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Deflazacort for Type-1 Autoimmune Hepatitis in a Korean Girl

Source: jkms.kams.or.kr
Topic: Autoimmune hepatitis
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Sort Desciption: Autoimmune hepatitis is a chronic necro-inflammatory hepatitis of unknown etiology. It is characterized by autoantibodies that target against liver-specific and non-organspecific antigens ...

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Autoimmune hepatitis is a chronic necro-inflammatory hepatitis of unknown etiology. It is characterized by autoantibodies that target against liver-specific and non-organspecific antigens (1). Corticosteroids, especially prednisone or prednisolone (PD), have been the key drugs of initial antiinflammatory and immuno-suppressive therapy of autoimmune hepatitis in children (2). However, it is well known that long-term use of corticosteroid is associated with the risk of steroid-induced toxicities, and this requires newer immunosuppressive agents for the treatment of autoimmune hepatitis, especially in growing children. We report on the case of a 11-yr-old Korean girl with type1 autoimmune hepatitis who discontinued PD due to steroidinduced toxicities, but who remained in biochemical and clinical remission while undergoing deflazacort and ursodeoxycholic acid (UDCA) combination therapy. CASE REPORT A 9-yr-old girl was admitted to Eul-Ji Hospital because of jaundice and right upper quadrant pain. Her height was 135 cm (75-90 percentile), and weight was 43 kg (over 97 percentile). The sclera was icteric, and the liver edge descended 4 cm below the right costal margin. The results of laboratory studies were as follows: aspartate aminotransferanse (AST) 1,106 IU/L, alanine aminotransferase (ALT) 1,888 IU/L, total bilirubin 25.7 mg/dL, direct bilirubin 18.2 mg/dL, total protein 6.9 g/dL, albumin 4.2 g/dL, serum alkaline phosphatase 1,160 IU/L, GGTP 71 IU/L, INR 2.4, aPTT >120 sec, antinuclear antibody (ANA) (+) speckled pattern, with 1:320 titer, anti-smooth muscle antibody (Ab) (-), antimitochondrial (AMA Ab) (-), IgG 1,397 mg/dL, anti-HAV IgM (-), anti-HBc IgM (-), HbsAg (-), EBV-VCA IgG/M (-/-), and ceruloplasmin 28 mg/dL. Liver biopsy results showed piecemeal necrosis at the portal-lobular interface accompaning mild to moderate infiltration of lymphocytes, moderate centrilobular cholestasis, ...

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