CAJ | 학술논문

目的总结永久性新生儿糖尿病患儿(PNDM)的临床特点,加强对该病的认识并提高临床诊治水平.方法分析北京协和医院2007年8月至2012年8月收治的30例PNDM患儿的临床资料,并对起病特点、症状体征、实验室检查、影像学表现、治疗反应及随诊情况进行分析.结果 30例PNDM患儿中出生体重异常(包括低出生体重和巨大儿)者7例(23.3％)；表现为酮症起病者25例(83.3％)；空腹C肽及胰岛素水平降低者19例(63.3％)；5例(16.6％)伴神经系统异常(包括肌力差、语言发育差、癫痫及中枢神经系统影像学异常)但预后良好；3例(10.0％)伴腹部发育异常(腹部超声结果).26例应用胰岛素皮下注射控制血糖,4例由胰岛素成功转换为格列本脲口服治疗.全部患者血糖控制良好.结论 30例永久性新生儿糖尿病患儿多以酮症起病,大多伴空腹C肽及胰岛素水平下降,部分伴其他系统发育异常.多数患儿需用胰岛素治疗,部分患儿可由胰岛素成功转换为磺脲类口服治疗.
목적 총결영구성신생인당뇨병환인(PNDM)적림상특점,가강대해병적인식병제고림상진치수평.방법 분석북경협화의원2007년8월지2012년8월수치적30례PNDM환인적림상자료,병대기병특점、증상체정、실험실검사、영상학표현、치료반응급수진정황진행분석.결과 30례PNDM환인중출생체중이상(포괄저출생체중화거대인)자7례(23.3％)；표현위동증기병자25례(83.3％)；공복C태급이도소수평강저자19례(63.3％)；5례(16.6％)반신경계통이상(포괄기력차、어언발육차、전간급중추신경계통영상학이상)단예후량호；3례(10.0％)반복부발육이상(복부초성결과).26례응용이도소피하주사공제혈당,4례유이도소성공전환위격렬본뇨구복치료.전부환자혈당공제량호.결론 30례영구성신생인당뇨병환인다이동증기병,대다반공복C태급이도소수평하강,부분반기타계통발육이상.다수환인수용이도소치료,부분환인가유이도소성공전환위광뇨류구복치료.
Objective To summarize the clinical characteristics,treatment response and follow-up data of Chinese permanent neonatal diabetes patients to improve clinical management.Methods A total of 30 permanent neonatal diabetes patients admitted to Peking Union Medical College Hospital from August,2007 to August,2012 were enrolled in this study.The clinical feature of onset,symptoms,signs,treatment response and follow-up data were analyzed.Results Abnormal birth weight,including both small for gestational age (SGA) and macrosomia,was observed in 7 (23.3 ％) cases.Ketosis was the manifestation at diagnosis in 25(83.3％) cases.Fasting C peptide and insulin levels were reduced in 19(63.3％) cases.Nerve system abnormalities were found in 5 cases (16.6％),including strength weakness,poor language function,epilepsy and central nerve system radiographic abnormalities,but with good prognosis.Abdominal malformation was found in 3 cases by ultrasound.A total of 4 cases had been successfully switched from insulin injection to oral glibenclamide.All cases were under good glycemic control without obvious adverse events.Conclusions Ketosis could be the first manifestation at diagnosis among most Chinese permanent neonatal diabetes patients,with reduced fasting C-peptide and insulin levels and sometimes with abnormalities in other systems.Most permanent neonatal diabetes patients should be treated with insulin,while oral glibenclamide could be efficacious in some patients.