中华糖尿病杂志
中華糖尿病雜誌
중화당뇨병잡지
CHINES JOURNAL OF DLABETES MELLITUS
2013年
7期
403-407
,共5页
曹冰燕%巩纯秀%吴迪%谷奕
曹冰燕%鞏純秀%吳迪%穀奕
조빙연%공순수%오적%곡혁
糖尿病,永久性新生儿%糖尿病,暂时性新生儿%胰岛素%格列本脲
糖尿病,永久性新生兒%糖尿病,暫時性新生兒%胰島素%格列本脲
당뇨병,영구성신생인%당뇨병,잠시성신생인%이도소%격렬본뇨
Diabetes mellitus,permanent neonatal%Diabetes mellitus,transient neonatal%Insulin%Glyburide
目的 分析13例新生儿糖尿病(NDM)的临床及随访资料,为临床诊治提供参考.方法 对2001年7月至2012年6月北京儿童医院诊治的13例NDM的临床表现、实验室检查及临床随访资料进行回顾性分析.结果 13例NDM的诊断时间为生后12 d～5个月,中位诊断时间为生后3个月.小于胎龄儿(SGA)7例.发热感染为首发症状者8例,尿液黏滞起病者2例,纳差、哭闹为首发症状者1例,反应差起病者1例,单纯抽搐起病者1例.全组4例伴抽搐.合并糖尿病酮症(DK)者4例,合并糖尿病酮症酸中毒(DKA)者6例.诊断时平均糖化血红蛋白为10.0％ (7.4％ ～14.2％).急性期均采用胰岛素治疗,剂量为1.0～1.2 U·kg-1·d-1,血糖控制均较好.随访至今(最短6个月,最长11年).除1例失访未进行分型外,4例为暂时性NDM(TNDM),于诊断后2周～3个月缓解；8例为永久性NDM(PNDM),其中1例Wolcott-Rallison综合征.PNDM中6例曾试用格列本脲治疗,1例有效,3例无效停用,2例因胃肠道反应停用.2例PNDM死亡,其中1例合并严重的智力、体力发育迟缓,诊断为发育迟缓、癫痫和新生儿糖尿病综合征(DEND),一直口服格列本脲治疗,于2.5岁时因感染诱发DKA死亡.另1例死于肝肾功能衰竭.所有PNDM随访时血糖控制均较好,7例有糖化血红蛋白值,中位数为6.7％(4.8％～7.0％).结论 NDM起病时症状不典型,临床易漏诊.近31％为暂时性,可自行缓解.胰岛素治疗均有效,血糖较易控制.婴幼儿对格列本脲耐受差,需要针对性使用.
目的 分析13例新生兒糖尿病(NDM)的臨床及隨訪資料,為臨床診治提供參攷.方法 對2001年7月至2012年6月北京兒童醫院診治的13例NDM的臨床錶現、實驗室檢查及臨床隨訪資料進行迴顧性分析.結果 13例NDM的診斷時間為生後12 d～5箇月,中位診斷時間為生後3箇月.小于胎齡兒(SGA)7例.髮熱感染為首髮癥狀者8例,尿液黏滯起病者2例,納差、哭鬧為首髮癥狀者1例,反應差起病者1例,單純抽搐起病者1例.全組4例伴抽搐.閤併糖尿病酮癥(DK)者4例,閤併糖尿病酮癥痠中毒(DKA)者6例.診斷時平均糖化血紅蛋白為10.0％ (7.4％ ～14.2％).急性期均採用胰島素治療,劑量為1.0～1.2 U·kg-1·d-1,血糖控製均較好.隨訪至今(最短6箇月,最長11年).除1例失訪未進行分型外,4例為暫時性NDM(TNDM),于診斷後2週～3箇月緩解；8例為永久性NDM(PNDM),其中1例Wolcott-Rallison綜閤徵.PNDM中6例曾試用格列本脲治療,1例有效,3例無效停用,2例因胃腸道反應停用.2例PNDM死亡,其中1例閤併嚴重的智力、體力髮育遲緩,診斷為髮育遲緩、癲癇和新生兒糖尿病綜閤徵(DEND),一直口服格列本脲治療,于2.5歲時因感染誘髮DKA死亡.另1例死于肝腎功能衰竭.所有PNDM隨訪時血糖控製均較好,7例有糖化血紅蛋白值,中位數為6.7％(4.8％～7.0％).結論 NDM起病時癥狀不典型,臨床易漏診.近31％為暫時性,可自行緩解.胰島素治療均有效,血糖較易控製.嬰幼兒對格列本脲耐受差,需要針對性使用.
