目的 探讨幼年皮肌炎（juvenile dermatomyositis，JDM）合并严重胃肠道受累患儿的临床特点、诊断与治疗。方法 回顾性分析3例JDM合并严重胃肠道受累及消化道穿孔患儿的临床资料及诊治经过，并进行文献复习。结果 3例患儿均为女孩，起病年龄2岁7个月至8岁，发现胃肠道受累于幼年皮肌炎确诊后2~26个月，例1消化道症状表现为腹痛、腹胀、发热、呕吐、便血伴肠鸣音减弱。例2消化道症状表现为一过性腹痛，自行缓解。例3患儿无明显消化道症状。例1有十二指肠球部溃疡伴血管裸露、食管炎、阑尾炎、胰腺炎。例2、3均有无症状消化道穿孔。例1给予禁食、胃肠减压、甲基泼尼松龙、环孢素、美罗培南、生长抑素等止血治疗。消化道症状好转，但肌力下降，后自动出院，死亡。例2、3给予禁食、通便、甲基泼尼松龙、环磷酰胺冲击治疗，例2加用人免疫球蛋白支持治疗，2例患儿气腹吸收。结论 JDM患儿病程中任何时候都可能发生消化道穿孔，早期临床表现隐匿，不易发现，出现明显消化道症状时，病情已危重，预后差。一旦发生胃肠道穿孔，需积极治疗原发病及外科干预。

Objective To investigate the clinical characteristics, diagnosis and treatment of children with juvenile dermatomyositis (JDM) combined with severe gastrointestinal involvement. Methods Clinical data, diagnosis and treatment of three children with JDM combined with severe gastrointestinal involvement and gastrointestinal perforation were retrospectively analyzed, and a literature review was conducted. Results All three cases were girls, with onset of disease between 2 years 7 months and 8 years of age. Gastrointestinal manifestations were observed within a timeframe ranging from 2 to 26 months subsequent to the diagnosis of juvenile dermatomyositis. In the first case, the individual presented with abdominal pain, abdominal distension, fever, vomiting, and hematochezia, accompanied by reduced bowel sounds. In the second case, the individual experienced gastrointestinal symptoms characterized by temporary abdominal pain that resolved without intervention. There were no apparent gastrointestinal complaints seen in case 3. The first example exhibited a duodenal bulb ulcer characterized by the presence of exposed blood vessels, along with concurrent conditions of oesophagitis, appendicitis, and pancreatitis. Asymptomatic gastrointestinal perforation was observed in cases 2 and 3. Hemostatic treatment was administered to case 1, which included fasting, gastrointestinal decompression, methylprednisolone, cyclosporine, meropenem, and a growth inhibitor. The patient experienced an amelioration of gastrointestinal complaints, however, there was a subsequent decline in muscle strength. Subsequently, the patient was discharged in an automated manner and subsequently succumbed to mortality. Cases 2 and 3 were subjected to fasting, laxative administration, methylprednisolone treatment, and cyclophosphamide shock therapy. In addition, case 2 received supportive treatment with human immunoglobulin, while two children underwent pneumoperitoneum. In conclusion, it can be inferred that the aforementioned points support the notion that the user’s gastrointestinal perforation has the potential to show at any point throughout the progression of juvenile dermatomyositis (JDM) in children. However, the initial clinical signs of this complication are often inconspicuous and challenging to identify. Once overt gastrointestinal symptoms become apparent, the illness reaches a critical state and the prognosis tends to be unfavorable. Following the occurrence of gastrointestinal perforation, it becomes imperative to initiate aggressive therapy for the underlying condition and proceed with surgicalintervention.