20例儿童甲型HlNl流感病例临床特点分析

目的总结甲型HlNl流感患儿的临床特征,为甲型HlNl流感防治工作积累经验。方法回顾性分析2009年9月至12月我院收治的20例甲型H1N1流感确诊患儿的临床资料,进行统计学分析。结果20例患儿均出现不同程度发热及咳嗽,影像学检查示15例（75％,15／20）表现为不同程度的肺炎,其中4例（20％,4／20）合并纵膈气肿,1例合并肺不张。行肺部CT检查患儿9例,有5例出现胸腔积液,4例伴肺不张。8例患儿行免疫功能检测,IgE均有不同程度的升高。结论儿童感染甲型H1N1流感病毒后易引起较严重的呼吸系统病变,且肺部影像学改变较临床症状恢复慢,甲型川N1流感可导致免疫系统发生改变,存在变态反应。     

11例重症甲型H1N1流感患儿脑电图分析

目的 了解重症甲型H1N1流感患儿的脑电图变化.方法 为我院收治的11例重症甲型H1N1流感患儿在病室内床边记录脑电图,并与同期住院的15例支气管肺炎患儿的脑电图对照.结果 11例重症甲型H1N1流感患儿的脑电图全部异常,对照组支气管肺炎患儿的脑电图53%异常,重症甲型H1N1流感患儿的脑电图异常率明显偏高(P=0.009 8).结论 脑电图检查能够帮助临床发现儿童甲型H1N1流感的中枢神经系统合并症.     

肾病综合征合并甲型H1N1 流感15 例临床研究

目的探讨肾病综合征合并甲型H1N1流感的临床特点。方法回顾分析15例肾病综合征合并甲型H1N1流感患儿的临床表现、实验室及影像学检查、治疗措施及预后。结果 15例确诊肾病综合征患儿均符合甲型H1N1流感诊断标准。患儿中位年龄4岁8个月(2岁2个月~6岁9个月),均单用激素或联合其他免疫抑制剂治疗;重症患儿3例,危重型患儿5例。4例患儿的肾病综合征全面复发,其中2例并发急性肾功能不全。所有患儿在入院时均给予奥司他韦抗病毒治疗,4例在起病48 h内使用奥司他韦,该4例患儿均表现为普通型甲型H1N1流感。14例患儿的甲型H1N1流感治愈出院,尿蛋白明显好转或转阴,中位住院时间8天(1~25天);1例患儿死亡,死于急性坏死性脑病、脑疝。结论肾病综合征患儿是重症或危重型甲型H1N1流感的高危人群,甲型H1N1流感流行期,应对肾病综合征患儿采取临床预防措施。     

小儿甲型H1N1流感危重症诊治体会

目的 探讨小儿甲型H1N1流感危重症患儿的发病特点及治疗措施.方法 2009年10月5日至11月15日期间我院PICU收治11例出现甲型H1N1流感样症状合并重症肺炎、急性呼吸窘迫综合征(ARDS)患儿,对其发病特点、治疗方法及转归等资料进行分析.结果 11例甲型H1N1流感样患儿合并重症肺炎、ARDS,其中6例经咽拭子检测甲型H1N1流感病毒核酸阳性.患儿平均年龄3.9岁(10个月～11岁).所有患儿都表现为发热和呼吸系统症状,从发病到出现危重症状的时间为5～10 d.6例行机械通气治疗.目前全部病例存活,无一例死亡.6例机械通气患儿已有4例安全脱机,2例仍在机械通气中.结论 重症甲型H1N1流感患儿病初为流感症状,无特殊临床表现;病情可在短时间内迅速加重,重症患儿以呼吸困难、低氧血症为突出表现;婴幼儿可伴有嗜睡、烦躁等神经系统症状;重症患儿肺部病变广泛,进展迅速,可在短时间内出现纵隔及皮下气肿、ARDS甚或肺出血并随之出现多脏器功能障碍综合征.     

