儿童原发性肾病综合征合并急性胰腺炎的临床特征分析

Clinical feature analysis of acute pancreatitis in children with primary nephrotic syndrome

目的观察原发性肾病综合征(PNS)合并急性胰腺炎(AP)的儿童病例发生率、临床类型及其预后,探讨其发生的相关因素、早期诊断和治疗方法。方法回顾性分析2015年1月1日-2016年3月31日由上海市儿童医院收治的327例PNS患儿的临床资料,筛选并纳入合并AP的6例儿童病例;分析其年龄、身高、体重、BMI、有无不合理饮食(高脂饮食或不规律进餐)、是否合并胆道疾病、PNS病程、免疫抑制剂使用情况、利尿剂使用情况、24 h尿蛋白定量水平、血清淀粉酶和脂肪酶水平、尿淀粉酶水平、相关血清生物化学检查结果、腹部超声检查结果、腹部增强CT检查结果、肾脏活组织病理类型等因素,以及根据Schwatz公式计算的6例患儿估算的肾小球滤过率(eGFR)。结果纳入的327例PNS患儿,年龄3~7岁,平均年龄为(5.51±3.89)岁,平均BMI为(16.87±2.57)kg/m^2。9例患儿腹部超声检查发现合并胰腺病变,6例确诊为AP,AP发生率为1.8%(6/327)。6例患儿腹部超声检查和增强CT检查均提示胰腺形态和结构的病变,并伴有明显的腹部症状。1例患儿血清淀粉酶水平升高。6例合并AP的PNS患儿均为难治性肾病综合征,肾病病程范围为19~43个月,中位病程为28个月。肾脏活组织病理类型主要为肾小球微小病变型(5例)、系膜增生性肾小球肾炎型(1例)。6例患儿在糖皮质激素联合利尿剂治疗肾病的基础上,予醋酸奥曲肽等对症治疗,并调整肾脏原发病的免疫抑制剂治疗方案\[将他克莫司和(或)霉酚酸酯更换为环孢素A或利妥昔单抗\]。患儿均在PNS缓解后出院,3个月内随访结果均正常,未出现相关并发症。结论儿童PNS合并AP并不罕见,但临床表现不典型,腹部超声和增强CT检查的诊断价值较高,早期诊断和及时治疗可使患儿取得良好的预后。

Objective To observe the incidence,clinical types and prognosis of acute pancreatitis in children with primary nephrotic syndrome,and to explore its related factors,early diagnosis and treatment.Methods Six from 327 children with primary nephrotic syndrome hospitalized in Children’s Hospital of Shanghai from January 1 st,2015 to March 31 st,2016 were diagnosed as acute pancreatitis.Their clinical data were retrospectively analyzed,including age,height,weight,BMI,whether there was an unreasonable diet(high fat diet or irregular diet),whether biliary tract disease was complicated,course of nephrotic syndrome,the use of immunosuppressive agents and diuretics,24 h urine protein quantitation,serum amylase and lipase,urine amylase,relevant serum biochemistry tests,abdominal ultrasound,abdominal enhanced CT,renal biopsy pathology,and eGFR calculated by the Schwatz formula.Results The mean age of the 327 children was(5.51±3.89)years(range,3-7 years)and BMI was(16.87±2.57)kg/m^2.Abdominal ultrasound showed pancreatic lesions in 9 patients,and 6 of them were diagnosed as acute pancreatitis.The incidence of acute pancreatitis was 1.8%(6/327)in this group.Morphological and structural lesions of the pancreas were found in both ultrasound and enhanced CT in the 6 patients,and they had obvious abdominal pain and other symptoms.Serum amylase was elevated only in one patient.All the 6 patients had refractory nephrotic syndrome with a median course of 28 months(range,19-43 months).The renal biopsy showed glomerular minimal change disease in 5 patients and mesangial proliferative glomerulonephritis in one patient.The six patients were all improved after receiving octreotide acetate and other supportive treatment based on glucocorticoid combined with diuretic treatment of nephropathy.Immunosuppressive agents for primary nephrotic syndrome were adjusted(tacrolimus and/or mycophenolate were replaced by cyclosporine A or rituximab).Short and medium term prognosis of the child cases was good.Conclusion Acute pancreatitis in children with primary nephrotic syndrome is not rare,but clinical manifestations are atypical.Abdominal ultrasound and enhanced CT have great value to make a definite diagnosis.Early diagnosis and timely treatment can achieve good prognosis.