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目的观察格列本脲治疗新生儿糖尿病(NDM)的疗效。方法收集2010年4月至2011年1月于我院收治并确诊为NDM的4例患儿临床资料,对其进行用格列本脲替代胰岛素治疗的前瞻性研究,分析用格列本脲治疗NDM的方法及疗效,并进行KCNJ11基因和ABCC8基因分析。结果 4例患儿出生体重2.5~3.0kg,就诊年龄3d至2个月,来诊时血糖均明显升高(30.5~45.3mmol/L),均伴有酮症酸中毒。入院后均给予胰岛素治疗,酮症酸中毒纠正,血糖降至15mmol/L以下时,给予格列本脲替换胰岛素治疗,其中2例(50%)格列本脲用量分别为每天0.5mg/kg及0.6mg/kg时可完全停用胰岛素。随访至观察截止日分别为9个月及7个月,均仍然需服用格列本脲每天0.3mg/kg,血糖基本正常,未见明显副反应发生。例2患儿的KCNJ11基因中发现一个突变(R201H),其余3例未发现突变。结论部分NDM患儿应用格列本脲治疗有效,可免除胰岛素注射的痛苦,因此对确诊NDM的患儿应试用本药治疗;同时对NDM患儿应进行KCNJ11基因和ABCC8基因分析,有助于本病临床预后的判断。     

儿童糖尿病酮症酸中毒17例临床分析

目的 分析儿童糖尿病酮症酸中毒(diabetic ketoacidosis,DKA)的临床特点及其相关治疗,以减少临床上的误诊、误治.方法 回顾性分析2003年1月至2007年12月我院收治的17例DKA的临床资料.结果 儿童DKA以原发性糖尿病为多见,伴有少数继发性药物性糖尿病,但疾病初期易误诊(47.1%).针对DKA的治疗以液体复苏、纠正酸中毒和控制血糖为原则,其中液体复苏应首选等张的生理盐水,纠正酸中毒应补充适量的碳酸氢钠,控制血糖以小剂量胰岛素静滴为宜.17例患儿中15例经上述处理均治愈出院,仅2例放弃治疗.结论 及时确诊、小剂量胰岛素静滴调控血糖、液体复苏纠正水电解质紊乱、维持酸碱平衡是救治DKA的关键.     

胰岛素泵治疗儿童1型糖尿病酮症酸中毒32例临床分析

目的 观察胰岛素泵持续皮下注射胰岛素对儿童1型糖尿病酮症酸中毒(DKA)的疗效.方法 将2005-2008年收治的1型DKA患儿64例分为治疗组32例和对照组32例.治疗组予胰岛素泵治疗,对照组予小剂量胰岛素持续静脉滴注治疗.比较两组患儿血精变化、DKA纠正时间及住院时间.结果 治疗组血糖下降相对稳定,酸中毒纠正时间治疗组[(16.91±4.223)h]短于对照组[(23.31±3.797)h](P<0.001),且无反复.治疗过程中治疗组未出现低血糖,对照组出现1例.住院时间治疗组[(15.63±2.458)d]短于对照组[(20.88±3.348)d](P<0.001).结论 胰岛素泵持续皮下注射胰岛索治疗儿童1型糖尿病酮症酸中毒安全有效.     

胰岛素泵治疗儿童1型糖尿病并酮症或酮症酸中毒26例

目的观察胰岛素泵持续皮下注射胰岛素对儿童1型糖尿病并酮症或酮症酸中毒(DK/DKA)的疗效。方法本院内分泌科2003~2005年收治的1型糖尿病并DK/DKA患儿43例,分为治疗组26例和对照组17例。治疗组予胰岛素泵治疗,对照组予小剂量胰岛素持续静脉滴注。比较二组患儿血糖、尿酮体、血pH值变化,住院时间长短。结果1.治疗组血糖下降相对稳定,纠正酸中毒后无反复。2.治疗过程中治疗组未出现低血糖,对照组2例出现。3.住院时间治疗组[(11.92±4.72)d]较对照组[(17.35±4.83)d]治疗组较对照组明显缩短(P<0.001)。结论胰岛素泵持续皮下注射胰岛素治疗儿童1型糖尿病并DK/DKA是安全有效的。     

