儿童1型糖尿病的10年回顾及白介素-10在糖尿病酮症酸中毒中的临床意义

目的：回顾浙江大学医学院附属儿童医院10年来住院儿童 1 型糖尿病的发病状况并探讨白介素-10（IL-10）在儿童 1 型糖尿病酮症酸中毒（DKA）中的临床意义。方法：对1999年1月至2009年2月在该院住院的263例334例次1型糖尿病患儿的临床资料进行回顾性分析;并对其中48例1型糖尿病患儿进行血脂、细胞因子等检查,根据有无酮症酸中毒分为 DKA组和非DKA组,24例正常健康儿童作为对照组,比较各组间血脂、细胞因子等参数的差异。结果：儿童1型糖尿病患儿中,女性多见（56.3%）,发病年龄以6~11.9岁多见。32.7% 的患儿以酮症酸中毒为就诊表现。DKA组血脂、血糖及糖化血红蛋白均高于非DKA组,二分类logistic 回归分析示上述指标水平的升高均为酮症酸中毒的危险因素。IL-10水平在DKA组明显升高,余细胞因子在DKA组和非DKA组无明显差异。糖尿病组各细胞因子水平明显高于正常对照组。结论：1型糖尿病患儿酮症酸中毒发生率较高,糖、脂代谢紊乱是酮症酸中毒的危险因素。IL-10可能为酮症酸中毒的敏感指标。［中国当代儿科杂志,2010,12（11）：849-854］     

25-羟维生素D与儿童1型糖尿病及酮症酸中毒的相关性研究

目的探讨血清25-羟维生素D[25-(OH)D]水平与儿童1型糖尿病(T1DM)及酮症酸中毒(DKA)的相关性。方法选取2006年1月—2009年12月期间152例住院患儿,其中52例为首次发病的T1DM患儿,包括酮症酸中毒(DKA组)21例,以及非酮症酸中毒(非DKA组)31例,其余100例为非T1DM组。检测并比较三组患儿的血清25-(OH)D水平,分析血清25-(OH)D水平与儿童T1DM及DKA的相关性。结果 DKA组患儿的血清25-(OH)D平均为(53.6±27.8)nmol/L,显著低于非DKA组的(69.7±27.9)nmol/L和非T1DM组的(81.8±28.3)nmol/L(P<0.05);非DKA组患儿的血清25-(OH)D水平显著低于非T1DM组(P<0.05)。结论 T1DM患儿的血清25-(OH)D水平低,尤以DKA患儿最为明显,维生素D在儿童T1DM发病中的潜在保护效应值得关注。     

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。     

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.     

儿童1型糖尿病酮症酸中毒合并低磷血症临床研究

目的 总结儿童1型糖尿病酮症酸中毒（DKA）合并低磷血症的临床特点,探讨DKA治疗期间低磷血症的发生率及预后。方法 回顾性分析2016年1月至2020年6月于广州市妇女儿童医疗中心遗传与内分泌科住院治疗的133例合并DKA的1型糖尿病患儿临床资料,分析入院后4～24h血磷水平,并对影响儿童血磷变化的相关因素进行分析。结果 133例DKA患儿中男55例（41%）,女78例（59%）,年龄6月龄至15岁,平均年龄（6.7±3.9）岁,新发1型糖尿病占72%。75例（56%）患儿出现血磷降低,其中41例（31%）患儿血磷0.81～1.29 mmol/L,22例（16%）患儿血磷0.61～0.81mmol/L,10例（7.5%）患儿血磷0.38～0.61mmol/L,2例（1.5%）患儿血磷<0.38mmol/L。正常血磷组与低血磷组之间pH值、碳酸氢根（HCO3-）、碱剩余（BE-B）、血钾、血氯差异均有统计学意义。酸中毒、年龄≤5岁为影响儿童DKA时血磷降低严重程度的危险因素。75例低血磷患儿均未出现低磷血症相关临床症状,未补充磷酸盐治疗。34例血磷<0.81mmol/L的患儿...     

