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...     

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.     

婴儿重症心内膜心肌病致泵及呼吸衰竭的治疗

治疗３７例心内膜心肌病致泵衰竭患儿。年龄１～９个月。使用多巴胺２～１０μｇ·ｍｉｎ￣（－１）／ｋｇ及硝普钠１～５μｇ·ｍｉｎ￣（－１）／ｋｇ治疗，用药时间１２～７２小时，同时吸氧、限制入量及适当纠正代谢性酸中毒，休克缓解后及时使用利尿剂并使其洋地黄化。１５例并发Ｉ型呼吸衰竭者加用鼻塞持续正压通气（ＣＰＡＰ）治疗。ＦｉＯ＿２０．４～０．８，压力０．３８～０．７８ｋＰａ（４～８ｃｍＨ＿２Ｏ），使用时间１２～１０９小时。１例合并ＤＩＣ，加用肝素及冷沉淀物，５例抽搐加用镇静剂及小量甘露醇。结果是除死亡３例，自动出院１例外，余３３例全部恢复。提示血管扩张剂及鼻塞ＣＰＡＰ可迅速改善微循环及心肺功能。     

应用胰岛素泵治疗儿童及青少年1型糖尿病对糖代谢的影响

为观察应用胰岛素泵治疗儿童及青少年1型糖尿病(T1DM)对糖代谢的影响 ,随访10例胰岛素泵治疗的T1DM患儿 ,分别观察胰岛素泵治疗前、后6个月的糖化血红蛋白值(HbA1c)、胰岛素用量、严重低血糖及酮症酸中毒发生次数的变化情况。结果显示 ,胰岛素泵治疗6个月后HbA1c 显著下降 ,治疗前为8.97 %±1.69 %,治疗后为7.51 %±1.17 % (t=2.52 ,P<0.05) ;胰岛素用量无显著下降 ;未发生严重低血糖和酮症酸中毒。表明胰岛素泵治疗可有效控制血糖 ,明显降低HbA1c,减少低血糖及酮症酸中毒的发生 ,是儿童及青少年T1DM常规治疗的较好选择。     

肾上腺皮质激素对血小板输注效果的影响

目的 研究肾上腺皮质激素对血小板输注效果的影响。方法 采用随机对照试验，实验组１１例血液病患儿在输注血小板前半小时输注氢化考的松（７．５ｍｇ／ｋｇ）对照组８例血液病患儿不输注氢化考的松，两组均在输注血小板后１，１８，４２及６６小时分别进行外周血血小板计数，计算纠正计数指数（ＣＣＩ）及回升率，并观察临床疗效，对比两组的输注效果，结果 试验组ＣＣＩ，回升率均明显高于对照组（Ｐ〈０．０５），其止血无效率     

糖尿病酮症与乳糜血症

糖尿病酮症与乳糜血症血浆呈乳糜混浊可由糖尿病酮症所致患儿１２岁。因腹痛、呕吐、不食３天，昏迷１小时入院。半年来多饮、多尿、多食。疑诊糖尿病急取血测血糖等，血浆呈重度乳糜状，血糖３８．０ｍｍｏｌ／Ｌ，用胰岛素治疗后，血糖下降，意识转清，数日后空腹乳糜血...     

急性中枢性尿崩症临床经过和治疗评估

目的 调查分析急性中枢性尿崩症的临床过程和疗效。方法 回顾性总结１９９７年５月～１９９９年１２月我院ＰＩＣＵ的１６例急性中枢性尿崩症患儿临床资料。结果 所有病例均继发于脑损伤和应用垂体加压素治疗，其剂量为０．０００３～０．０００６５Ｕ／（ｋｇ．ｍｉｎ），治疗时间为５小时～１８天，平均２８．５小时。治疗目标达到：尿量２～３ｍｌ（ｋｇ．ｈ），尿比重１．０１０～１．０２０，血钠１４０～１４５ｍｍｏｌ／Ｌ     

早产儿应用脂肪乳剂的安全性研究

目的评价２０％英脱利匹特（ｉｎｔｒａｌｉｐｉｄ）在早产儿应用中的安全性。方法４６例早产儿随机分为４组：治疗组分别于生后第１天（Ａ组,１１例）和第３天（Ｂ组,１２例）经外周静脉按０１５ｇ／（ｋｇ·ｈ）输入０５～３５ｇ／（ｋｇ·ｄ）脂肪乳剂１周;Ｃ（１１例）、Ｄ（１２例）组（不用脂肪乳剂）分别与Ａ、Ｂ组对照,并于实验前后监测血气、血生化、脂肪酸生化指标和血糖的变化。结果生后第１,３天治疗组和对照组动脉血氧分压、氧饱和度、胆红素、丙氨酸转氨酶、过氧化脂质、６－酮－前列腺素Ｆ１α、血栓素Ｂ２及血糖浓度差异均无显著性意义（Ｐ均＞００５）。结论自生后第１天或第３天即开始经外周静脉按０１５ｇ／（ｋｇ·ｈ）速率,逐渐增加剂量,从第１天０５ｇ／（ｋｇ·ｄ）,每天增加１ｇ／（ｋｇ·ｄ）,至最大量３５ｇ／（ｋｇ·ｄ）,供给早产儿２０％ｉｎｔｒａｌｉｐｉｄ１周是安全的     

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.     

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   

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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.     

A profile of respiratory symptoms in urban and rural South Australian school children

This report describes the cross-sectional analyses of data from the first year of a longitudinal study using questionnaire and respiratory function data over a 5 year period from a sample of rural South Australian school children. The cumulative or lifetime prevalences of respiratory symptoms were estimated in 825 rural and 1261 urban school children aged between 5 and 15 years in order to determine if the prevalence rates differed between rural and urban school children. The study found the overall cumulative prevalence of asthma and/or wheezy breathing (AWB) to be 24.1% in the rural school children compared to 27.6% in the urban school children. Most children developed AWB symptoms before the age of 7 years, with 20% reporting moderately severe symptoms and 10% having more than one attack per fortnight. The cumulative prevalence of bronchitis, loose/rattly cough (BLRC) differed significantly between the rural school children (34.1%) and urban school children (47.9%). The BLRC symptoms preceded the development of AWB in many cases. Urban school children also reported a higher prevalence of atopic conditions.     

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.     

Hauttemperaturen verschiedener Körperoberflächenareale des Rumpfes bei Säuglingen

Summary In two groups of infants (3–53 weeks old) skin temperatures were controlled in different areas of the trunk—i.e.: regions of sternum, lungs, heart, liver, spleen, kidneys—at different room-temperatures (group I: 21–25°C; group II: 29–32°C). Rectal temperatures of some probands in both groups also had been controlled simultaneously. A definite change in the reaction to heat was proofed in different periods of the first year of life. In higher environmental temperatures the skin temperature was almost constant at every controll-point of the skin, even in older infants. In lower environmental temperatures the skin temperatures lowered continuously with age till 7. to 9. moth. From 10. to 12. month the lowering of skin temperature discontinued. The rectal temperatures were relatively constant in all infants. Only in infants from 7. to 12. month, whose skin temperatures were controlled in lower as well as in higher environmental temperatures, a tendency to higher rectal temperatures was proofed in warmer environmental temperatures.The significance of these results is discussed.

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Untersuchungen mit Unterstützung durch die Deutsche Forschungsgemeinschaft.