格列本脲治疗6q24暂时性新生儿糖尿病一例

新生儿糖尿病(neonatal diabetes mellitus, NDM)是一种罕见的基因异质性疾病。迄今为止, NDM至少与26个基因的遗传缺陷有关。其特点是生后6个月内起病, 持续存在严重的高血糖, 持续时间至少2周, 由于胰岛素分泌不足需要依赖外源性胰岛素治疗[1, 2]。NDM的发病率约为1/(40～50)万活产婴儿[3]。NDM诊断需注意与其他引起新生儿高血糖症的病因如肠外静脉输注葡萄糖速度过快、窒息、感染、颅脑损伤、低体温、体外膜肺氧合的应用、类固醇激素药物应用等相鉴别[1, 4, 5]。对疑似NDM患儿的初步评估应包括血糖、尿酮、C肽、胰岛素的实验室评估, 并应进行胰腺超声检查[6]。根据临床表型不同, NDM分为暂时性新生儿糖尿病(transient neonatal diabetes mellitus, TNDM)、永久性新生儿糖尿病(permanent neonatal diabetes mellitus, PNDM)、症状性糖尿病[1, 2, 7, 8]。本研究报道1例TNDM患者, 总结其临床表现、基因特点及诊疗过程, 以期增强临床医师对TNDM的认识, 及...     

连续性血液透析滤过治疗糖尿病酮症酸中毒危重患儿2例北大核心CSCD

对2017年苏州大学附属儿童医院内分泌遗传代谢科和重症医学科收治的2例糖尿病酮症酸中毒危重患儿的临床表现、实验室检查及抢救治疗过程进行回顾性分析。例1,女,13岁5个月,诊断"1型糖尿病、酮症酸中毒、急性肾损伤(3期)";例2,女,2个月8 d,诊断"新生儿糖尿病、酮症酸中毒、高渗高血糖综合征",予常规补液、胰岛素降糖治疗效果不佳,行连续性血液透析滤过治疗后缓解;为明确病因,行基因检测,例2检出KCNJ11基因存在c.602G>A(p.R201H)杂合突变。提示对于儿童糖尿病酮症酸中毒危重患者,在常规补液、胰岛素降糖治疗效果不佳,合并其他器官功能损伤及顽固性酸中毒时,积极行连续性血液透析滤过治疗,可获得显著效果。     

新发儿童1型糖尿病酮症酸中毒与重症儿童应激性高血糖的鉴别诊断

目的 探讨新发儿童1型糖尿病酮症酸中毒与重症儿童应激性高血精的鉴别诊断指标.方法 前瞻性研究30例1型糖尿病酮症酸中毒(DKA组)患儿[年龄(6.5±3.6)岁]和20例重症应激性高血糖(SHG组)患儿[年龄(5.8±3.1)岁]的鉴别诊断指标,分别比较两组糖化血红蛋白(HbAlc)、空腹血糖(FBG)、二氧化碳结合力(CO_2-CP)、阴离子间隙(AG)、空腹C-肽(FCP)、空腹胰岛素(FINS)、胰岛素抵抗指数(IRI)、皮质醇(COR)及是否依赖胰岛素治疗.对照组(C组)30例为健康体检儿童,年龄(6.1±3.4)岁.结果 DKA组和SHG组均有高血糖、AG和皮质醇升高.CO_2-CP降低.DKA组HbAlc显著高于正常对照和SHG组(P均<0.001),DKA组HbAlc>7.3%,SHG组HbAlc<6.5%.FINS、FCP及IRI在DKA组均显著低于SHG组和对照组(P均<0.001),DKA组FINS<2.6 U/L,FCP<0.16μg/L,IRI<2.7(mmol·U./L);SHG组FINS>9.3 U/L,FCP>0.9mg/L,IRI>5.3(mmol·U/L).SHG组对胰岛素无依赖性,而DKA组需依赖胰岛素才能控制血糖.结论 HbAlc、FINS、FCP及IRI是鉴别DKA和SHG的简便、良好的指标.DKA组HbAlc显著升高,FINS、FCP及IRI均显著降低;SHG组FINS、FCP及IRI均显著升高,HbAlc<6.5%.且DKA的治疗依赖胰岛素,而SHG组治疗不依赖胰岛素.     

