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Chapter: Paediatrics: Gastroenterology and nutrition

Paediatrics: 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.

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’PO43 –, ‘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.

 

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