Paeds · gastroenterology-hepatology-and-nutrition
Viral, autoimmune and metabolic hepatitis
Also known as Hepatitis in children · Paediatric hepatitis · Viral hepatitis A to E · Autoimmune hepatitis · Wilson disease hepatitis · Alpha-1-antitrypsin deficiency hepatitis
Fellowship guide to hepatitis in children across viral, autoimmune and metabolic causes: the faecal-oral acute viruses hepatitis A and E that never become chronic, the parenteral and perinatal chronic viruses hepatitis B, C and D with their serological panels and phases, the birth-dose vaccine and hepatitis B immunoglobulin within twelve hours that prevents perinatal chronicity of ninety per cent, the direct-acting antiviral era for hepatitis C with sustained virologic response above ninety-five per cent, the simplified and revised International Autoimmune Hepatitis Group scoring systems and the type 1 and type 2 antibody profiles treated with prednisolone and azathioprine, the Leipzig score and Kayser-Fleischer ring of Wilson disease with ceruloplasmin under zero point two grams per litre and urinary copper over forty micrograms, and the PiZZ phenotype and PAS-positive diastase-resistant globules of alpha-1-antitrypsin deficiency managed supportively with transplant for end-stage liver disease.
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Red flags
- A child with acute liver failure and a Coombs-negative haemolytic anaemia and low urate has Wilson disease until proven otherwise and is a transplant emergency; send ceruloplasmin, urinary copper and a slit-lamp examination urgently
- Perinatal hepatitis B infection becomes chronic in around ninety per cent of infants without immunoprophylaxis, so every infant of an HBsAg-positive mother must receive hepatitis B vaccine plus hepatitis B immunoglobulin within twelve hours of birth
- Hepatitis A and E are faecal-oral and acute-only and never become chronic, whereas hepatitis B and C become chronic in a large fraction of perinatally infected children; the route of spread and the serology, not the symptoms, set the prognosis
- Ceruloplasmin is an acute-phase reactant and can be normal in early or inflammatory Wilson disease, so a normal ceruloplasmin alone never excludes the diagnosis; combine it with urinary copper and the Kayser-Fleischer ring
- Alpha-1-antitrypsin augmentation therapy treats the lung disease but does not help and does not prevent the liver disease; never offer it for the hepatic phenotype
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Board mappings
- Viral hepatitis in children
- Viral, autoimmune and metabolic hepatitis: serology, the autoimmune hepatitis scoring systems, Wilson disease and the Leipzig score
- Hepatitis B and C in children, autoimmune hepatitis, Wilson disease and alpha-1-antitrypsin deficiency
- Short case: the child with hepatomegaly and jaundice
- Long case: the adolescent with chronic hepatitis and cirrhosis
- Hepatology: viral, autoimmune and metabolic hepatitis
- Hepatitis in children: pathophysiology, diagnosis, and management
- Hepatology: the child with chronic hepatitis and portal hypertension
- Gastroenterology: hepatitis in children
- Patient Care: hepatitis in children
- Medical Expert: hepatitis in children
Overview & Definition
Hepatitis is inflammation of the liver with hepatocellular injury, and in a child it presents most often as a triad of jaundice, dark urine and pale stools, or more subtly as fatigue and raised liver transaminases on a routine blood test. The whole task of the clinician is to move from the inflamed liver to its cause, because the cause alone sets the prognosis and the treatment. A child with hepatitis A will recover fully in weeks and needs supportive care and hygiene advice, whereas a child with perinatally acquired hepatitis B carries a lifelong chronic infection that may end in cirrhosis or hepatocellular carcinoma, and a child with Wilson disease will progress to liver failure unless copper is removed for life. Sorting these outcomes is what the aetiological framework is for. [1] [2]
