Juvenile Autoimmune Hepatitis: Recent Advances in Diagnosis, Management and Long-Term Outcome.

active chronic hepatitis liver transplantation acute liver failure autoimmune hepatitis autoimmune liver disease

Journal

Diagnostics (Basel, Switzerland)
ISSN: 2075-4418
Titre abrégé: Diagnostics (Basel)
Pays: Switzerland
ID NLM: 101658402

Informations de publication

Date de publication:
24 Aug 2023
Historique:
received: 11 07 2023
revised: 11 08 2023
accepted: 13 08 2023
medline: 9 9 2023
pubmed: 9 9 2023
entrez: 9 9 2023
Statut: epublish

Résumé

Juvenile autoimmune hepatitis (JAIH) is severe immune-mediated necro-inflammatory disease of the liver with spontaneous progression to cirrhosis and liver failure if left untreated. The diagnosis is based on the combination of clinical, laboratory and histological findings. Prothrombin ratio is a useful prognostic factor to identify patients who will most likely require a liver transplant by adolescence or early adulthood. JAIH treatment consists of immune suppression and should be started promptly at diagnosis to halt inflammatory liver damage and ultimately prevent fibrosis and progression to end-stage liver disease. The risk of relapse is high especially in the setting of poor treatment compliance. Recent evidence however suggests that treatment discontinuation is possible after a prolonged period of normal aminotransferase activity without the need for liver biopsy prior to withdrawal.

Identifiants

pubmed: 37685291
pii: diagnostics13172753
doi: 10.3390/diagnostics13172753
pmc: PMC10486972
pii:
doi:

Types de publication

Journal Article Review

Langues

eng

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Auteurs

Silvia Nastasio (S)

Division of Gastroenterology, Hepatology & Nutrition, Boston Children's Hospital and Harvard Medical School, Boston, MA 02115, USA.

Antonella Mosca (A)

Hepatogastroenterology, Rehabilitative Nutrition, Digestive Endoscopy and Liver Transplant Unit, ERN RARE LIVER, Bambino Gesù Children's Hospital, IRCCS, 00165 Rome, Italy.

Tommaso Alterio (T)

Hepatogastroenterology, Rehabilitative Nutrition, Digestive Endoscopy and Liver Transplant Unit, ERN RARE LIVER, Bambino Gesù Children's Hospital, IRCCS, 00165 Rome, Italy.

Marco Sciveres (M)

Pediatric Department and Transplantation, ISMETT, 90133 Palermo, Italy.

Giuseppe Maggiore (G)

Hepatogastroenterology, Rehabilitative Nutrition, Digestive Endoscopy and Liver Transplant Unit, ERN RARE LIVER, Bambino Gesù Children's Hospital, IRCCS, 00165 Rome, Italy.

Classifications MeSH