K74.6
DESCRIPTION
The end result of irreversible damage to the liver tissue, causing a widespread, diffuse process of fibrosis with regenerating nodule formation. The fibrosis and abnormal portosystemic vascular connections that result cause ongoing damage. The progression rate is variable, but ultimately results in liver failure.
Causes are divided into biliary cirrhosis due to bile duct obstruction and post necrotic cirrhosis where the lesion is hepatocellular.
Complications include:
- fat malabsorption,
- liver failure,
- portal hypertension,
- ascites secondary to portal hypertension.
DIAGNOSTIC CRITERIA
Clinical
- Clubbing may be present.
- Jaundice.
- Hepatomegaly and/or splenomegaly and/or ascites.
- Signs and symptoms of complications.
Investigations
- Liver enzymes may be normal.
- FBC may show signs of hypersplenism with reduced circulating red cells, white cells and platelets.
- Prolonged prothrombin time/INR.
- Hypo-albuminaemia.
- Ultrasound of the liver and spleen may be abnormal.
- Liver biopsy confirms cirrhosis.
GENERAL AND SUPPORTIVE MEASURES
- Ensure adequate nutrition:
- Consult dietician, if available.
- If not encephalopathic:
- High protein diet, i.e. 3 g/kg/day and medium chain triglyceride supplementation (if cholestatic jaundice).
- High carbohydrate diet, supplement with glucose polymers.
- If high serum cholesterol or if xanthelasma: low cholesterol diet.
MEDICINE TREATMENT
- Multivitamin, oral, 5 mL as a single daily dose.
If INR is abnormal, consider a trial of vitamin K and if no response stop.
- Vitamin K1 (phytomenadione), oral, 2-5 mg three times weekly.
- Monitor INR and titrate dose accordingly.
- In the presence of cholestatic jaundice vitamin K should be given parenterally.
REFERRAL
- All children with suspected cirrhosis should be referred to determine possible cause.