학술논문

A pediatric case of hepatic sarcoidosis diagnosed at recurrence of liver dysfunction / 反復する肝機能障害を呈して診断に至った肝サルコイドーシスの一小児例
Document Type
Journal Article
Source
肝臓 / Kanzo. 2021, 62(8):479
Subject
child
hepatic sarcoidosis
hepatitis
noncaseous epithelioid granuloma
steroid pulse therapy
ステロイドパルス
小児
肝サルコイドーシス
肝炎
非乾酪性肉芽腫
Language
Japanese
ISSN
0451-4203
1881-3593
Abstract
An 8-year-old boy was admitted to our hospital because of anorexia, jaundice, and liver dysfunction. Based on liver biopsy, the patient was initially diagnosed with noninfectious acute hepatitis. Treatment with prednisolone (PSL) improved hepatobiliary enzyme levels. The patient was readmitted due to liver dysfunction, which recurred following PSL dose tapering. Although the re-administration of PSL was partly effective, cyclosporine and azathioprine had to be added because the liver function data did not improve sufficiently. Finally, liver biopsy revealed noncaseous epithelioid granulomas in the portal tracts. On the histopathological findings, serum angiotensin-converting enzyme and soluble interleukin-2 receptor levels were elevated; thus, the patient was diagnosed with hepatic sarcoidosis. Combined steroid pulse therapy and azathioprine markedly improved liver function data and histological findings. Case reports of hepatic sarcoidosis in children are rare, but this disease should not be overlooked as a possible cause of acute liver dysfunction based on immunological abnormalities.