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Referral and warning signs
When to refer
Referral to specialist (Gastroenterologist/Hepatologist/Surgeon) usually required to determine underlying cause and initiate specific management, especially if: Jaundice is persistent or progressive. Evidence of liver failure (encephalopathy, coagulopathy). Suspected biliary obstruction requiring ERCP/surgery. Suspected malignancy.
Red flags
- Altered mental status / Encephalopathy
- Signs of bleeding / Coagulopathy (prolonged INR)
- High fever, chills, severe abdominal pain (suggests cholangitis)
- Weight loss, palpable mass (suggests malignancy)
- Signs of severe haemolysis
- Progressive worsening of jaundice or LFTs
Signs and symptoms
- Yellow discoloration of sclera (icterus) - often first sign.
- Yellow discoloration of skin.
- Dark urine (bilirubinuria - occurs with conjugated hyperbilirubinemia).
- Pale stools (acholic stools - occurs with biliary obstruction).
- Pruritus (itching - common with cholestasis).
- Associated symptoms depend on underlying cause (e.g., fever/malaise in hepatitis, abdominal pain in gallstones/pancreatitis, weight loss/anorexia in malignancy, confusion in liver failure).
Risk factors
- Depend on underlying cause (see specific conditions like Viral Hepatitis, Cirrhosis, Gallstones, Haemolysis, etc.)
Diagnosis
Clinical assessment
Confirm presence of jaundice (check sclera in natural light). History is key: Onset/duration, associated symptoms (pain, fever, weight loss, pruritus, change in urine/stool colour), risk factors (alcohol, drugs/herbs, viral hepatitis exposure, family history, travel). Physical exam: Assess for stigmata of chronic liver disease, hepatomegaly, splenomegaly, abdominal masses, tenderness (e.g., RUQ - Murphy's sign), lymphadenopathy.
Investigations
- Fractionated Bilirubin (Total and Direct/Conjugated): Key initial test. Determines if hyperbilirubinemia is predominantly unconjugated (pre-hepatic/hepatic uptake/conjugation defect) or conjugated (hepatocellular injury or biliary obstruction).
- Liver Function Tests (LFTs):
- - ALT/AST: Markedly elevated suggests hepatocellular injury (hepatitis).
- - ALP/GGT: Markedly elevated suggests cholestasis/biliary obstruction.
- - Albumin, INR/PT: Assess liver synthetic function.
- Complete Blood Count (CBC) & Peripheral Smear: Assess for anaemia, signs of haemolysis (schistocytes, spherocytes), reticulocyte count (elevated in haemolysis), thrombocytopenia (hypersplenism/cirrhosis).
- Coomb's Test: If haemolysis suspected.
- Viral Hepatitis Serology (HAV IgM, HBsAg, Anti-HCV, etc.): Rule out viral causes.
- Abdominal Ultrasound: Essential initial imaging. Assess liver parenchyma, detect biliary dilatation (indicates obstruction), gallstones, masses, ascites, splenomegaly.
- Further investigations based on initial findings:
- - If obstruction suspected: MRCP or ERCP.
- - If hepatocellular pattern: Further serology (autoimmune markers, iron/copper studies), toxicology screen, potentially liver biopsy.
- - If haemolysis suspected: Haptoglobin, LDH.
Diagnostic criteria
Elevated serum bilirubin level, with further classification based on conjugated vs unconjugated fraction and results of investigations identifying the underlying cause.
Management
Non-pharmacological management
- Treat the Underlying Cause.
- Supportive care.
- Avoid hepatotoxins (alcohol, certain drugs/herbs).
Pharmacological treatment
- Treat the Underlying Cause. (e.g., antivirals for viral hepatitis, antibiotics for cholangitis, steroids for autoimmune hepatitis, ursodeoxycholic acid for PBC, chelation for Wilson's).
- Symptomatic treatment for Pruritus (Cholestasis):
- - Cholestyramine (bile acid sequestrant - first line).
- - Rifampicin (induces hepatic enzymes).
- - Naltrexone (opioid antagonist).
- - Antihistamines (limited efficacy, may help with sedation).
- Phototherapy: For neonatal jaundice (unconjugated hyperbilirubinemia) to prevent kernicterus.
Monitoring
- Monitor bilirubin levels and LFTs.
- Monitor clinical symptoms (jaundice, pruritus).
- Monitor based on underlying cause.
Complications
- Depend on underlying cause.
- Kernicterus (bilirubin encephalopathy) in neonates with severe unconjugated hyperbilirubinemia.
- Cholangitis, sepsis (from biliary obstruction).
- Liver failure.
- Cirrhosis.
- Malabsorption of fat-soluble vitamins (chronic cholestasis).
Prevention
- Prevent underlying causes (e.g., HBV vaccination, safe water/food for HAV/HEV, moderate alcohol, avoid hepatotoxic drugs).
Patient education
- Explain jaundice as a sign of an underlying issue.
- Discuss the likely cause based on investigations.
- Explain the treatment plan for the underlying cause.
- Advise on avoiding alcohol/hepatotoxins.
- Explain symptomatic treatment if needed (e.g., for itching).
- Importance of follow-up.
Uganda-specific notes
Viral hepatitis (esp. HBV), malaria (haemolysis), sepsis, traditional herbal medicine toxicity, and advanced liver disease (cirrhosis, HCC, schistosomiasis) are important causes. Fractionated bilirubin and basic LFTs may be available; advanced serology/imaging often limited.
References
- Uganda Clinical Guidelines (UCG) 2023
Chapter 6, Section 6.5.7