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目的 探讨婴幼儿持续性高胰岛素血症(persistent hyperinsulinemic hypoglycemia in infancy,PHHI)的病理分型以及术中冰冻病理对外科手术治疗方法选择的指导意义.方法 2011年4月至2016年10月,复旦大学附属儿科医院共25例PHHI患儿经外科手术治疗,回顾性分析其相关临床资料、手术治疗经过及术后病理特征.结果 25例患儿中男17例,女8例,年龄16d至12个月,术前经内分泌科明确诊断患有PHHI,空腹血糖0.6～5.5 mmol/L,禁食实验胰岛素水平为3.1～50.1 mU/L.结合术前检查及术中冰冻结果,5例患儿诊断为局灶性病变,行胰腺病灶切除术,20例诊断为弥漫性病变,行胰腺次全切术.术后随访2～38个月,空腹血糖3.0～12.6 mmol/L,15例术后血糖恢复较好,1例仍有低血糖症状,需加用激素治疗,3例空腹时血糖偏低,进食后可恢复,另6例有术后高血糖症状,需药物治疗.1例术前疑诊为局灶性病变,行50％胰腺切除术后2周复发.术后病理检查有5例诊断为局灶型,1例为不典型型,余19例为弥漫型.结论 对于内科治疗无效的PHHI以手术治疗为主,术前明确病理分型对手术方式的选择极为重要,术中快速冰冻切片结合术前辅助检查可以较为准确的指导手术方式.婴幼儿PHHI主要以弥漫型为主,但从术后病理区分弥漫型及局灶性仍存在困难,其中不典型型尚无明确的定义或分类,还有待在病理学方面进一步详细研究.“,”Objective To explore the pathological types and clinical values of intraoperative frozen pathology for surgical children with persistent hyperinsulinemic hypoglycemia of infancy (PHHI).Methods A total of 25 PHHI infants were operated from April 2011 to October 2016.And retrospective analyses were performed for clinical data,surgical approaches and pathological characteristics.Results There were 17 boys and 8 girls with an age range from 16 days to 12 months.The fasting levels of blood glucose and insulin were (0.6-5.5) mmol/L and (3.1-50.1) mU/L respectively.Based upon the preoperative examinations and intraoperative frozen pathology,they underwent focal type and subtotal pancreatectomy respectively.During a postoperative follow-up period of (2-38) months,fasting blood glucose had a range of (3.0-12.6) mmol/L.Fifteen children recovered well without any complication.One case of hypoglycemia required glucagon.And a low fasting level of glucose normalized after ingesting (n =3) and postoperative hyperglycemia required medications for controlling blood glucose (n =6).One case with suspected focal pancreatic lesion underwent a focal resection of 50％ and relapsed after 2 weeks.According to the pathological classification criteria,their clinical types were focal (n =6),diffuse (n =18) and atypical (n =1).Conclusions Clinically it is important to confirm the classification of pathological types.Preoperative examinations and intraoperative frozen pathology may help to find effective treatments and improve the outcomes.The most common pathological type is diffuse Further studies are required for atypical type.