淋巴瘤患儿感染甲型H1N1流感病毒后所致重症肺炎四例诊治分析

目的 探讨化疗期间免疫抑制的恶性淋巴瘤患儿感染甲型H1N1病毒后所致重症肺炎的临床特点及治疗.方法 回顾性分析4例非霍奇金淋巴瘤患儿化疗期间合并甲型H1N1流感肺炎的临床表现、影像学特点、误诊原因、治疗体会及预后.结果 同期收治的54例恶性血液病患儿中,甲型H1N1流感病毒感染共4例,均为恶性淋巴瘤化疗后患儿,中性粒细胞绝对值均小于0.5×109/L.起病时体温均大于39℃,伴畏寒,血压下降,迅速出现呼吸困难和低氧血症,2例继发急性呼吸窘迫综合征.C反应蛋白均大于50 mg/L,2例大于200 mg/L;4例影像学均提示广泛间实质病变.例1早期被误诊为败血症,4例患儿17次血培养均阴性,20次痰培养中2例患儿各1次痰培养真菌阳性,考虑过真菌性肺炎.4例患儿均使用了磷酸奥司他韦,第1例于第5天加用,余3例于发热的第1天加用;4例均应用了丙种球蛋白,3例应用了甲泼尼龙治疗.治疗后2例死亡,2例好转.结论 恶性淋巴瘤患儿化疗期间合并甲型H1N1流感易致重症肺炎,进展迅速,早期症状与败血症不易鉴别,影像学与单纯真菌感染不易鉴别,易误诊,死亡率高.在H1N1流感流行季节,出现高热及时做病毒筛查,及早应用磷酸奥司他韦,并予大剂量丙种球蛋白冲击及甲泼尼龙等治疗可减少病死率.     

6例急性白血病患儿并发重症甲型H1N1流感的临床和胸部CT表现特点

目的 探讨急性白血病患儿并发重症甲型H1N1流感的临床和胸部CT表现特点.方法 分析6例急性白血病患儿经流行病学、临床、实验室和影像学检查确诊并发重症甲型H1N1流感的临床资料和胸部CT表现.结果 6例患儿入院前虽无明确的甲型H1N1流感密切接触史,但发生在同一时间段、同一病区住院期间,发热为首发症状,5例体温在39....     

儿童危重症甲型H1N1流感呼吸系统并发症的临床与影像对照分析

目的 探讨儿童危重症2009甲型H1N1流感呼吸系统并发症的临床及影像学特点.方法 回顾性分析31例危重症2009甲型H1N1流感患儿的呼吸系统并发症的临床及影像学资料.结果 31例中,男27例(87.1%),年龄中位数5岁,以发热、咳嗽起病,可伴呕吐、抽搐、昏迷等症状,5例死亡.影像学检查均有肺炎表现,其中双肺野中内带多发斑片状模糊影、实变影28例(90.3%),伴磨玻璃样密度影12例(38.7%),多位于外周胸膜下.在初次检查时伴有肺段/叶实变15例(48.4%),可见支气管充气征,伴或不伴节段性肺不张;复查时增至21例(67.7%).在初次检查时伴有胸腔积液7例(22.6%),随访增至12例(38.7%).在初次检查时伴有气胸及纵隔气肿6例(19.3%),随访增至8例(25.8%).结论 儿童危重症2009甲型H1N1流感可出现严重的呼吸系统并发症,影像学特点为双肺多发斑片状模糊影、实变影,伴磨玻璃样密度影;胸腔积液、气胸及纵隔气肿的发生率高;呼吸系统并发症多数愈后良好.     

儿童重症甲型H1N1流感43例临床分析

目的 探讨小儿重症甲型H1N1流感的临床特点及治疗.方法 回顾分析2009年11月 - 2010 年1月长春市儿童医院收治的43例重症甲型H1N1流感患儿的临床特点及治疗情况.结果 43例均为本土病例,男32例,女11例;年龄最大13岁,最小6个月.重症43例中有8例危重症.有明确甲型H1N1流感接触史者7例.均以呼吸道感染为首发症状、体征,包括发热、咳嗽、喘息和肺部啰音、双肺阴影等改变,均以呼吸系统损害为重.危重症可出现呼吸衰竭、多脏器衰竭,部分出现肺水肿、肺出血,病情危重.所有患儿均参照卫生部颁布的<甲型H1N1流感诊疗方案>进行治疗,全部治愈出院.结论 儿童重症甲型H1N1流感主要表现为呼吸系统症状、体征,大部分经过良好,但危重症病例病情进展迅速,病势凶险,很快出现呼吸衰竭,可伴有各个脏器受损,应及时应用机械通气治疗.     

