期刊
EUROPEAN JOURNAL OF PEDIATRICS
卷 170, 期 6, 页码 803-805出版社
SPRINGER
DOI: 10.1007/s00431-010-1368-3
关键词
Papillon-Lefevre syndrome; Peritonitis; Liver abscess; Rhizopus oryzae
类别
Papillon-Lefevre syndrome (PLS) is an autosomal recessive disease that is characterized by symmetric palmoplantar keratodermatitis and severe periodontal destruction. Mutations in the cathepsin C gene (CTSC) have recently been detected in PLS. Immune dysregulation, due to a mutation in CTSC, increases the risk of pyogenic infections in PLS patients. A child with PLS is presented here with liver abscesses and peritonitis caused by Rhizopus oryzae. His liver abscess and peritonitis were cured with amphotericin B without surgical care. This is the first case in the literature liver abscess due to Rhizopus oryzae in a child with PLS.
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