儿童糖尿病合并酮症酸中毒和高血糖高渗状态临床特征分析

目的了解儿童糖尿病合并酮症酸中毒(DKA)和高血糖高渗状态(HHS)的临床特征。方法依据诊断标准,从1995年1月至2009年12月新确诊的糖尿病患儿中筛选DKA和HHS患儿,比较糖尿病同时合并DKA和HHS(DKA-HHS)与单纯DKA患儿的临床特点。结果 1 065例新诊断儿童糖尿病患儿中有483例合并DKA,占45.35%;16例符合DKA-HHS,占1.50%;无单纯HHS糖尿病患儿。HHS在DKA患儿中的发生率为3.31%,其中1型糖尿病14例,2型糖尿病2例。HHS在年龄≥10岁的DKA患儿中的发生率明显高于小年龄患儿(χ2=6.05,P<0.05)。DKA-HHS患儿中重度脱水的比例、平均有效渗透压、血糖、三酰甘油及尿素氮水平均显著高于单纯DKA患儿,且酸中毒程度明显重于单纯DKA患儿,差异均有统计学意义(P均<0.05);DKA-HHS患儿的校正血清钠水平显著高于单纯DKA患儿,差异有统计学意义(P<0.05)。结论新诊断糖尿病患儿的HHS发生率较DKA低,且均与DKA合并存在;更常见于≥10岁患儿;DKA-HHS较单纯DKA患儿的代谢紊乱程度、脱水及酸中毒程度更严重,肾功能异常率更高。     

初发1型糖尿病患儿酮症酸中毒的调查

目的调查初发1型糖尿病患儿酮症酸中毒(DKA)的发生情况。方法以224例初发1型糖尿病患儿为研究对象,进行回顾性分析,分为DKA组和未合并DKA组,各112例。DKA组患儿根据年龄分为≥5岁组(65例)和5岁组(47例),并根据酸中毒情况分为轻度(26例)、中度(29例)、重度(57例)3组。分析DKA发生的影响因素以及不同年龄DKA患儿的临床及实验室特点。结果 224例初发1型糖尿病患儿中最常见的症状为多饮(86.2%)、多尿(78.6%)及体重下降(57.1%)。与未合并DKA患儿比较,DKA组5岁、低收入、父母教育程度高中及以下所占的比例均较高,随机血糖、Hb A1C水平较高,pH、HCO_3~-及C肽水平更低,差异均具有统计学意义(P0.05)。≥5岁组与5岁组的轻、中、重度DKA所占比例的差异无统计学意义(P0.05)。与5岁组相比,≥5岁组DKA患儿的症状持续时间较长,随机血糖较低,HbA1C、C肽水平较高,差异具有统计学意义(P0.05)。结论 1型糖尿病患儿DKA发生率高,DKA的发生与年龄、父母文化程度及家庭收入有关。     

儿童糖尿病酮症酸中毒诊疗指南(2009年版)

糖尿病酮症酸中毒(diabetic ketoacidosis,DKA)是以高血糖、高血酮、酮尿、脱水、电解质紊乱、代谢性酸中毒为特征的一组症候群.DKA是糖尿病患儿血循环中胰岛素缺乏/胰岛素抵抗,反调节激素增加,导致代谢紊乱进展,病情不断加重的结果,是儿童糖尿病最常见的死亡原因之一.新发1型糖尿病患儿DKA的发生率与地域、社会经济状况及发病年龄相关,年龄越小,DKA越多.各国报道不一,约15%～70% [1].国内尚缺乏多中心流行病学调查的结果,北京地区报道约为20% [2] ,浙江为43% [3].国外报道儿童2型糖尿病患者诊断时DKA的发生率可高达25% [4],而北京儿童医院6年来97例住院2型糖尿病儿童中,首次诊断糖尿病时DKA的发生率为7.4%.DKA的诊断和处理在各地也存在较大差异.为规范儿童DKA的诊断治疗,降低DKA的死亡风险和严重不良事件发生率,儿科学分会内分泌遗传代谢学组根据国内外儿童糖尿病DKA诊治研究进展,拟定2009版DKA诊断治疗指南.     

