VOL: 102, ISSUE: 01, PAGE NO: 22
Helen L. Day, MSc, BSc, RGN, RSCN, DipN, is paediatric critical care clinical educator and outreach facilitator
Rachel M. Taylor, MSc, RGN, RSCN, DipRes, is nurse researcher; both at the Paediatric Liver Centre, King's College Hospital, LondonPart 4 of this series summarises the most common chronic liver diseases seen in children and adults.
Part 4 of this series summarises the most common chronic liver diseases seen in children and adults.
Jaundice occurs when serum bilirubin rises above 50-100mcmol/L. High concentrations lead to deposits in the skin and eyes, with the patient's colour going from a pale yellow to green if very severe. This is often accompanied by dark urine and pale stools. A complication of unconjugated hyperbilirubinaemia is severe brain damage (kernicterus). Treatment includes drugs and phototherapy for some unconjugated disorders (Whittington, 1996; Kirsch et al, 1995).
Pruritus is intense itching caused by the irritation of the cutaneous sensory nerves, probably by retained bile salts. Treatments include pharmacological and complementary therapies, but these have minimal effect. Severe pruritus can reduce quality of life significantly and can be an indication for liver transplantation (Mela et al, 2003; Whittington, 1996).
Hepato/splenomegaly - A fibrotic liver is enlarged and hard. The spleen enlarges because of the hyperplasia of the reticuloendothelial tissue and congestion. Hepato/splenomegaly is often associated with portal hypertension (Kirsch et al, 1995).
Portal hypertension (PHT) is an increase in portal venous pressure above 5-10mmHg and the formation of portosystemic collaterals, which divert blood to the systemic circulation, bypassing the liver. PHT can be intrahepatic or extrahepatic. In extrahepatic PHT, the liver works normally. The main complication is ruptured varices. The commonest presentation is malaena or haematemesis. Once collaterals are formed, resistance to portal blood flow is higher than normal, so they do not provide total decompression. Varices develop from the collateral circulation and, where they extend toward a superficial surface, such as the rectum, they may rupture, which can be life threatening. Ruptured varices are treated endoscopically with sclerotherapy, or the varix will be banded. Many patients will be given intravenous octreotide acetate. Propranolol is often used, although the efficacy of this in children has not been established (Sherlock and Dooley, 1997).
Ascites is a protein-rich fluid, which accumulates in the peritoneal cavity. It is associated with portal hypertension, hepatocellular damage and a drop in serum albumin. Ascites is thought to develop due to a combination of factors (Fig 1) and can compromise the patient's health in a number of ways:
- Pressure on the abdomen causes diaphragmatic splinting and can result in respiratory distress;
- Increased pressure in the abdomen reduces stomach capacity, causing nausea, vomiting, poor weight gain in children and abdominal compartment syndrome;
- Poor mobility and resulting impeded development.
Treatments of ascites include ascitic tap or intravenous low-sodium albumin infusions to increase oncotic pressure. Monitoring of intra-abdominal pressure may guide further management (Sherlock and Dooley, 1997; Kirsch et al, 1995).
Coagulopathy - The pathophysiology of this was discussed in part 2. Caution is needed with invasive procedures and coagulation factors and blood products may be needed (Sherlock and Dooley, 1997).
Encephalopathy is usually observed in adults and some children with acute liver failure; chronic encephalopathy occurs in some adult patients with chronic liver disease (Sherlock and Dooley, 1997; Kirsch et al, 1995). This will be discussed in part 5.
Malnutrition and failure to thrive - As the liver is responsible for metabolism, malnutrition in adults and children and failure to thrive in infants is commonly observed. Nutritional management is essential to promote recovery from disease, optimise preparation for liver transplantation and aid recovery after transplantation (Whittington, 1996; Baker et al, 1995).
Xanthomas are caused by high serum cholesterol concentrations. Small, yellow nodules of fat, they accumulate under the skin, particularly around joints (Fig 2). They cannot be treated but are harmless and reabsorbed after transplantation (Whittington, 1996).
Spider naevi are superficial arterioles developing into a series of fine, radiating branches on the face, neck, forearms and backs of hands. The presence of 10 or more suggests chronic liver disease (Kirsch et al, 1995).
Palmar erythema - The loss of capillary dilatation in severe liver disease results in the palms of the hands becoming mottled, bright red and warm. This is not life threatening. There is no treatment (Kirsch et al, 1995).
Hepatorenal syndrome may occur in patients with chronic liver disease and PHT. Patients present with impaired renal function but normal tubular function. It may be difficult to distinguish hepatic from renal failure. Survival depends upon the reversibility of the liver disease. Treatment includes pharmacological therapies and liver transplantation (Arroyo et al, 2005).
Hepatopulmonary syndrome - About one-third of adult patients and some children with decompensated cirrhosis are cyanotic. This may be due to intrapulmonary shunting through arteriovenous fistulae, which usually resolves within weeks of liver transplantation (Brown and Zacks, 2000).
Chronic liver disease and associated signs and symptoms present a challenge for clinical management as liver failure affects all body systems.
While this part of the series concentrates on the physical symptoms of chronic liver disease, it needs to be remembered that these can also have many far-reaching implications for the patients' psychosocial well-being.
- This article has been double-blind peer-reviewed.
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