胰岛素泵在1型糖尿病患儿的合理使用

目的 探讨胰岛素泵强化治疗对1型糖尿病(T1DM)患儿的疗效,并分析影响疗效和胰岛素用量的因素.方法 本院内分泌科2003-2008年收治的T1DM患儿68例.对其进行短期胰岛素泵强化治疗,将其分为初诊组和复诊组、感染组和非感染组,并酮症酸中毒(DKA)和非DKA组,观察影响使用胰岛素泵治疗效果的因素,在年龄、初诊、感染和并DKA等情况下胰岛素泵的使用情况.结果 68例患儿均予胰岛素泵强化治疗,血糖达标天数为(4.37±1.60) d,达标时胰岛素用量为(1.22±0.34) U/(kg·d);初诊组和复诊组血糖达标时间、胰岛素用量比较均无显著差异(Pa>0.05);非感染组达标时间明显均较感染组、并DKA组短(Pa<0.05);与非感染组比较,感染组、并DKA组基础胰岛素用量大(Pa<0.01),小年龄组胰岛素输注管堵塞和发生低血糖例次明显多于大年龄组儿童.结论 胰岛素泵的使用在T1DM患儿存在着差别,小年龄组胰岛素泵使用要慎重.     

儿童糖尿病酮症酸中毒13例治疗体会

糖尿病酮症酸中毒（DKA）是儿科急症之一，也是儿童糖尿病死亡的主要原因。因此，早期诊断、及时治疗是关键。DKA是体内胰岛素水平低下或缺乏引起的高血糖、高有机酸及酮体堆积引起的代谢性酸中毒。若不及时治疗引起糖尿病昏迷后果相当严重。现在就我院近5年来儿科住院患儿DKA13例的诊治经过进行概括总结如下。     

儿童1型糖尿病胰岛素泵治疗长期随访的疗效观察

目的 比较1型糖尿病(T1DM)患儿应用持续皮下胰岛素输注(CSII)与每日多次皮下胰岛素注射(MDI)治疗对血糖控制的疗效差异.方法 回顾性收集91例应用CSII方式治疗1年以上T1DM患儿的临床资料,评估其糖化血红蛋白(HbA1C)水平、糖尿病酮症酸中毒(DKA)再发生情况,通过与75例应用MDI治疗的T1DM患儿...     

Neonatal diabetes mellitus in China: a case report and review of the Chinese literature

To recognize the clinical characteristics and outcomes of neonatal diabetes mellitus (NDM), the authors retrospectively reviewed 1 NDM baby in their department and compared their data with 39 NDM cases reported in the available Chinese literature between January 1986 and December 2010. Most of the cases were located near the eastern and southern coasts of China, and clinical manifestation of 72.5% of the cases occurred in 4-week-old infants. Hyperglycemia and glycosuria findings were seen in all the patients and in 47.5% with intrauterine growth retardation. Moreover, 30.0% of the cases had polyuria, 52.5% had dehydration, and 47.5% had ketoacidosis. Cases with hyperglycemia, dehydration, and ketoacidosis recovered mostly. Ten NDM cases had persisted after 1 to 11 years of follow-up, 3 cases maintained normal blood sugar, and 7 cases had poor sugar control. NDM is a rare condition and early management includes fluid and insulin and later management depends on the transient or permanent nature of the condition.     

Neonatal diabetes with hyperchylomicronemia

Neonatal diabetes mellitus (NDM) is defined as hyperglycemia occurring in the first few weeks of life. It can be either transient (TNDM) or permanent (PNDM). A 25 days old newborn was brought to the hospital with restlessness, respiratory depression and cyanosis. He was born at term with a birth weight of 2,000 g. There was no consanguinity between his parents. His physical examination findings were as follows: Weight and height were under 3th percentile, he was hypoactive and dehydrated. Serum glucose level was 800 mg/dl; C-peptide was 0.41 ng/ml. Upon investigation for dyslipidemia in association with his neonatal diabetes, hyperchylomicronemia was found both in the patient and his father. Pancreatitis, anemia and cholestasis were also observed. Insulin treatment was started for his diabetes together with a special diet for dyslipidemia. At the end of 28 months of follow-up, dyslipidemia has resolved but the need for insulin therapy was still existing. However, TNDM was considered in differential diagnosis because he was small for gestational age (SGA) at birth and his symptoms had started at the 25th day of the neonatal period. Delayed recovery from insulin dependency brought out the possibility of PNDM. Furthermore, neonatal diabetes combined with hypechylomicronemia is a rare clinical picture. Reported cases of NDM with different clinical evaluation will help to better understanding of this disorder.     