不同血钾水平1型糖尿病175例的临床特征

目的了解不同血钾水平儿童及青少年1型糖尿病临床特征。方法1型糖尿病患者175例根据血钾水平将其分为3组：A组血钾〈4 mmol/L,C组血钾≥5 mmol/L,B组血钾正常（4~5 mmol/L）。对3组性别、年龄、住院时间、伴发呕吐、感染、酮症酸中毒（DKA）的比例及生化指标等临床特征进行观察。分析血钾紊乱和不同临床表现之间的关系及可能原因。结果血钾异常组较易发生代谢紊乱及伴发症状。A组40例,其发生酮症酸中毒及感染比例比B组要高。A组血氯水平最高。C组36例,发生呕吐比例比B组高,其患儿年龄较A组小,C组入院时血糖水平在3组中最高。结论糖尿病发生代谢紊乱和急性并发症时,易并血钾异常,治疗方面应积极纠正血钾紊乱。     

以酮症酸中毒为首发症状的小儿糖尿病20例分析

该文总结了Ⅰ型糖尿病 (DM ) 32例 ,发生酮症酸中毒 (DKA) 4 0例 (75 % ) ,以DKA为首发症状者2 0例 (6 2 .5 % ) ,指出DKA是儿童Ⅰ型DM最常见的合并症之一 ,亦是Ⅰ型DM患儿死亡的主要原因。并探讨了发生DKA的病因及临床特点 ,引起DKA的原因主要与激素异常和代谢紊乱有关 ,感染可诱发DKA并加重病情 ,DKA时也易感染而掩盖原发病。提高对该病的警惕性 ,防治感染 ,谨防误诊误治 ,早期诊断 ,合理治疗 ,DKA是可以避免的。     

Allergic factors associated with the development of asthma and the influence of cetirizine in a double-blind, randomised, placebo-controlled trial: First results of ETA®

There is a common progression known as the allergic march from atopic dermatitis to allergic asthma. Cetirizine has several antiallergic properties that suggest a potential effect on the development of airway inflammation and asthma in infants with atopic dermatitis. Methods. Over a two year period, 817 infants aged one to two years who suffered from atopic dermatitis and with a history of atopic disease in a parent or sibling were included in the ETAC^® (Early Treatment of the Atopic Child) trial, a multi-country, double-blind, randomised, placebo-controlled trial. The infants were treated for 18 months with either cetirizine (0.25mg/ kg b.i.d.) or placebo. The number of infants who developed asthma was compared between the two groups. Clinical and biological assessments including analysis of total and specific IgE antibodies were performed. Results. In the placebo group, the relative risk (RR) for developing asthma was elevated in patients with a raised level of total IgE (≥ 30 kU/I) or specific IgE (≥ 0.35 kUA/I) for grass pollen, house dust mite or cat dander (RR between 1.4 and 1.7). Compared to placebo, cetirizine significantly reduced the incidence of asthma for patients sensitised to grass pollen (RR = 0.5) or to house dust mite (RR = 0.6). However, in the population that included all infants with normal and elevated total or specific IgE (intention-to-treat - ITT), there was no difference between the numbers of infants developing asthma while receiving cetirizine or placebo. The adverse events profile was similar in the two treatment groups. Discussion. Raised total IgE level and raised specific IgE levels to grass pollen, house dust mite or cat dander were predictive of subsequent asthma. Cetirizine halved the number of patients developing asthma in the subgroups sensitised to grass pollen or house dust mite (i.e. 20% of the study population). In view of the proven safety of the drug, we propose this treatment as a primary pharmacological intervention strategy to prevent the development of asthma in specifically sensitised infants with atopic dermatitis.     

