儿童门冬酰胺酶相关性胰腺炎21例临床分析  

Clinical analysis of 21 cases of childhood asparaginase-associated pancreatitis

作　　者：张智晓 陆爱东[1] 左英熹[1] 贾月萍[1] 丁明明[1] 张乐萍[1] ZHANG Zhixiao;LU Aidong;ZUO Yingxi;JIA Yueping;DING Mingming;ZHANG Leping(Department of Pediatrics,Peking University People’s Hospital,Beijing 100044,China)

机构地区：[1]北京大学人民医院儿科,北京100044

出　　处：《临床儿科杂志》2021年第11期860-864,共5页Journal of Clinical Pediatrics

基　　金：2018年度北京市临床重点专科建设项目-儿科(No.2199000726)。

摘　　要：目的总结儿童门冬酰胺酶(Asp)相关性胰腺炎(AAP)的临床特征及预后。方法纳入2012年1月至2019年12月初诊并接受Asp联合化疗后出现急性胰腺炎的21例急性淋巴细胞白血病(ALL)患儿,分析其临床特征、实验室检查、治疗情况和预后。结果研究期间初诊并接受Asp治疗的ALL患儿共711例,21例患儿发生AAP,发生率为3.0%。21例患儿中,男10例、女11例,中位年龄8岁(3~18岁);7例AAP发生于诱导化疗期,L-asp中位次数7次(1~10次);14例发生于巩固化疗期,PEG-asp治疗后16.5天(2~22天),中位次数4.5次(1~9次)。轻型AAP 14例,重型7例;腹痛、呕吐为最常见的症状。AAP诊断时,血清淀粉酶和脂肪酶中位数分别为471 U/L(范围25~1632 U/L)和287.1 U/L(范围4.6~2213.7 U/L)。13例患儿行腹部超声,6例(46.2%)阳性;20例行腹部CT,17例(85.0%)阳性。4例AAP患儿出现胰腺假性囊肿。所有患儿均在早期予禁食、抑酸、抑制胰酶分泌、营养支持等治疗,3例采用经鼻空肠管喂养,5例行手术干预。4例轻型AAP患儿再次使用L-asp化疗,其中1例再发AAP。末次随访时,除1例患儿放弃治疗外,无胰腺炎相关死亡病例,1例发生慢性胰腺炎,其余19例胰腺炎已愈。结论AAP是Asp治疗过程中的严重并发症,早期识别是关键,总体预后良好。Objective To analyze clinical characteristics,diagnosis,treatment and prognosis of asparaginase-associated pancreatitis(AAP)in children.Methods From January 2012 to December 2019,21 children with acute pancreatitis who were newly diagnosed with acute lymphoblastic leukemia(ALL)and received asparaginase-containing chemotherapy were included.Their clinical characteristics,laboratory examinations,treatment and prognosis were retrospectively analyzed.Results Among the 711 children newly diagnosed with ALL and treated with L-asparaginase(L-asp)during the study period,the incidence of AAP was 3.0%(n=21).Among the 21 cases,there were 10 boys and 11 girls,with a median age of 8 years(range:3~18 years).Seven cases occurred during induction chemotherapy,after a median L-asp doses of 7(range:1~10 doses),and 14 cases occurred in consolidation chemotherapy,with a median interval of 16.5 days(range:2~22 days)after PEG-asp administration,and the median number of PEG-asp doses was 4.5(range:1~9 doses).Fourteen cases were classified as mild and 7 were severe.Abdominal pain and vomiting were the most common symptoms.The median values of serum amylase and lipase at AAP diagnosis were 471U/L(range:25~1632U/L,upper normal limit[UNL]:125U/L)and 287.1U/L(range:4.6~2213.7U/L,UNL:31U/L)respectively.Thirteen children underwent abdominal ultrasound examination,6(46.2%)were positive;20 cases underwent abdominal CT scan,17(85.0%)were positive.Pancreatic pseudocyst occurred in 4 cases.All children received conservative management including fasting,administration of somatostatin or acid suppression drugs and nutrition support.Three cases received nasojejunal feeding,and 5 cases required surgery.Four cases with mild AAP were re-exposed to L-asp,of which one case had recurrent AAP.Except for one child who gave up treatment,there were no pancreatitis-related deaths.One case developed chronic pancreatitis and the other 19 cases were cured.Conclusion AAP is a serious complication of asparaginase therapy.Early identification is of utmost importance.

关 键 词：门冬酰胺酶 门冬酰胺酶相关性胰腺炎 急性淋巴细胞白血病 儿童 

分 类 号：R725.7[医药卫生—儿科] R733.71[医药卫生—临床医学]