Naslov
Cyclosporin-Induced Biochemical Remission In Childhood Autoimmune Hepatitis

Autori
Žaja Franulović, Orjena ; Lesar, Tatjana, Požgaj Šepec, Marija

Vrsta, podvrsta i kategorija rada
Sažeci sa skupova, sažetak, znanstveni

Izvornik
Journal of Pediatric Gastroenterology, Hepatology and Nutrition - JPGN 2011 ; 52, (Suppl 1) / - , 2011, 188-189

Skup
The 44th Annual meeting of The European Society of Paediatric Gastroenterology, Hepatolology and Nutrition- ESPGHAN

Mjesto i datum
Sorrento, Italija, 24.05.2011. - 27.05.2011

Vrsta sudjelovanja
Poster

Vrsta recenzije
Međunarodna recenzija

Ključne riječi
autoimmune hepatitis, children, therapy, cyclosporine

Sažetak
Objectives and Study: The conventional treatment of autoimmune hepatitis (AIH) in children, which includes prednisone either alone or in combination with azathioprine, induces remission in most cases but is often associated with poorly tolerated side effects. To avoid the adverse effects of steroids, Alvarez et al introduced back in 1999 an alternative treatment regimen, using cyclosporin A (cys) as primary immunosuppression with similar remission rate. We carried out a retrospective study to evaluate the efficacy and tolerance of cyclosporin treatment in 9 children and adolescents with AIH treated in our center. Methods: During 2000–2010 period, 9 children (6 female) were diagnosed with AIH according to established international criteria. Following the suggested protocol, cys was administered orally in 2 divided doses (3–5 mg/kg/d), adjusted to maintain therapeutic serum cys levels within 200–300 ng/mL. After 3 months, when the transaminase activity tended to normalise, oral dose of cys was adjusted to achieve serum concentrations of 100–200 ng/mL. Conversion to low dose of prednisone and azathioprine was started after 6 months, with gradual tapering of cys dose and drug discontinuation over a period of 2 weeks. Results: All nine patients, aged 4.0–17.7 years (median 11.2 y), had elevated transaminases and gammaglobulin levels (Table 1), with proven histological changes typical for AIH in 8 patients that underwent liver biopsy (in one patient biopsy was contraindicated due to the prolonged prothrombin time). Serum ANA/SMA autoantibodies were positive in all but 1 patient, who had positive anti-LKM1. Complete ornear complete and persistent normalisation of transaminase activity was observed in 8/9 patients within first 3–6 months of therapy. In one male adolescent where complete biochemical response was absent, an overlap syndrome was established. After ursodeoxycholic acid was added, complete biochemical and clinical remission was observed. All patients had excellent clinical course and histological improvement on follow-up liver biopsy. During the longterm follow-up (median 4.6 years), biochemical relapse occured in one patient after discontinuation of maintenance corticosteroid dose. Despite registered improvement, none of the patients fulfilled the criteria for therapy discontinuation, so all of them are still receiving maintenance doses of prednisone or azathioprin.

Izvorni jezik
Engleski

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