Department of Pediatric Surgery, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa- ku, Nagoya, 466-8550, Japan.
Department of Rare/Intractable Cancer Analysis Research, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya, 466-8550, Japan.
BMC Pediatr. 2023 Jul 25;23(1):376. doi: 10.1186/s12887-023-04190-z.
Immunoglobulin G4-related disease (IgG4-RD) is a systemic inflammatory disease and affected individuals typically present with an increased infiltration of IgG4-positive plasma cells in the pancreas, hepatobiliary tract, and liver but rarely in the gastrointestinal tract.
A 12-year-old girl presented with vomiting and poor weight gain. Gastroscopy revealed duodenal stenosis and ulceration. Computed tomography revealed edematous duodenal wall thickening and air-fluid levels on the right side of the duodenum, which suggested duodenal perforation or penetration. She underwent pancreaticoduodenectomy, and IgG4-RD was diagnosed via histopathology.
This is the first pediatric case of isolated duodenal IgG4-RD resulting in duodenal obstruction after multiple ulcers. Gastrointestinal IgG4-RD should be among the differential diagnoses of unexplained gastrointestinal obstruction or ulceration even in children.
免疫球蛋白 G4 相关疾病（IgG4-RD）是一种系统性炎症性疾病，受影响的个体通常在胰腺、肝胆道和肝脏中表现出 IgG4 阳性浆细胞的浸润增加，但在胃肠道中很少见。
一名 12 岁女孩出现呕吐和体重增长不良。胃镜检查显示十二指肠狭窄和溃疡。计算机断层扫描显示十二指肠壁水肿增厚，十二指肠右侧有气液平面，提示十二指肠穿孔或穿透。她接受了胰十二指肠切除术，组织病理学诊断为 IgG4-RD。
这是首例儿童孤立性十二指肠 IgG4-RD 导致多发性溃疡后发生十二指肠梗阻的病例。即使在儿童中，对于不明原因的胃肠道梗阻或溃疡，也应将胃肠道 IgG4-RD 作为鉴别诊断之一。
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