Kendirli Tanl, Yüksel Selçuk, Oral Mehmet, Unal Necmettin, Tulunay Melek, Dilek Umman Sanl, Yalçnkaya Fatoş
Division of Pediatric Intensive Care Unit, Ankara University School of Medicine, Dikimevi, Ankara, Turkey.
Pediatr Emerg Care. 2006 Dec;22(12):810-2. doi: 10.1097/01.pec.0000245172.38967.d0.
Polyarteritis nodosa (PAN) is a necrotizing angiitis that predominantly affects small- and medium-sized arteries. Polyarteritis nodosa occurs rarely during childhood. Boys and girls seem to be equally affected, with a peak at the age of 10 years. Rarely, severe and fatal gastrointestinal involvement is seen in PAN. Here, we report a 15-year-old boy with PAN, who had gastrointestinal involvement with multiple aneurysms of the hepatic and superior mesenteric arteries. This involvement could be demonstrated with conventional angiography and gastrointestinal bleeding scintigraphy. The progression of the symptoms and the decrease in the size of the aneurysms were noted after combination treatment with cyclophosphamide and prednisolone, but there was severe bleeding from small bowel, and it was taken under control by resection of jejunum. However, the patient died because of sepsis. In conclusion, severe gastrointestinal involvement in PAN is usually fatal despite aggressive therapy, as is the case in our patient.
结节性多动脉炎(PAN)是一种主要累及中小动脉的坏死性血管炎。结节性多动脉炎在儿童期很少发生。男孩和女孩受影响的几率似乎相同,发病高峰在10岁。在结节性多动脉炎中,罕见严重且致命的胃肠道受累情况。在此,我们报告一名15岁患结节性多动脉炎的男孩,其胃肠道受累,肝动脉和肠系膜上动脉出现多个动脉瘤。这种受累情况可通过传统血管造影和胃肠道出血闪烁扫描得以证实。在环磷酰胺和泼尼松龙联合治疗后,观察到症状进展及动脉瘤大小减小,但小肠出现严重出血,通过空肠切除术得以控制。然而,患者因败血症死亡。总之,尽管积极治疗,结节性多动脉炎严重的胃肠道受累通常是致命的,我们的患者就是如此。