Paediatric Department, Paediatric Unit, University Hospital, 41124 Modena, Italy.
J Pediatr Surg. 2011 Jul;46(7):E27-9. doi: 10.1016/j.jpedsurg.2011.03.084.
Systemic polyarteritis nodosa (PAN) is a rare disease in childhood affecting small and midsized arteries. The typical presentation in children is of isolated 1- or 2-organ involvement, and the diagnosis is often based on the histopathology. We report a case of pediatric PAN whose presenting symptom was jejunal obstruction owing to ischemic necrosis of the small bowel. The postoperative recovery was uneventful, and the patient responded well to steroid therapy. There are only a few cases of PAN with intestinal involvement described in the literature, some of them fatal. The diagnosis of PAN should be considered in children with acute intestinal obstruction with no obvious etiology, as early recognition and treatment may reduce its high mortality.
系统性多动脉炎(PAN)是一种罕见的儿童疾病,影响小动脉和中等大小的动脉。儿童的典型表现为孤立的 1 个或 2 个器官受累,诊断通常基于组织病理学。我们报告了 1 例儿科 PAN 病例,其首发症状为由于小肠缺血性坏死引起的空肠梗阻。术后恢复顺利,患者对类固醇治疗反应良好。文献中仅描述了少数几例伴有肠道受累的 PAN 病例,其中一些是致命的。对于无明显病因的急性肠梗阻患儿,应考虑 PAN 的诊断,因为早期识别和治疗可能降低其高死亡率。