목적 분석13례신생인당뇨병(NDM)적림상급수방자료,위림상진치제공삼고.방법 대2001년7월지2012년6월북경인동의원진치적13례NDM적림상표현、실험실검사급림상수방자료진행회고성분석.결과 13례NDM적진단시간위생후12 d～5개월,중위진단시간위생후3개월.소우태령인(SGA)7례.발열감염위수발증상자8례,뇨액점체기병자2례,납차、곡료위수발증상자1례,반응차기병자1례,단순추휵기병자1례.전조4례반추휵.합병당뇨병동증(DK)자4례,합병당뇨병동증산중독(DKA)자6례.진단시평균당화혈홍단백위10.0％ (7.4％ ～14.2％).급성기균채용이도소치료,제량위1.0～1.2 U·kg-1·d-1,혈당공제균교호.수방지금(최단6개월,최장11년).제1례실방미진행분형외,4례위잠시성NDM(TNDM),우진단후2주～3개월완해；8례위영구성NDM(PNDM),기중1례Wolcott-Rallison종합정.PNDM중6례증시용격렬본뇨치료,1례유효,3례무효정용,2례인위장도반응정용.2례PNDM사망,기중1례합병엄중적지력、체력발육지완,진단위발육지완、전간화신생인당뇨병종합정(DEND),일직구복격렬본뇨치료,우2.5세시인감염유발DKA사망.령1례사우간신공능쇠갈.소유PNDM수방시혈당공제균교호,7례유당화혈홍단백치,중위수위6.7％(4.8％～7.0％).결론 NDM기병시증상불전형,림상역루진.근31％위잠시성,가자행완해.이도소치료균유효,혈당교역공제.영유인대격렬본뇨내수차,수요침대성사용.
Objectives To analyze the clinical features among 13 cases of neonatal diabetes mellitus (NDM).Methods Thirteen cases with NDM received treatment in our department between July 2001 and June 2012.The clinical features were reviewed retrospectively.Results The age at diagnosis was 0.4-5.0 months,the median age of diagnosis was 3 months.Seven cases were small for gestational age (SGA) infants.Infection presented in 8 cases,2 cases presented with viscose urine,2 cases presented with poor reaction,2 cases presented with poor appetite,1 case presented with seizure.Seizure was seen in 4cases.Six cases had diabetic ketoacidosis(DKA).The mean glycated hemoglobin A1c(HbA1c) at onset was 10.0％ (7.4％-14.2％).Insulin treatment was started in all 13 patients,the initial dose was 1.0-1.2 U · kg-1 · d-1,and their blood glucose was well under control.Among the 13 cases followed up,8 cases were verified as permanent NDM (PNDM),4 were transient NDM (TNDM),1 case missed after discharge.One child had skeletal dysplasia and was diagnosed as Wolcott-Rallison syndrome.Six cases were treated with glyburide,only 1 boy who diagnosed as developmental delay,epilepsy and neonatal diabetes syndrome(DEND) got good sugar control,2 cases stopped glyburide treatment for gastrointestinal side effects.Unfortunately,the patient diagnosed with DEND died at the age of 2.5 years because of DKA,another case died of hepatic and renal failure at the age of 1.5 years.The blood glucose of most patients was well controlled at the follow-up.Conclusions The clinical expressions of NDM were atypical and easily miss-diagnosed.Of all the NDM cases,up to 31％ was TNDM and relieved automatically.Insulin was the best choice before genetic identification.
