Paediatrics: Chronic liver failure

Chronic liver failure

Causes

• Chronic hepatitis (after viral hepatitis B or C).
• Biliary tree disease, e.g. biliary atresia.
• Toxin-induced, e.g. paracetamol, alcohol.
• A₁-antitrypsin deficiency.
• Autoimmune hepatitis.
• Wilson’s disease (age >3yrs).
• Cystic fibrosis.
• Alagille syndrome or non-syndromic paucity of bile ducts.
• Tyrosinaemia.
• Primary sclerosing cholangitis.
• PN-induced.
• Budd–Chiari syndrome.

Presentation

• Jaundice (not always).
• GI haemorrhage (portal hypertension and variceal bleeding).
• Pruritis.
• FTT.
• Anaemia.
• Enlarged hard liver (though liver often small in cirrhosis).
• Non-tender splenomegaly.
• Hepatic stigmata, e.g. spider naevi.
• Peripheral oedema and/or ascites.
• Nutritional disorders, e.g. rickets.
• Developmental delay or deterioration in school performance.
• Chronic encephalopathy.

Investigations

Blood tests

• LFT (i or n bilirubin, ‘rise’AST/ALT (x 2–10), albumin <35g/L).
• FBC (d Hb if GI bleeding); ‘fall’ WCC and platelets (hypersplenism).
• Coagulation (prothrombin time ‘rise if vitamin K deficiency).
• d or n blood glucose.
• U&E (d Na⁺, ‘fall’ Ca2⁺, ‘rise’PO₄^{3 –}, ‘raise alkaline phosphatase if biochemical rickets).
• Viral serology or PCR for hepatitis B and C.
• i IgG, ‘fall’ complement (C3, C4), autoimmune antibodies.

Metabolic studies

• Sweat test (cystic fibrosis); A₁-antitrypsin level and phenotype.
• d Serum copper and caeruloplasmin (Wilson’s disease).
• ‘fall’ 24hr urinary copper (Wilson’s disease).

The abdominal US

• Hepatomegaly.
• Echogenic liver.
• Splenomegaly.
• Ascites.

Upper GI endoscopy

• Oesophageal or gastric varices.
• Portal hypertension-related gastritis.

EEG 

To confirm chronic encephalopathy if suspected.

Liver biopsy 

Histology; enzymes; electron microscopy.

Management

• Treat the underlying cause and give nutritional support.
• Lower protein, increased energy, higher carbohydrate diet.
• Vitamin supplementation, particularly fat-soluble vitamins A, D, E, K. Involve a paediatric dietitian.

Drug therapy

• Prednisolone +/– azathioprine for autoimmune hepatitis.
• Interferon-A +/– ribavirin for chronic viral hepatitis.
• Penicillamine for Wilson’s disease.
• Colestyramine may be useful to control severe pruritis.
• Vitamin K₁and FFP (10mL/kg) if significant coagulopathy or bleeding.

Oesophageal varices 

Endoscopy, i.e. sclerotherapy or surgery.

Ascites

• Fluid and Na⁺restriction (2/3 maintenance and 1mmol/kg/day, respectively).
• Spironolactone (1–2mg/kg 12-hourly).
• Consider IV 20% albumin if ascites is resistant to the above treatment.

Encephalopathy 

Reduce GI ammonia absorption using oral or rectal lactulose, neomycin, or soluble fibre pectin.

Liver transplantation.

Prognosis

There is up to 50% 5yr mortality without liver transplant. Poor prognostic factors are:

• bilirubin >50µmol/L;
•  albumin <30g/L;
•  PT >6s;
•  ascites;
•  encephalopathy;
•  malnutrition.