[Clinical characteristics of autoimmune hepatitis in 11 children].

OBJECTIVE

To investigate the clinical features of autoimmune hepatitis (AIH) in children so as to improve the awareness of the disease.

METHOD

The medical records of 12 children who were clinically diagnosed as AIH between 2004 and 2008 were reviewed. The scoring system of the International Autoimmune Hepatitis Group (IAIHG) for diagnosis of AIH was used to confirm the diagnoses. Clinical manifestations, laboratory examinations, liver pathology results and prognosis were retrospectively analyzed.

RESULT

Eleven patients were diagnosed as AIH by the scoring system and 10 of them were type I, one had not been typed. The average time from onset to diagnosis was (7.5 ± 7.4) months. Seven patients (63.6%) had acute onset, among them 2 cases progressed to subacute severe hepatitis, 3 (27.3%) had deliquescence onset and 1 (9.1%)was complicated with hepatic cirrhosis. Levels of serum globulin and IgG were tested and were higher in 10 cases (90.9%) with average (39.4 ± 7.4) g/L and (31 ± 12) g/L respectively. Antinuclear antibodies (ANA) were measured positive in 10 cases, and 1 was anti-smooth muscle antibody (SMA) positive. Liver-kidney microsomal antibody (LKM-1) and anti-mitochondrial antibody (AMA) were detected in none of them. The liver pathology of 11 cases could be divided into acute and chronic hepatitis in 5 and 6 cases, respectively. Severe submassive liver necrosis and severe fibrosis were identified in 3 cases respectively. Lymphocytes infiltration, interfaces hepatitis and rosette-like annulation of hepatocytes were found in 81.8%, 36.4%, and 18.2% of cases on liver pathology. Eleven patients were followed up with therapy of single glucocorticoids or glucocorticoids combined with immunosuppressive agents. The disease of 2 cases deteriorated and 3 cases died. One case was still under therapy, 1 case was stabilized and 4 cases had recurrence.

CONCLUSION

The children with AIH had diverse symptoms, signs, onsets and laboratory test results. The liver pathological changes were less typical. Rate of misdiagnosis was high in early stage. Prognosis was poor in most cases even though properly treated. Therefore close attention needs to be paid to children with AIH.