儿童甲型H1N1流感12例分析

目的 了解儿童甲型H1N1流感的特点.方法 回顾分析2009年5月1日至2009年7月15日复旦大学附属儿科医院发热门诊及病房诊治的12例甲型H1N1流感的流行特征及临床特点;采取患儿鼻咽拭子标本,冰壶保存立即送上海市疾病预防控制中心,采用实时逆转录核酸扩增聚合酶链反应(RT-PCR)进行甲型H1N1流感病毒核酸检测.结果 12例儿童甲型H1N1流感均为输入性病例,5例患儿有明确的甲型H1N1流感患者密切接触史.12例有发热症状,有咳嗽、流涕、食欲不佳症状的各为7例,1例有喘息症状,所有病例均无呕吐和腹泻.11例能准确表述自身感受的患儿中,均无肌肉酸痛,6例有咽痛,3例有腹痛.2例患儿并发肺炎,其中1例患儿病情危重.1例患儿居家隔离对症治疗,11例患儿住院治疗,均参照中国国家卫生部颁布的<甲型HINI流感诊疗方案(2009年试行版第一版)>进行治疗,其中10例息儿接受奥斯他韦抗病毒治疗,未见明显不良反应,所有患儿均痊愈.结论 儿童甲型H1N1流感的症状主要表现为典型的流感症状,大部分患儿临床过程轻微,及时隔离和治疗预后良好,奥斯他韦抗病毒治疗无明显副作用.儿童甲型H1N1流感的流行特征及临床特点尚需要多地区大样本的研究资料.     

新型甲型H1N1流感患儿消化系统表现

目的：研究新型甲型H1N1流感患儿消化系统表现。方法：对深圳市儿童医院2009年11月至2010年1月因新型甲型H1N1流感住院的153例患儿及其中有消化系统表现的69例患儿进行前瞻性调研,对其临床特征及转归进行总结。结果：在153例住院的新型甲型H1N1流感患儿中,有消化系统表现者69例（45%）,其中有胃肠道症状者50例（33%）,无胃肠道症状仅有肝功能异常者19例（12%）。有消化道表现患儿出现昏迷、神经系统并发症及肌酸激酶增高的比例、入住PICU及死亡人数均高于无消化道表现的患儿(P<0.05)。除5例患儿死于严重并发症外,其余患儿均痊愈,胃肠道症状一般在1~3 d消失,肝功能多在4~7 d恢复正常。结论：新型甲型H1N1流感患儿消化道表现较常见,有消化道表现的患儿神经系统受累比无消化道表现的甲型H1N1流感患儿更多见,应该引起临床医生关注。［中国当代儿科杂志,2010,12（10）：793-795］     

LINE1-ORF1p过表达对肾母细胞瘤细胞WT_CLS1增殖的影响

目的 制备LINE1-ORF1p多克隆抗体,研究LINE1-ORF1p过表达对肾母细胞瘤细胞WT_CLS1增殖的影响。方法 利用基因工程方法原核表达LINE1-ORF1p,免疫家兔制备多克隆抗体。间接ELISA法检测抗体效价,通过Western blot及免疫组化方法检测抗体对LINE1-ORF1p的特异性识别能力。构建真核表达载体pEGFP-N1-LINE1-ORF1,转染WT_CLS1细胞,通过Western blot和qRT-PCR检测LINE1-ORF1蛋白和基因的表达情况,采用细胞增殖实验和平板克隆形成实验检测LINE1-ORF1p对WT_CLS1细胞增殖及肿瘤细胞克隆形成的影响。结果 制备的LINE1-ORF1p抗体效价 > 1：16 000,能对细胞及肿瘤组织内LINE1-ORF1p特异识别。转染pEGFP-N1-LINE1-ORF1的WT_CLS1细胞,其LINE1-ORF1的mRNA及蛋白水平显著增高（P < 0.05）,细胞增殖能力和克隆形成能力都显著增强（P < 0.05）。结论 LINE1-ORF1p可以促进肾母细胞瘤细胞的生长和肿瘤细胞克隆形成,可能参与肾母细胞瘤的发病机制。     

FIZZ1及NOTCH1在哮喘中的作用

目的探讨气道重塑中低氧诱导的有丝分裂因子(FIZZ1)和NOTCH1的表达及罗格列酮的干预作用。方法健康6～8周龄雄性Sprague-Dawley大鼠45只,分为哮喘组、对照组及罗格列酮干预组。制备肺组织石蜡切片进行病理学检查,免疫组化测定各组气道重塑特异性指标a-肌动蛋白(a-SMA)在肺组织中的表达,用RT-PCR方法测定各组肺组织中FIZZ1-mRNA及NOTCH1-mRNA的表达。结果哮喘组出现气道重塑的特征性改变,罗格列酮干预组气道病理学改变较哮喘组减轻。哮喘组a-SMA、FIZZ1-mRNA及NOTCH1-mRNA表达较对照组增高,罗格列酮干预后表达均显著降低,但仍高于对照组。a-SMA蛋白的表达与FIZZ1-mRNA及NOTCH1-mRNA的表达均呈正相关(r分别为0.826和0.9,P<0.01),FIZZ1-mRNA及NOTCH1-mRNA的表达成正相关(r=0.76,P<0.01)。结论 FIZZ1及NOTCH1可促使a-SMA表达增强,是引起哮喘早期气道重塑的重要炎症因子。罗格列酮可缓解气道重塑的过程。     

