutrition
Presenting in Infancy

34. Infectious:
1. Chronic hepatitis B +/- delta
2. Chronic hepatitis C
Metabolic/ Genetic
1. Wilson disease
2. Alpha 1 antitrypsin deficiency
3. Cystic fibrosis
Hepatobiliary anatomic
1. Choledochal cyst
2. Intrahepatic cystic biliary dilation (Caroli disease)
3. Congenital hepatic fibrosis
4. Sclerosing cholangitis
Toxic/ drug
1. Malnutrition
2. Hepatotoxic drugs
Presenting in childhood and adolescence

35. Budd-Chiari syndrome
 Occlusion of the hepatic veins and/or
suprahepatic inferior venacava
 May be primary or secondary
 Doppler ultrasound and venography
confirm the diagnosis
 Hypercoagulable states should be
looked for
 Treatment to maintain the patency of
hepatic vein/inferior vena cava

36. Wilson disease
 Toxic accumulation of copper in liver, brain, cornea and other
tissues
 Diagnosis:
 Low ceruloplasmin
 High urinary copper
 KF ring
 Elevated copper content
 Treatment:
 Ammonium tetrathiomolybdate
 D-penicillamine or trientine
 Zinc (with mercaptides formation with copper)
 Liver transplantation

37. Chronic hepatitis B infection
 HBV infection may result in AVH and ALF and chronic
hepatitis, cirrhosis and hepatocellular carcinoma
 Persons who become chronic carriers are:
 > 90 % of infected neonates
 20-25 % of infected preschool children
 5 % of adults
 Chronic HBV infection is persistence of positive
HBsAg for > 6 mo
 Most clear of HBeAg by age 15-30 yr
 Cirrhosis develops in 3-10 %
 Carcinoma (HCC) develops in 1-4 %

38. Management
 Liver function monitoring
 Test for coinfection
 Liver biopsy for grading and staging of disease
 Use of drugs:
 Interferon
 Lamivudine
 Adefovir
 Follow up for disease flares
 Avoidence of hepatotoxic agents
 Immunization of negative family members

39. Thank You