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孤独症谱系障碍(autistic-spectrum disorders,ASDs)近年来患病率逐年攀升至1%左右,其症状往往伴随终生,成为严重威胁儿童健康和发展的神经发育性疾患;注意缺陷多动障碍(attention deficit hyperactivity disorder,ADHD)是儿童期最常见的精神障碍,国内报道患病率为4.13%～5.83%,其症状可延续至青少年期,甚至到成年期[1]。这两类精神障碍在成年期的临床表现、共患病、治疗策略和预后与儿童期有哪些不同呢?本文通过回顾相     

EXCITING NEW TREATMENT APPROACHES FOR PATHYPHYSIOLOGIC MECHANISMS OF SICKLE CELL DISEASE

During the past several decades, our understanding of the complex pathophysiology of vasoocclusion associated with sickle cell disease has improved greatly. Interaction of genes, hemoglobin molecules, red cell membrane and metabolic changes, cell-cell interactions and cell-plasma interactions, red cell adhesion to vascular endothelium, activation of coagulation, and vascular reactivity play a role in vaso occlusion. Penicillin prophylaxis of pneumococcal infections and appropriate use of blood transfusions and other supportive measures improved survival of sickle cell patients. Hydroxyurea made a major impact on sickle cell therapy when it was shown to decrease acute painful episodes, acute chest syndrome, and the need for blood transfusion in adults. Significant experience in the use of hydroxyurea has been accumulated in older children. The benefits and risks of hydroxyurea for younger children and long-term risks in all patients will be evaluated in future investigations. Other promising therapies include butyrate compounds, clotrimazole, magnesium supplementation, poloxamer 188, antiadhesion agents, anticoagulant approaches, and nitric oxide. Hemopoietic transplantation remains the only curative therapy. However, several transgenic mouse models are available for studies of gene therapy or other treatment approaches on biochemical, cellular, and pathologic effects of mutant genes.     

Infiltrations of Epstein-Barr virus-carrying cells in granular lymphocyte-proliferative disorders corresponding to chronic active Epstein-Barr virus infection

A 21-year-old man with granular lymphocyte-proliferative disorders (GLPD) associated with chronic active Epstein-Barr virus (EBV) infection is described. Chromosomal analyses revealed several clonal abnormalities and two of them were mainly repetitious. High copy numbers of monoclonal EBV genome were also detected in the proliferative large granular lymphocytes (LGLs), indicating the monoclonal expansion of EBV-infected LGLs. The patient had an indolent course for several years, and there was no evidence of infiltrations of his bone marrow until the end stage. At autopsy, microscopic studies revealed marked infiltrations of LGL in the liver and spleen, and the infiltrating cells were NK-cell immunophenotype. The infiltrated LGLs showed latency I.     

LUTEINIZING HORMONE RECEPTOR MUTATIONS IN DISORDERS OF SEXUAL DEVELOPMENT AND CANCER

Human male sexual development is regulated by chorionic gonadotropin (CG) and luteinizing hormone (LH). Aberrant sexual development caused by both activating and inactivating mutations of the human luteinizing hormone receptor (LHR) have been described. All known activating mutations of the LHR are missense mutations caused by single base substitution. The most common activating mutation is the replacement of Asp-578 by Gly due to the substitution of A by G at nucleotide position 1733. All activating mutations are present in exon 11 which encodes the transmembrane domain of the receptor. Constitutive activity of the LHR causes LH releasing hormone-independent precocious puberty in boys and the autosomal dominant disorder familial male-limited precocious puberty (FMPP). Both germline and somatic activating mutations of the LHR have been found in patients with testicular tumors. Activating mutations have no effect on females. The molecular genetics of the inactivating mutations of the LHR are more variable and include single base substitution, partial gene deletion, and insertion. These mutations are not localized and are present in both the extracellular and transmembrane domain of the receptor. Inactivation of the LHR gives rise to the autosomal recessive disorder Leydig cell hypoplasia (LCH) and male hypogonadism or male pseudohermaphroditism. Severity of the clinical phenotype in LCH patients correlates with the amount of residual activity of the mutated receptor. Females are less affected by inactivating mutation of the LHR. Symptoms caused by homozygous inactivating mutation of the LHR include polycystic ovaries and primary amenorrhea.     

