Pyogenic liver abscess and peritonitis due to Rhizopus oryzae in a child with Papillon-Lefevre syndrome
Pyogenic liver abscess and peritonitis due to Rhizopus oryzae in a child with Papillon-Lefevre syndrome
复制标题
DOI：
10.1007/s00431-010-1368-3
复制
发表时间：
2011-06-01
影响因子：
3.6
通讯作者：
Sari, Sinan
中科院分区：
文献类型：
--
作者：
Dalgic, Buket;Bukulmez, Aysegul;Sari, Sinan
乳头-勒菲综合征是一种常染色体隐性遗传病，以对称性掌跖角化皮炎和严重的牙周破坏为特征。组织蛋白酶C基因（CTSC）的突变最近已在PLS中检测到。由于CTSC突变导致的免疫失调增加了PLS患者化脓性感染的风险。一个PLS的孩子被提出在这里与肝脓肿和腹膜炎引起的根霉。他的肝脓肿和腹膜炎用阿替霉素B治愈，无需手术治疗。这是第一例在文献中肝脓肿由于根霉菌在儿童PLS。
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.