Alleles of HLA-DQB1 and familial aggregation of type 1 diabetes

The HLA complex, located on the short arm of chromosome 6, is the strongest genetic marker for type 1 diabetes (T1DM). In previous study we demonstrated association between genes HLA-DRB1 and HLA-DQB1 and T1DM in the Polish population. There is a strong-independent association of alleles HLA-DRB1*0401 and DQB1*302, despite population linkage disequilibrium among alleles of these genes. The aim of the current study was to verify a hypothesis that some alleles or haplotypes of HLA-DRB1, DQA1 and DQB1 genes increase the risk for familiar aggregation of T1DM. We analysed 507 patients with IDDM derived from 80 multiplex and 325 patients from simplex families. PCR and hybridisation with SSO probes performed HLA typing for DRB1, DQA1 and DQB1 alleles. Genetic analysis demonstrated strong association of allele HLA-DQB1*0302 with T1DM in the Polish population in families with single (DM1) and more numerous cases (DM2) cases, compared with healthy cases (n=103). The HLA-DQB1*302 allele frequencies were 27.8% vs 8.7%; Pc<10(-5); OR(95%CI)=4,03(3.80-4.25) and 16.3% vs 8.7%; Pc<0.04; OR(95%CI)=2.04(1.79-2.89), respectively. The presence of allele HLA-DQB1*0602 has a strong protective effect from T1DM in both studied groups (1.46% vs. 13.6%; Pc<10(-5); OR(95%CI)=0.09(-0.25-0.44) and 0.98% vs. 13.6%; Pc<10(-5); OR(95%CI)=0.06(-0.46-0.58), respectively. Interestingly, HLA-DRB1*04 allele more often co-segregated with DM2 families as comparing the DM1 group (31.0% vs. 15.8%, respectively; Pc<10(-5)). However in both cases differences remain significant as compared to controls: Pc<10(-5), OR (95%CI)=3.52(3.33-3.70) and Pc<10(-5) OR(95%CI)=6.17(5.97-6.37), for DM1 and DM2 respectively. Subtyping of HLA-DRB1*04 alleles demonstrated that the strongest predisposing effect has been identified with DRB1*0401. Moreover, difference in frequencies of the protective allele HLA-DQB1*0301 among DM1 and DM2 group was revealed (8.8% vs. 13.7%, respectively; Pc<10(-5)) and the protective effect of this allele remained only significant in DM1 group: 8.8% vs. 19.9%; Pc<10(-5); OR(95%CI)=0.39(0.19-0.58). The results suggest that it is likely that familial aggregation of T1DM is associated with lower frequency of protective alleles of HLA-DQB1 gene.     

Clinical profile of H1N1 positive HIV-infected children

We report five cases of HIV infected children, who presented with flu-like symptoms and were diagnosed to have H1N1 infection (swine origin influenza). Four of these children were admitted with respiratory distress and pneumonia and were managed in swine flu isolation ICU. Two children required nonivasive ventilatory support. All children recovered completely and at discharge were referred for initiation of ART.     

心肌营养素-1在大鼠缺血再灌注心肌中的表达及神经调节蛋白-1对其表达的促进作用

目的研究大鼠缺血再灌注心肌中心肌营养素-1(CT-1)的表达及神经调节蛋白-1(NRG-1)对其表达的影响。方法制备大鼠缺血再灌注心肌模型,分为模型组(n=8),NRG-1预处理组(n=9),假手术组(n=8)和正常对照组(n=10)。利用RT-PCR技术检测各组CT-1 mRNA表达,计算CT-1 mRNA相对量,并作统计学处理。结果模型组CT-1 mRNA结果(63.96±9.34)高于假手术组(36.16±5.43)和正常对照组(36.84±4.64),三者比较差异有显著性(F=47.37 P<0.01);NRG-1预处理组(89.49±6.99)高于模型组,差异有显著性(t=6.43 P<0.01)。结论大鼠心肌缺血再灌注后CT-1 mRNA表达升高,其原因可能是缺血再灌注激活机体内源性保护机制,NRG-1预处理对心肌的保护作用可能与提高心肌细胞中CT-1的表达有关。实用儿科临床杂志,2006,21(1):29     