The causes fall into three families that the title names. Viral hepatitis is caused by the five hepatotropic viruses A to E, which divide cleanly by how they spread and whether they persist. Autoimmune hepatitis is a loss of immune tolerance to liver antigens that drives chronic interface hepatitis and responds to immunosuppression. The metabolic hepatitides are Wilson disease, where a copper-transport defect poisons the liver and the brain, and alpha-1-antitrypsin deficiency, where a misfolded protein accumulates inside hepatocytes. Drug-induced, ischaemic and systemic causes belong in the differential, but the three named families are the ones a fellowship candidate must hold as a model. [8] [11]
The single most important dividing line is acute against chronic. Hepatitis A and E are acute-only infections spread by the faecal-oral route that the immune clears and that never become chronic, whereas hepatitis B and C are parenteral and perinatal viruses that evade clearance and become chronic in a large fraction of children, especially the perinatally infected. Autoimmune and metabolic hepatitis are chronic by nature. This acute-versus-chronic split is the first question to answer at the bedside, because it decides whether the child needs only supportive care or a lifetime of surveillance and treatment. [1] [5]
References12ShowHide
- [1]Abutaleb A; Kottilil S; Wilson E Hepatitis A: Epidemiology, Natural History, Unusual Clinical Manifestations, and Prevention. Gastroenterol Clin North Am, 2020.PMID 32389358
- [2]Indolfi G; Gramenzi A; Degasperi E; Lorini FL; De Santis A; Saccardi F Hepatitis B virus infection in children and adolescents. Lancet Gastroenterol Hepatol, 2019.PMID 30982722
- [3]Terrault NA; Lok ASF; McMahon BJ; Chang KM; Hwang JP; Jonas MM Update on prevention, diagnosis, and treatment of chronic hepatitis B: AASLD 2018 hepatitis B guidance. Hepatology, 2018.PMID 29405329
- [4]Schillie S; Vellozzi C; Reingold A; Harris A; Haber P; Ward JW Prevention of Hepatitis B Virus Infection in the United States: Recommendations of the Advisory Committee on Immunization Practices. MMWR Recomm Rep, 2018.PMID 29939980
- [5]Indolfi G; Easterbrook P; Dusheiko G; El-Sayed MH; Leser M; Thorne C Hepatitis C virus infection in children and adolescents. Lancet Gastroenterol Hepatol, 2019.PMID 30982721
- [6]Indolfi G; Hierro L; Debray D; D'Antiga L; Nossova N; Arenas JI Direct-acting antivirals for children and adolescents with chronic hepatitis C. Lancet Child Adolesc Health, 2018.PMID 30169301
- [7]Verghese VP; Rongsen-Chandola T; Sridhar S; Chitambar SD; Goyal S; Jana A A systematic review of hepatitis E virus infection in children. Clin Infect Dis, 2014.PMID 24846637
- [8]Mieli-Vergani G; Vergani D; Baumann U; Czubkowski P; Debray D; Dezsofi A Diagnosis and Management of Pediatric Autoimmune Liver Disease: ESPGHAN Hepatology Committee Position Paper. J Pediatr Gastroenterol Nutr, 2018.PMID 29356770
- [9]Hennes EM; Zeniya M; Czaja AJ; Parés A; Dalekos GN; Krawitt EL Simplified criteria for the diagnosis of autoimmune hepatitis. Hepatology, 2008.PMID 18537184
- [10]Alvarez F; Berg PA; Bianchi FB; Bianchi L; Burroughs AK; Cancado EL International Autoimmune Hepatitis Group Report: review of criteria for diagnosis of autoimmune hepatitis. J Hepatol, 1999.PMID 10580593
- [11]Schilsky ML; Roberts EA; Ala A; Allen KR; Almeida MP; Anand MK A multidisciplinary approach to the diagnosis and management of Wilson disease: 2022 Practice Guidance from the American Association for the Study of Liver Diseases. Hepatology, 2025.PMID 36151586
- [12]Suri A; Altraja A; Mäkelä S; Krams A; Mindikoglu AL; Galvin Z Alpha-1 Antitrypsin Deficiency Liver Disease. Clin Liver Dis, 2022.PMID 35868681