Seronegative autoimmune hepatitis in children: Spectrum of disorders

Maggiore, Giuseppe; Socié, Gèrard; Sciveres, Marco; Roque-Afonso, A.M.; Nastasio, Silvia; Johanet, Catherine; Gottrand, Frédéric; Fournier-Favre, S.; Jacquemin, Emmanuel; Bernard, Olivier

doi:10.1016/j.dld.2016.03.015

Cited by 49 publications

(60 citation statements)

References 33 publications

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“…Here, we report on nine children who underwent LT for acute hepatitis and developed AA, for example, a severe form of HAAA. Seronegative hepatitis is rare in children and is estimated to comprise 3.1% of acute and chronic hepatitis in a recent series of patients . Among these patients, a subgroup with high risk of AA may be individualized.…”

Section: Discussionmentioning

confidence: 99%

“…Among these patients, a subgroup with high risk of AA may be individualized. These patients present at hepatitis diagnosis with lymphocytopenia (8/10), notably for T‐CD4, which can be predictive of blood disorders, including HAAA …”

Section: Discussionmentioning

confidence: 99%

“…Even if unknown viral trigger cannot be excluded, autoimmune origin is very likely for these forms of hepatitis based on histological and biological criteria and good evolution with IST. To note, an autoimmunity background can be present in some children or in their family …”

Section: Discussionmentioning

confidence: 99%

“…Seronegative hepatitis is rare in children and is estimated to comprise 3.1% of acute and chronic hepatitis in a recent series of patients. 13 Among these patients, a subgroup with high risk of AA may be individualized. These patients present at hepatitis diagnosis with lymphocytopenia (8/10), notably for T-CD4, which can be predictive of blood disorders, including HAAA.…”

Section: Discussionmentioning

confidence: 99%

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Management of childhood aplastic anemia following liver transplantation for nonviral hepatitis: A French survey

Delehaye

Habès

Dourthe

et al. 2020

Pediatric Blood & Cancer

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Background Hepatitis‐associated aplastic anemia (AA) is a rare syndrome combining acute hepatitis of variable severity and AA. Hepatitis may be severe enough to require urgent liver transplantation (LT). Herein, we describe clinical presentation and management of a cohort of pediatric patients diagnosed with AA after undergoing LT for nonviral hepatitis. Methods To describe this rare clinical situation, we performed a national survey and identified nine children treated for AA following LT during the last 10 years in France. Results All patients were treated first for hepatic failure with urgent LT. AA was diagnosed with a median delay of 34 days [21‐200] from the diagnosis of hepatitis. Seven children were treated with antithymocyte globulin/cyclosporine, one with CSA alone and one received bone marrow transplantation. At the last visit (median follow‐up: 4 years), outcomes were excellent: all patients were alive and in hematological remission (complete remission: 7; partial remission: 2). Immunosuppressive therapy was pursued in all patients due to the liver transplant. No unusual toxicities were reported. Conclusion AA after LT is considered a therapeutic challenge. Nevertheless, hematological outcome is good using a standard immunosuppressive approach.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Management of childhood aplastic anemia following liver transplantation for nonviral hepatitis: A French survey

Delehaye

Habès

Dourthe

et al. 2020

Pediatric Blood & Cancer

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“…It is reassuring to note that nearly all children in this group were screened for the common hepatitis viruses, and they recovered without sequelae. In severe cases and for cases that do not resolve quickly, early liaison with paediatric specialists may help identify less common causes, such as hepatitis E virus7 and seronegative hepatitis 8…”

Section: Discussionmentioning

confidence: 99%

Acute infectious hepatitis in hospitalised children: a British Paediatric Surveillance Unit study

et al. 2017

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Prognostic modeling in pediatric acute liver failure

Jain

Dhawan

2016

Liver Transpl

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Liver transplantation (LT) is the only proven treatment for pediatric acute liver failure (PALF). However, over a period of time, spontaneous native liver survival is increasingly reported, making us wonder if we are overtransplanting children with acute liver failure (ALF). An effective prognostic model for PALF would help direct appropriate organ allocation. Only patients who would die would undergo LT, and those who would spontaneously recover would avoid unnecessary LT. Deriving and validating such a model for PALF, however, encompasses numerous challenges. In particular, the heterogeneity of age and etiology in PALF, as well as a lack of understanding of the natural history of the disease, contributed by the availability of LT has led to difficulties in prognostic model development. Several prognostic laboratory variables have been identified, and the incorporation of these variables into scoring systems has been attempted. A reliable targeted prognostic model for ALF in Wilson's disease has been established and externally validated. The roles of physiological, immunological, and metabolomic parameters in prognosis are being investigated. This review discusses the challenges with prognostic modeling in PALF and describes predictive methods that are currently available and in development for the future. Liver Transplantation 22 1418-1430 2016 AASLD.

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Seronegative autoimmune hepatitis in children: Spectrum of disorders

Cited by 49 publications

References 33 publications

Management of childhood aplastic anemia following liver transplantation for nonviral hepatitis: A French survey

Management of childhood aplastic anemia following liver transplantation for nonviral hepatitis: A French survey

Acute infectious hepatitis in hospitalised children: a British Paediatric Surveillance Unit study

Prognostic modeling in pediatric acute liver failure

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