Viral, autoimmune and metabolic hepatitis — branching viva

Station opening

Examiner: "Classify paediatric hepatitis and outline how the cause sets the management." [1]

Strong candidate (must-hit)

• Classifies paediatric hepatitis into viral, autoimmune and metabolic causes, and by the acute-versus-chronic split: hepatitis A and E are faecal-oral and acute-only, hepatitis B, C and D are parenteral and perinatal and become chronic, autoimmune hepatitis is a chronic loss of tolerance, and the metabolic diseases Wilson and alpha-1-antitrypsin deficiency are chronic; frames the management as supportive care for the acute viral causes, phase-based antiviral suppression for chronic hepatitis B, direct-acting antiviral cure for hepatitis C, immunosuppression for autoimmune hepatitis, and chelation or transplant for the metabolic diseases. [1] [2]

Weak candidate

• "Hepatitis is a liver infection; I would test the liver function and refer to gastroenterology." [1]

Branch A — The school-age child with jaundice after overseas travel

Examiner: "A six-year-old returns from overseas with jaundice, dark urine and pale stools. His father had a milder illness two weeks earlier. What is the diagnosis, and does it become chronic?" [1]

Strong

• Makes the diagnosis of acute hepatitis A from the faecal-oral spread through the household contact and the acute icteric course, confirms it with IgM anti-HAV, and states that hepatitis A is cleared by the immune system and never becomes chronic; management is supportive with hydration and monitoring for fulminant failure, and prevention is the vaccine and hand and food hygiene. [1]

Weak

• "I would admit him for intravenous antibiotics and test for hepatitis B." [1]

Branch B — The perinatally infected boy with a high viral load

Examiner: "A ten-year-old whose mother is hepatitis B surface antigen positive has a positive surface antigen, a positive e antigen, an HBV DNA of two hundred million and persistently normal alanine aminotransferase. What phase is he in, and do you treat?" [2]

Strong

• Identifies the immune-tolerant phase from the high viral load with positive e antigen and persistently normal transaminases, and states that the correct management is surveillance not treatment, because treating here gains little and risks resistance; treatment begins when the child enters the immune-active phase with rising transaminases and active inflammation, using entecavir or tenofovir, and hepatocellular carcinoma surveillance with six-monthly ultrasound begins now. [2] [3]

Weak

• "The viral load is very high, so I would start tenofovir immediately." [3]

Branch C — The adolescent cured of hepatitis C

Examiner: "A fourteen-year-old with vertically acquired hepatitis C is referred for treatment. What can you offer, and what is the expected outcome?" [6]

Strong

• Offers a short course of a direct-acting antiviral regimen such as ledipasvir-sofosbuvir, licensed from three years of age and given as a single daily tablet for eight to twelve weeks, achieving a sustained virologic response in over ninety-five per cent regardless of genotype; confirms cure with an HCV RNA twelve weeks after completion; states that the modern approach is to treat every infected child and that the remaining challenge is case-finding in the vertically infected cohort. [6]

Weak

• "Hepatitis C is incurable, so I would monitor her for cirrhosis and treat the complications." [6]

Branch D — The girl with fatigue and a positive antinuclear antibody

Examiner: "A thirteen-year-old has fatigue, jaundice, amenorrhoea, a raised immunoglobulin G and a positive antinuclear antibody at one in six hundred forty, with viral serology negative and interface hepatitis on biopsy. What is the diagnosis, the score, and the treatment?" [8]

Strong

• Diagnoses definite type 1 autoimmune hepatitis and scores eight out of eight on the simplified International Autoimmune Hepatitis Group score (two for the high-titre antinuclear antibody, two for the raised immunoglobulin G, two for typical interface hepatitis, two for the absence of viral hepatitis), and treats with prednisolone at one to two milligrams per kilogram per day plus azathioprine at one to two milligrams per kilogram per day, checking thiopurine methyltransferase activity before the full azathioprine dose and tapering the steroid to maintenance. [8]

Weak

• "I would start interferon and ribavirin." [8]

Branch E — The child in acute liver failure with Coombs-negative haemolysis

Examiner: "A nine-year-old presents in acute liver failure with a Coombs-negative haemolytic anaemia and a low urate. The ceruloplasmin is borderline. What is the diagnosis, and what do you do now?" [11]

Strong

• Recognises fulminant Wilsonian crisis from the acute liver failure with Coombs-negative haemolysis and low urate, states that a borderline ceruloplasmin does not exclude Wilson disease because it is an acute-phase reactant, sends a twenty-four-hour urinary copper and a slit-lamp examination for the Kayser-Fleischer ring, and refers urgently for liver transplantation because the only curative treatment is transplant, with chelation and plasmapheresis as a bridge. [11]

Weak

• "The ceruloplasmin is normal, so Wilson disease is excluded; I would treat as a viral hepatitis." [11]

Close

Examiner: "Summarise your approach to a child with hepatitis in one sentence." [1]

Strong

• "Paediatric hepatitis is classified into viral, autoimmune and metabolic causes: I separate the faecal-oral acute viruses A and E from the parenteral and perinatal chronic viruses B, C and D, confirm autoimmune hepatitis with the simplified score of seven or more and treat it with prednisolone and azathioprine, diagnose Wilson disease with the Leipzig score of four or more combining the Kayser-Fleischer ring and urinary copper and treat it with lifelong chelation, prevent perinatal hepatitis B with the birth-dose vaccine and immunoglobulin within twelve hours, and cure chronic hepatitis C with a short direct-acting antiviral course." [1] [11]