CHRONIC LIVER DISEASE IN CHILDREN

 
Most chronic liver disease of Childhood
Result in cirrhosis which is a chronic diffuse
liver disease characterized by widespread
hepatic fibrosis with regenerative nodule
formation.
 
Diagnostic Consideration:
Diagnosis- Essentially a stepwise process,
involving a range of-
-         Clinical
-         Laboratory
-         Radiological imaging and
-         Pathological investigations:
1.     Confirming the presence and type of
liver disease.
2.     determining aetiology.
3.     Assessing the extent of complications.
Active- Presence of biochemical or
histological evidence of hepatocellular
necrosis, apoptosis and inflammation.
Inactive- No such evidence.
Compensated- No clinical or laboratory
features of liver failure.
Decompensated- Such features are evident.
 
Cl .Presentation:
Compensated liver disease:
-         No symptoms
-         May not be jaundiced
-         Ist indication- May be an incidental
finding of-
- Hepatosplenomegaly
- Splenomegaly alone
- Increased hepatic transaminases
or
Increased alkaline phosphates .
- Liver small and impalpable (commonly)
But it can be enlarged, hard or nodular with, in
some patients, a small right lobe or
splenomegaly.
-    Cutaneous features
Spider angiomata
Prominent periumbilical veins (clue to the
presence of liver diseases)
Palmar erythema
Other cutaneous features:
-    Easy bruising
-    Fine telangiectasia on the face & upper back.
-   White spots often on buttocks and arms
-   Clubbing of the findings
 
In adults Mostly cryptogenic
In children Metabolic liver disease such as
Wilson’s disease & α1-antitrypsin deficiency.
Children with cholestatic liver disease
-         Predominant sign or symptoms
of Persisting jaundice as in biliary
cirrhosis
or
Pruitus
Liver- usually enlarged
Xauthelasma
Malnutrition
Dep. Of fat-soluble vit
(particulary vit D & K)
Clubbing – more likely
Decompensated liver disease:
       Clinical and laboratory findings of
i)       Liver SYNTHETIC failure
ii)     Occurrence of complications &
iii)    Poor response to supportive medications
Major features include
-         Malnutrition,
-         Ascites,
-         Peripheral oedema,
-         Coagulopathy &
-         Gastrointestinal bleeding
-     Signs hepatic encephalopathy-
Subtle-    Jaundice- a late feature (except biliary
cirrhosis)&
indicates very advanced disease.
Cases of chronic liver disease in children:
1. Biliary:
-     extrahepatic billiary atresia- Sclerosing
cholangitis
-    Choledochalcyst, tumours,stones – Graft-
vs-host-disease
-  Alagille syndrome, biliary hypoplasis
- Histiocytosis X
-  Progressive intrahepatic Cholestasis,drugs.
2.     Hepatic:
-    Neonatal hepatitis - Autoimmune hepatitis
-    Hepatitis B  delta - Drugs toxins
-    Hepatitis C
3.  Genetic/metabolic :
= Carbohydrate defects
Galactosaemia,
fructosaemia, glycogen storage disease III and IV
= Amino acid defects
Tyrosinaemia, Urea cycle disorders
= Metal storage defects
 Neonatal haemachromatosis , Wilson’s disease
= Lopid storage disease
Gaucher’s disease , Neimann-pick type C
Early acid – oxidation defects
Peroxisomal disorders
  Zellweger’s syndrome
Mitochondrial disorders
 Respiratory chain defects
Cystic fibrosis
Fibropolycystic disorders ( Do not cause
cirrhosis)
 
 
2.     Vascular:
-    Hepatic vein thrombosis
Budd-chiari syndrome
- Veno-occlusive disease
-   Cardiac
 Complications of CLD and cirrhosis
A) Primarily due to impaired:
1. Hepatic function-
a)      Nutritional and metabolic disturbance
(Malnutrition & Growth failure)
b)Impaired protein synthesis and coagulopathy
2. Haemodynamic changes-
a)      Portal hypertension & variceal bleeding
b)     Hepatorenal syndrome
c)      Hepatopulmonary syndrome
B) Both of those above (1&2) major patho-
physiological events-
a)    Encephalopathy
b)   Ascites
C) Due to cholestasis-
a)      Prurities
b)     Malabsorption
D) Impaired immunity: Bacterial infection,
Spontaneous bacterial peritonitis
Hepatocellular carcinoma can complicate any
causes of cirrhosis in childhood, particularly in chronic
hepatitis B & Tyrosinaemia type 1.
 Investigations:
1. Full ranges of laboratory: May be diagnostic
of the underlying cause and
2. Full range of imaging: may allow
appropriate handling of the
specimen.
3. Liver Biopsy:
-    ALL forms of SUSPECTED liver disease
-    Confirmation will ultimately rest with the
interpretation of biopsy findings, which may
confirm
- The presence
- The type
- The degree of activity of cirrhosis
and
may contribute to a diagnosis of the cause of
liver disease.
Liver Pathology
Classification- Based on morphology,histology
aetiology and degree of activity
Morphological classification
-         Micro nodular (< 3mm)
-         Macro nodular
-         Mixed
** Fibrosis is not synonymous with cirrhosis **
= Fibrosis with nodules typically in
-         Congenital hepatic fibrosis
-         Granulomatous liver disease (interlobular)
= Nodular formation without fibrosis in
Portal nodular transformation.
Histological classification:
Generally more helpful
i)    Indefineing aetiology as
a)   Post necrotic
b)   Biliary (periportal) or
c)   Hepatic venous outflow (cardiac) cirrhosis
and ii) In management.
Specific histological patterns
- Wilson’s disease copper pigment deposition
- α1 - antitrypsin deficiency Intracellular periodic
acid- sceiff positive, diastase resistant inclusions
-  Certain storage disorders.
Post- necrotic Result of liver Result of liver cell
damage
Most commonly seen in chromic
hepatitis
Features Piecemeal necrosis,bridging fibrosis,
collapse of the
hepatic lobules, and regeneration, with
the development of
macro nodular cirrhosis.
Biliary cirrhosis Result from cholestatic disorder
Fibrosis developing from within the portal tracts
extending out into the parenchyma linking adjacent
portal tracts with little change in the hepatic
parenchyma, and preservation of lobular architecture.
Obstruction to hepatic venous out flow:
centrilobular haemorrhagic necrosis, with
fibrosis extending from central veins to portal
tracts, caused by cardiac lesions---- and vaso-
occlusive disorders.
 Adverse Prognostic features of CLD in
children
1)        Bilirubin > 300mmol/L
2)        Prolonged prothrombin time
unresponsive to intravenous vitamin K
3)        Partial thromboplastin time > 20s
4)        Malnutrition (Wt. SD score < 1.5)
5)        Low plasma cholesterol
6)        Ascites.
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