Diabetes mellitus in newborns and infants

Diabetes mellitus is uncommon in infancy and newborn period. The two common forms seen are the transient and permanent forms of diabetes mellitus of the newborn. They have to be differentiated from the transient hyperglycemic states (Blood sugar >125 mg/dl) seen in newborns who receive parenteral glucose infusions and in those with septicemia and CNS disorders. Transient diabetes mellitus of the newborn (TDNB) is defined as hyperglycemia occurring within the first month of life lasting at least 2 weeks and requiring insulin therapy. Most of these cases resolve spontaneously by 4 months. It has a reported incidence of 1 in 45,000 to 60,000 live births. The most likely etiology is a maturational delay of cAMP mediated insulin release. The clinical features include small for datedness, proneness for birth asphyxia, open-eye alert facies, dehydration, emaciation, polyuria and poydipsia. These children are prone to septicemia and urinary tract infections. They have hyperglycemia, glucosuria, absent or mild ketonuria, low basal insulin, C-peptide and IGF-1 levels. Treatment consists of hydration and judicious administration of insulin with close monitoring. Thirty percent of these children are likely to develop permanent neonatal diabetes. Compared to transient form, permanent diabetes mellitus is uncommon. It is usually due to pancreatic dysgenesis often associated with other malformations and rarely due to type 1 diabetes mellitus. The diagnosis is based on the demonstration of both exocrine and endocrine pancreatic dysfunction. These children are managed as type 1 diabetes mellitus. They are prone to develop the vascular complications of diabetes at an earlier date.     

Neonatal diabetes with hyperchylomicronemia

Neonatal diabetes mellitus (NDM) is defined as hyperglycemia occurring in the first few weeks of life. It can be either transient (TNDM) or permanent (PNDM). A 25 days old newborn was brought to the hospital with restlessness, respiratory depression and cyanosis. He was born at term with a birth weight of 2000 g. There was no consanguinity between his parents. His physical examination findings were as follows: Weight and height were under 3^th percentile, he was hypoactive and dehydrated. Serum glucose level was 800 mgJdl; C-peptide was 0.41 ngJml. Upon investigation for dyslipidemia in association with his neonatal diabetes, hyperchylomicronemia was found both in the patient and his father. Pancreatitis, anemia and cholestasis were also observed. Insulin treatment was started for his diabetes together with a special diet for dyslipidemia. At the end of 28 months of follow-up, dyslipidemia has resolved but the need for insulin therapy was still existing. However, TNDM was considered in differential diagnosis because he was small for gestational age(SGA) at birth and his symptoms had started at the 25^th day of the neonatal period. Delayed recovery from insulin dependency brought out the possibility of PNDM. Furthermore, neonatal diabetes combined with hypechylomicronemia is a rare clinical picture. Reported cases of NDM with different clinical evaluation will help to better understanding of this disorder.     

Use of long-term microdialysis subcutaneous glucose monitoring in the management of neonatal diabetes. A first case report

In neonatal diabetes mellitus (NDM), a rare genetic disorder, insulin therapy is required but the management is difficult. Frequent blood glucose determinations are necessary in most cases. Microdialysis subcutaneous glucose monitoring (MSGM) is feasible in neonates and has been proposed to reduce painful blood sampling and blood loss. We have applied long-term MSGM to a small-for-date female newborn with transient NDM. We found a good correlation of subcutaneous and blood glucose concentration over a wide range of values. MSGM enabled a reduction in blood glucose determinations during optimization of intravenous insulin treatment and initiation of continuous subcutaneous insulin infusion. We conclude that long-term MSGM is feasible and may reduce painful blood sampling and blood loss in NDM. Furthermore, long-term MSGM may hold a potential for avoiding hypoglycemic episodes and earlier discharge.     

