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 –}, ‘rise’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).

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 K₁ and 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 above treatment.

Encephalopathy 

Reduce GI ammonia absorption using oral or rectal lactu-lose, 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.