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3 results on '"Andries, Sybille"'

1. Neuroserpin mutation causes electrical status epilepticus of slow-wave sleep

Author

UCL - (SLuc) Service de pédiatrie générale, UCL - (SLuc) Centre neurologique William Lennox, UCL - (SLuc) Service de neurologie pédiatrique, UCL - (SLuc) Centre de référence pour l'épilepsie réfractaire, UCL - MD/NOPS - Département de neurologie et de psychiatrie, UCL - (SLuc) Service de neurochirurgie, UCL - MD/BICL - Département de biochimie et de biologie cellulaire, UCL - (SLuc) Centre de malformations vasculaires congénitales, UCL - MD/MNOP - Département de morphologie normale et pathologique, UCL - (SLuc) Service d'anatomie pathologique, Coutelier, M., Andries, Sybille, Ghariani, Sophie, Dan, B., Duyckaerts, C., Van Rijckevorsel, Kenou, Raftopoulos, Christian, Deconinck, N., Sonderegger, P., Scaravilli, F., Vikkula, Miikka, Godfraind, Catherine, UCL - (SLuc) Service de pédiatrie générale, UCL - (SLuc) Centre neurologique William Lennox, UCL - (SLuc) Service de neurologie pédiatrique, UCL - (SLuc) Centre de référence pour l'épilepsie réfractaire, UCL - MD/NOPS - Département de neurologie et de psychiatrie, UCL - (SLuc) Service de neurochirurgie, UCL - MD/BICL - Département de biochimie et de biologie cellulaire, UCL - (SLuc) Centre de malformations vasculaires congénitales, UCL - MD/MNOP - Département de morphologie normale et pathologique, UCL - (SLuc) Service d'anatomie pathologique, Coutelier, M., Andries, Sybille, Ghariani, Sophie, Dan, B., Duyckaerts, C., Van Rijckevorsel, Kenou, Raftopoulos, Christian, Deconinck, N., Sonderegger, P., Scaravilli, F., Vikkula, Miikka, and Godfraind, Catherine

Published
2008

2. Posttransplant immune hepatitis in pediatric liver transplant recipients: incidence and maintenance therapy with azathioprine.

Author

UCL - MD/CHIR - Département de chirurgie, UCL - MD/GYPE - Département de gynécologie, d'obstétrique et de pédiatrie, UCL - (SLuc) Centre neurologique William Lennox, UCL - (SLuc) Service de neurologie pédiatrique, UCL - (SLuc) Service d'anatomie pathologique, UCL - MD/MNOP - Département de morphologie normale et pathologique, UCL - (SLuc) Centre de pathologie anorectale de l'enfant, UCL - (SLuc) Service de chirurgie et transplantation abdominale, UCL - (SLuc) Service de gastro-entérologie et hépatologie pédiatrique, UCL - (SLuc) Service de pédiatrie générale, UCL - (SLuc) Centre de thérapie tissulaire et cellulaire, Andries, Sybille, Casamayou, Lucia, Sempoux, Christine, Burlet, Muriel, Reding, Raymond, Otte, Jean-Bernard, Buts, Jean-Paul, Sokal, Etienne, UCL - MD/CHIR - Département de chirurgie, UCL - MD/GYPE - Département de gynécologie, d'obstétrique et de pédiatrie, UCL - (SLuc) Centre neurologique William Lennox, UCL - (SLuc) Service de neurologie pédiatrique, UCL - (SLuc) Service d'anatomie pathologique, UCL - MD/MNOP - Département de morphologie normale et pathologique, UCL - (SLuc) Centre de pathologie anorectale de l'enfant, UCL - (SLuc) Service de chirurgie et transplantation abdominale, UCL - (SLuc) Service de gastro-entérologie et hépatologie pédiatrique, UCL - (SLuc) Service de pédiatrie générale, UCL - (SLuc) Centre de thérapie tissulaire et cellulaire, Andries, Sybille, Casamayou, Lucia, Sempoux, Christine, Burlet, Muriel, Reding, Raymond, Otte, Jean-Bernard, Buts, Jean-Paul, and Sokal, Etienne

Abstract

BACKGROUND: Cases of so-called autoimmune hepatitis (AH) have been reported after liver transplantation. Our aim was to evaluate the incidence in a series of 471 pediatric liver transplant recipients. METHODS: Between 1984 and 1998, 471 children had orthotopic liver transplantation (OLT). Children are followed up on a regular basis, with full clinical, biochemical, and histologic evaluation at 6 months, 1, 2, 5, 7, and 10 years after OLT. Children with unexplained abnormal liver tests were screened for autoimmune markers (total gamma-globulins, smooth muscle antibodies [SMA], liver kidney microsome antibodies [LKM], antinuclear factor [ANA]). From January of 1998 until December of 1998, autoimmune markers were prospectively searched in all children admitted for regular posttransplant follow-up (n = 118). RESULTS: Eleven of 471 children (2.35%) were found with autoimmune hepatitis, 9 retrospectively and 2 prospectively. None had previous autoimmune liver disease. Patients had a history of steroid-dependent hepatitis. Histology showed variable degree of portal and lobular inflammation, piecemeal necrosis, and bridging collapse. Mean (+/-SDS) aspartate aminotransferase (AST) and alanine aminotransferase (ALT) activities at diagnosis were 173+/-145 and 196+/-157 IU/L, respectively (nl<32). Median gamma-globulin levels reached 1365 mg/dl versus 931 mg/dl in controls (P<0.05). Nine had ANA (titer 1/80 up to 1/10,000), 1 SMA (1/320), and 2 LKM1 antibodies (1/1280). Patients did not respond to increasing charge of cyclosporine (n=10) or tacrolimus (n=1). Eleven received steroids (prednisolone: 2 mg/kg per day, then tapered) and azathioprine (1.5 to 2.5 mg/kg per day). All patients normalized within 3 months (mean AST/ALT levels of 26+/-8 and 30+/-9 IU/L). Three had mild to moderate relapse with increase of ALT thereafter. Gamma-globulins decreased to 1190 mg/dl (ns). Amongst the 116 remaining prospectively evaluated patients, 85 had normal evaluation, despite low titers of

Published
2001

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