目的粒细胞缺乏性小肠结肠炎在临床上以粒细胞缺乏、腹痛和高热为三大主症。本文回顾了粒细胞缺乏性小肠结肠炎患儿17例,对其临床特征、治疗和预后进行分析。方法总结复旦大学附属儿科医院2004至2009年5年间粒细胞缺乏性盲肠炎患儿的临床资料,并进行文献复习。结果17例患儿,男11例,女6例,平均年龄7.2岁。2例淋巴瘤,11例白血病,1例再生障碍性贫血,1例神经母细胞瘤,2例单纯性粒细胞缺乏而无明显的基础性疾病证据。所有患儿均有典型的临床表现,4例有弥漫性腹膜炎,中毒性休克,其中3例需要呼吸机辅助通气治疗。体检发现有右下腹压痛和肌紧张。伴有CRP的升高和电解质的紊乱。血培养阳性率47%。CT、B超提示肠壁增厚,盲肠或右侧结肠充气减少,气腹等。13例患儿接受内科保守治疗,4例手术干预。2例死亡。结论粒细胞缺乏性小肠结肠炎是由粒细胞缺乏所引起的危及生命的消化道并发症,临床及CT表现可明确诊断。早期发现和积极的内科治疗可减少病死率。大多数的患儿可通过保守治疗,避免外科手术而获得良好的预后。
Objective To summarize our experience with disgnosis and treatment of neutropenic enterocolitis in children. Methods From 2004 to 2009, 17 patients were diagnosed with neutropenic enterocolitis, and treated at this center. Their clinical data were retrospectively analyzed to summarize the clinical features, managment experience and evaluate the outcome of neutropenic enterocolitis. Resuits Among the 17 patients, 11 were males and 6 were females. Their avergae age at diagnosis was 7. 2 years old. The primary diseases that caused neutropenia in these patients were lyphoma in 2 patients, leukemia in 11 patients,aplastic anemia in 1, neuroblastoma in 1, and primary neutropenia in 2. All the patients presented enterocolitis symptoms like fever, vomitting, and diarrhea. Physical examination found lower right abdominal pain and abdominal rigidity. C-reactive protein (CRP) and electrolyte disorder were also noted in these patients. The postive culture rate of blood bacterial was 47%. Computered tomography and ultrasonography revealed intestinal wall thickening, decreased air content in cecum and right colon, and peumoperitoneum. Four patients had diffused peritonitis and sepsis shock. Three of them need mechical ventilation. Among the 17 patients, 4 had surgery. Two patients died. Conclusions Neutropenic enterocolitis is a life-threatening gastrointestinal complication of neutropenia. Early diagnosis and intervention can reduce mortality and improve the prognose of these patients.