Giuseppe Maggiore1, Gérard Socie2, Marco Sciveres3, Anne-Marie Roque-Afonso4, Silvia Nastasio5, Catherine Johanet6, Fréderic Gottrand7, Sébastien Fournier-Favre8, Emmanuel Jacquemin9, Olivier Bernard10. 1. Dipartimento di Medicina Clinica e Sperimentale, Università di Pisa, Gastroenterologia Pediatrica, Azienda Ospedaliera Universitaria Pisana, Pisa, Italy; Epatologia Pediatrica e Trapianto di fegato, ISMETT, University of Pittsburgh Medical Center, Palermo, Italy. Electronic address: giuseppe.maggiore@med.unipi.it. 2. Hématologie/Greffe de Moelle, INSERM U 1160 AP-HP, Université Paris VII, Hôpital Saint Louis, Paris, France. 3. Epatologia Pediatrica e Trapianto di fegato, ISMETT, University of Pittsburgh Medical Center, Palermo, Italy. 4. Virologie, AP-HP, Hôpital Paul Brousse Villejuif, France. 5. Dipartimento di Medicina Clinica e Sperimentale, Università di Pisa, Gastroenterologia Pediatrica, Azienda Ospedaliera Universitaria Pisana, Pisa, Italy. 6. Autoimmunité, AP-HP, Hôpital Saint Antoine, Paris, France. 7. Gastroentérologie, Hépatologie, et Nutrition, INSERM U 995, Hôpital Jeanne de Flandre, Lille, France. 8. Hépatogastroentérologie et Nutrition Pédiatriques, Hôpital Arnaud de Villeneuve, Montpellier, France. 9. Hépatologie Pédiatrique et Centre de référence national de l'atrésie des voies biliaires, Hôpital Bicêtre, AP-HP, Université Paris-Sud 11, Le Kremlin Bicêtre, France; Inserm U 757, Université Paris-Sud 11, Orsay, France. 10. Hépatologie Pédiatrique et Centre de référence national de l'atrésie des voies biliaires, Hôpital Bicêtre, AP-HP, Université Paris-Sud 11, Le Kremlin Bicêtre, France.
Abstract
BACKGROUND: A few children with acute or chronic liver disease display histological features compatible with autoimmune hepatitis, but lack specific serological markers. AIM: To describe features, management and outcome of childhood seronegative autoimmune hepatitis. METHODS: From 1988 to 2010, 38 children were included under the following criteria: negative virological studies, no serum autoantibodies, exclusion of other causes of liver diseases, and liver histology compatible with autoimmune hepatitis. RESULTS: Four groups were identified: (1) 12 with increased serum gamma globulin concentrations; (2) 10 with normal or low serum gamma globulins and no combined blood disease; (3) 10 with combined aplastic anemia; and (4) 6 with peripheral thrombocytopenia with/without neutropenia. Immunosuppressive treatment was associated with aminotransferases normalization in all but one child who required liver transplantation. Relapses occurred in 10 children. Lymphocytopenia was found at the time of the diagnosis of hepatitis in 13 children, 12 in groups 3 or 4. All 38 children are alive after 4-17 years, 18 still under immunosuppression. CONCLUSIONS: Childhood seronegative autoimmune hepatitis includes a spectrum of disorders. Early liver histology is recommended and, if compatible with autoimmune hepatitis, immunosuppressive treatment should be started. Initial lymphocytopenia may indicate future hematological complication.
BACKGROUND: A few children with acute or chronic liver disease display histological features compatible with autoimmune hepatitis, but lack specific serological markers. AIM: To describe features, management and outcome of childhood seronegative autoimmune hepatitis. METHODS: From 1988 to 2010, 38 children were included under the following criteria: negative virological studies, no serum autoantibodies, exclusion of other causes of liver diseases, and liver histology compatible with autoimmune hepatitis. RESULTS: Four groups were identified: (1) 12 with increased serum gamma globulin concentrations; (2) 10 with normal or low serum gamma globulins and no combined blood disease; (3) 10 with combined aplastic anemia; and (4) 6 with peripheral thrombocytopenia with/without neutropenia. Immunosuppressive treatment was associated with aminotransferases normalization in all but one child who required liver transplantation. Relapses occurred in 10 children. Lymphocytopenia was found at the time of the diagnosis of hepatitis in 13 children, 12 in groups 3 or 4. All 38 children are alive after 4-17 years, 18 still under immunosuppression. CONCLUSIONS: Childhood seronegative autoimmune hepatitis includes a spectrum of disorders. Early liver histology is recommended and, if compatible with autoimmune hepatitis, immunosuppressive treatment should be started. Initial lymphocytopenia may indicate future hematological complication.
Authors: José V Arcos-Machancoses; Cristina Molera Busoms; Ecaterina Julio Tatis; María V Bovo; Javier Martín de Carpi Journal: J Clin Exp Hepatol Date: 2018-11-08
Authors: Mohammed A Khedr; Nermin M Adawy; Tahany A Salim; Menan E Salem; Ramy M Ghazy; Ahmed S Elharoun; Mervat M Sultan; Nermine A Ehsan Journal: J Clin Exp Hepatol Date: 2021-05-04
Authors: Mohammed A Khedr; Tahany A Salem; Ghada M Boghdadi; Ahmed S Elharoun; Allia A El-Shahaway; Hany R Atallah; Mostafa M Sira Journal: Wien Klin Wochenschr Date: 2021-07-20 Impact factor: 1.704