Home | | Paediatrics | Paediatrics: Chronic liver failure

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.

 

Study Material, Lecturing Notes, Assignment, Reference, Wiki description explanation, brief detail
Paediatrics: Gastroenterology and nutrition : Paediatrics: Chronic liver failure |


Privacy Policy, Terms and Conditions, DMCA Policy and Compliant

Copyright © 2018-2023 BrainKart.com; All Rights Reserved. Developed by Therithal info, Chennai.