Comparison of clinical presentation of respiratory tract infections in H1N1/09-positive and H1N1/09-negative patients

The true burden of influenza in children is difficult to assess and is probably underestimated as clinical signs are usually nonspecific, and formal viral identification is rarely searched. In this study, we compare the clinical features of infections related to the new H1N1/09 influenza virus with infections due to other respiratory viruses in children consulting in a tertiary care pediatric hospital in Geneva. Between October 1, 2009 and February 10, 2010, 109 patients were recruited, with a median of age of 7 years (range 0.1–18). There were 75 H1N1/09-positive patients (69%), and 32 (43%) had identified risk factors such as asthma or a history of wheezing. Fever (87%), cough (92%), and rhinitis (85%) were the most frequent reported presenting symptoms in both patient groups. H1N1/09-positive patients were significantly older (median of 8.2 vs. 4.6 years) and were more likely to have risk factors (43% vs. 24%) and myalgias (41% vs. 20%). H1N1/09-negative patients had more wheezing episodes (29% vs. 9%), higher rates of dyspnea (28% vs. 20%) and of hospital admissions (35% vs. 16%). Conclusion: Clinical signs cannot reliably differentiate H1N1/09-positive and H1N1/09-negative patients, although we found a higher proportion of myalgias in H1N1/09-positive patients. Severity of disease was lower in H1N1/09-positive than in H1N1/09-negative patients, mostly because of a higher proportion of asthma/wheezing episodes among H1N1/09-negative patients.     

2009 H1N1 Influenza Pandemic

The 2009 H1N1 influenza pandemic took health care workers worldwide by surprise. Early in the course of the pandemic it was determined that children and pregnant women were at high risk of increased morbidity and mortality from the novel influenza virus. The Centers for Disease Control and Prevention and state and local public health officials quickly rallied to develop treatment guidelines for the new strain of influenza A, including emergency approvals for off-label use of some antiviral drugs. Prevention of the spread of influenza via vaccination and environmental controls is critical to the health of children. The 2009 H1N1 influenza virus emerged too late to be included in the 2009/2010 seasonal influenza vaccine, so production of a monovalent vaccine was set in motion. Five months from when the first cases of novel H1N1 appeared in Mexico and the United States, a vaccine was being distributed to high-risk patients. Looking ahead to the 2010/2011 influenza season, it is difficult to predict 2009 H1N1 activity. The 2010/2011 seasonal influenza vaccine will include the 2009 H1N1 strain, so it is critical to get all children vaccinated early in the flu season.     

Upregulation of S1P1 and Rac1 receptors in the pulmonary vasculature of nitrofen-induced congenital diaphragmatic hernia

Purpose

Sphingolipids play a crucial role in pulmonary development. The sphingosine kinase 1 (SphK1) modulates the synthesis of sphingolipid sphingosine-1-phosphate (S1P). S1P regulates cell proliferation and angiogenesis via different receptors, S1P₁, S1P₂ and S1P₃, which all influence the expression of Ras-related C3 botulinum toxin substrate 1 (Rac1). We designed this study to test the hypothesis that the S1P/Rac1 pathway is altered in the nitrofen-induced CDH model.

Methods

Pregnant rats received nitrofen or vehicle on D9. On D21, fetuses were killed and divided into nitrofen and control group (n = 12). QRT-PCR, western blotting and confocal-immunofluorescence microscopy were performed to reveal pulmonary gene and protein expression levels of SphK1, S1P₁, S1P₂, S1P₃ and Rac1.

Results

Pulmonary gene expression of S1P₁ and Rac1 was significantly increased in the CDH group compared to controls, whereas S1P₂ and S1P₃ expression was decreased. These results were confirmed by western blotting and confocal microscopy. SphK1 expression was not found to be altered.

Conclusion

The increased expression of S1P₁ and Rac1 in the pulmonary vasculature of nitrofen-induced CDH lungs suggests that S1P₁ and Rac1 are important mediators of PH in this model.

     

Glycogenoses type 1b and 1c

In type 1 glycogen storage diseases, glucose-6-phosphatase may be present but associated with impaired transport of glucose-6-phosphate (type 1b) or inorganic phosphate (type 1c) through microsomal membranes. The type 1c is very rare (2 published cases). The more frequent type 1b presents all the clinical manifestations of type 1a and specific signs: recurrent stomatitis, frequent infections, chronic inflammatory bowel disease secondary to neutropenia and neutrophil dysfunction. Glucose-6-phosphatase activity is low when measured on fresh liver tissue, but is restored after detergent treatment. A good metabolic control does not influence neutropenia and its consequences.