Neonatal diabetes mellitus: patient report and review of the literature

A female infant born at 33 weeks gestation to a gestationally diabetic mother developed apnea and respiratory distress at 6 hours of age. Laboratory investigation demonstrated persistent hyperglycemia, and the patient was treated with continuous intravenous and subsequent subcutaneous insulin therapy. Detailed laboratory investigation to reveal the etiology of hyperglycemia and further endocrine evaluation were not significant. The baby's insulin requirement has continued thereafter, and she is being followed up in an outpatient clinic still under insulin therapy at 18 months of age. Neonatal diabetes mellitus should be considered in the differential diagnosis of neonatal hyperglycemia, and it may develop in newborns born to diabetic mothers, as well as neonatal hypoglycemia. Insulin treatment with close blood glucose monitoring is essential as long as hyperglycemia persists since neonatal diabetes mellitus may be either transient or permanent and it is not possible to differentiate these two outcomes before 18 months of age.     

Sustained IL-1α, IL-4, and IL-6 elevations following correction of hyperglycemia in children with type 1 diabetes mellitus

Objective:  An imbalance of pro-/anti-inflammatory cytokines may accelerate diabetic vascular complications and interfere with proper wound healing. Currently, limited available literature suggests that plasma concentrations of certain pro- and anti-inflammatory cytokines may be altered during hyperglycemia/diabetes mellitus. It is still unclear, however, whether these concepts also apply to children with diabetes, and whether alterations in circulating cytokine levels are a permanent feature of diabetes or an acute effect of fluctuating glucose concentrations.
Methods:  Twenty-two children with type 1 diabetes mellitus (T1DM) were studied. In 13 children, postprandial morning plasma glucose was >11.1 mmol/L at least once (hyperglycemic group, or HyG group); in 9 subjects, plasma glucose never exceeded 10.6 mmol/L (non-hyperglycemic group, or non-HyG group). After admission, intensive euglycemia (5.0–6.1 mmol/L) was achieved in all participants via intravenous insulin and dextrose for at least 90 min. Blood samples were drawn every 30 min to determine plasma levels of 14 cytokines and chemokines.
Results:  Interleukin IL-1α, IL-4, and IL-6 were elevated in HyG group compared with non-HyG not only when plasma glucose was elevated but also during the first 2 h following return to euglycemia. The levels of the other 11 cytokines were not significantly different.
Conclusions:  Specific cytokines (IL-1α, IL-4, and IL-6) are acutely elevated during hyperglycemia in children with T1DM, and these elevations persist for hours after hyperglycemia has been corrected. Therefore, aside from glycemic control, additional therapeutic measures against elevated proinflammatory signals may be necessary for preventing vascular complications in children with hyperglycemic diabetes.     

Sustained IL-1alpha, IL-4, and IL-6 elevations following correction of hyperglycemia in children with type 1 diabetes mellitus.

OBJECTIVE: An imbalance of pro-/anti-inflammatory cytokines may accelerate diabetic vascular complications and interfere with proper wound healing. Currently, limited available literature suggests that plasma concentrations of certain pro- and anti-inflammatory cytokines may be altered during hyperglycemia/diabetes mellitus. It is still unclear, however, whether these concepts also apply to children with diabetes, and whether alterations in circulating cytokine levels are a permanent feature of diabetes or an acute effect of fluctuating glucose concentrations. METHODS: Twenty-two children with type 1 diabetes mellitus (T1DM) were studied. In 13 children, postprandial morning plasma glucose was >11.1 mmol/L at least once (hyperglycemic group, or HyG group); in 9 subjects, plasma glucose never exceeded 10.6 mmol/L (non-hyperglycemic group, or non-HyG group). After admission, intensive euglycemia (5.0-6.1 mmol/L) was achieved in all participants via intravenous insulin and dextrose for at least 90 min. Blood samples were drawn every 30 min to determine plasma levels of 14 cytokines and chemokines. RESULTS: Interleukin IL-1alpha, IL-4, and IL-6 were elevated in HyG group compared with non-HyG not only when plasma glucose was elevated but also during the first 2 h following return to euglycemia. The levels of the other 11 cytokines were not significantly different. CONCLUSIONS: Specific cytokines (IL-1alpha, IL-4, and IL-6) are acutely elevated during hyperglycemia in children with T1DM, and these elevations persist for hours after hyperglycemia has been corrected. Therefore, aside from glycemic control, additional therapeutic measures against elevated proinflammatory signals may be necessary for preventing vascular complications in children with hyperglycemic diabetes.