Resurgence of measles in Singapore: profile of hospital cases

OBJECTIVE: To ascertain the profile of cases of measles seen at a general hospital during a recent outbreak that occurred despite a measles vaccination program. METHODOLOGY: A retrospective study from January 1991 to March 1998. All patients with measles (ICD code 055. 9) seen at the emergency unit or as inpatients were included. RESULTS: There were 87 cases identified. The diagnosis was clinical in all and proven serologically in 71%. Eighty-five per cent of the cases occurred between January 1997 and March 1998. There was a bi-modal age distribution with peaks in the very young (相似文献   

Personality profiles and heart rate variability (vagal tone) in children with recurrent abdominal pain

The aim of the study was to explore psychological factors and autonomic activity in children with recurrent abdominal pain and to compare them with those in a control group of healthy children. The Personality Inventory for Children was used for assessment of developmental, emotional and psychosocial factors in 25 children with recurrent abdominal pain (age, 7-15 y). Parasympathetic and sympathetic functions in these children and in 23 healthy control subjects (age, 7-13 y) were also investigated, non-invasively using a computerized polygraph. Vagal tone (parasympathetic function) was indexed by calculation of respiratory sinus arrhythmia in beats/min. Skin conductance (sympathetic function) was recorded by the constant current method. On the Personality Inventory for Children, 16 patients had high scores on somatic concern. Several patients had scores in the clinical range for depression, withdrawal and anxiety, but the mean scores for these personality profile scales were well within the normal range of healthy children. Interestingly, there was a spike on the L (Lie)-scale for most of the patients and 15 patients had scores above or close to the clinical cut-off value. As compared with the scores in healthy children, vagal tone and sympathetic tone were normal. Conclusion: Many children with recurrent abdominal pain have scores in the clinical range for depression, withdrawal, anxiety and L-scale indicating coping problems, denial and a trend towards somatic concern that may contribute to the evolution of abdominal pain. Autonomic nerve activity was not disturbed in these children.     

Overcoming surfactant inhibition with polymers

Inhibition of the function of pulmonary surfactant in the alveolar space is an important element of the pathophysiology of many lung diseases, including meconium aspiration syndrome, pneumonia and acute respiratory distress syndrome. The known mechanisms by which surfactant dysfunction occurs are (a) competitive inhibition of phospholipid entry into the surface monolayer (e.g. by plasma proteins), and (b) infiltration and destabilization of the surface film by extraneous lipids (e.g. meconium-derived free fatty acids). Recent data suggest that addition of non-ionic polymers such as dextran and polyethylene glycol to surfactant mixtures may significantly improve resistance to inhibition. Polymers have been found to neutralize the effects of several different inhibitors, and can produce near-complete restoration of surfactant function. The anti-inhibitory properties of polymers, and their possible role as an adjunct to surfactant therapy, deserve further exploration.     

Understanding infant formula

The World Health organisation recommends breast feeding infants for the first six months of life. When this breast feeding does not occur either through parental choice or medical need, infant formulas will be required. There is a bewildering array of formulas on the UK market for many different requirements. When faced with an unsettled infant many parents (and healthcare professionals) will experiment with the infant formula available and then attend the paediatric clinic looking for help and advice. It is therefore essential that paediatricians understand what milks are available and what the key differences between different products are. This review attempts to provide a simple guide through many of the formulations currently available in the UK; and offers advice for the dietary management of the child with extra calorie requirements, infants with cow's milk protein allergy, gastro oesophageal reflux disease, apparent unresolved hunger and infantile colic. Whatever the underlying condition, there is likely to be an infant formula that is suitable in this generation of ever expanding formulations.