血清补体活性和C反应蛋白水平及血小板计数与全身炎症反应综合征的预后

目的　探讨全身炎症反应综合征 (SIRS)患儿血清补体 (C3 、C4 )活性、C反应蛋白 (CRP)水平及血小板计数变化的临床意义。方法　采用前瞻性研究方法 ,用全自动免疫比浊定量分析法和全自动血细胞计数仪测定 6 8例SIRS患儿血清C3 、C4 、CRP水平和血小板计数及其动态变化。　结果SIRS患儿首次测定的血清C3 [(4 0 6± 1 0 9)g/L]、C4 [(1 6 4± 0 89)g/L]、CRP[(5 7± 2 0 )g/L]及血小板计数 [(6 10± 140 )× 10 9/L]均显著高于非SIRS患儿组和正常对照组 (F分别为 2 77 4、10 2 0、2 2 7 0及196 2 ,P <0 0 5或 <0 0 1) ,但非SIRS患儿组和正常对照组四组参数比较差异均无显著意义 (P >0 0 5 )。动态观察发现 :SIRS组病情好转后四项参数均较好转前低 (C3 、C4 、CRP及血小板成对比较的t值分别为 14 9、8 9、14 7及 18 0 ,P <0 0 1) ;而恶化组CRP持续升高、C3 、C4 和血小板计数持续下降 ,明显不同于好转前 (C3 、C4 、CRP及血小板计数的成对比较t值分别为 8 0、4 7、2 4及 11 4,P <0 0 5或 <0 0 1)。而且病情恶化组SIRS患儿病情变化后C3 、C4 和血小板水平均低于好转组 [C3(1 5 4± 0 6 8)g/L与 (0 86± 0 6 7)g/L ,P <0 0 5 ;C4 (0 45± 0 2 3)g/L与 (0 2 5± 0 0 9)g/L ,P <0 0 5 ;血     

快速定量检测C反应蛋白在儿科的应用

目的：探讨快速定量检测C反应蛋白在儿科疾病中的应用价值。方法：采集90例不同疾病患儿末稍血20 μl,用快速CRP分析仪进行测定。结果：15例细菌性感染疾病中相应培养阳性例数为2例,阳性率13.3%,而CRP升高9例,占60%;16例非细菌感染疾病相应培养均阴性,CRP均<10 mg/L;59例病原不确定疾病中相应细菌培养阳性例数为5例,阳性率为8.5%,而CRP升高9例,占15%。细菌感染疾病与非细菌感染疾病及病原不确定疾病比较,CRP升高比例大,P<0.01,差异有显著性意义。结论：快速定量检测CRP在区别细菌和非细菌感染,判断病情变化及治疗效果方面具有一定价值。     

湿疹血小板减少伴免疫缺陷综合征1例

患儿，男，1岁，因反复皮疹11个月、双下肢皮肤坏疽3d入院。入院前11个月患儿无明确诱因反复出现皮疹，首次入本院。查体：头颈、躯干见弥散性分布的斑丘疹，糜烂、渗血，皮肤小疖肿。血WBC 38．4×10^9／L，RBC 4．3×10^12／L，Hb 120g／L．PLT 100×10^9／L，ESR 36mm／1h，C反应蛋白（CRP）76mg/L。脓液及皮肤创面分泌物培养均为金黄色葡萄球菌。     

热性惊厥患儿外周血白细胞计数的变化及其意义

目的 探讨热性惊厥后白细胞计数(white blood cell,WBC)的变化及其意义.方法 回顾性分析我院2010年4月至201 1年10月收治的热性惊厥104例及同期住院发热无惊厥95例患儿的资料,同步监测WBC及C反应蛋白(C reactive protein,CRP)的变化情况并进行统计学分析.结果 (1)惊厥组中29例CRP增高者,WBC值为(14.0±4.8)×109/L,与非惊厥组中50例CRP增高者的WBC值[(10.9±4.3)×109/L]相比,差异有统计学意义;惊厥组中75例CRP正常者,WBC值为(12.6 ±4.8)×109/L,与非惊厥组中45例CRP正常者的WBC值[(7.4±3.3)×109/L]相比,差异有统计学意义.(2)惊厥组中75例CRP正常者,其中WBC增高46例,占61.3％,而非惊厥组中45例CRP正常者,WBC增高9例,仅占20％,差异有统计学意义(P＜0.05).结论 在同样感染因素影响的前提下,惊厥患儿WBC多高于非惊厥患儿.惊厥患儿外周血WBC与CRP可能不平行.     

全身炎症反应综合征患儿血清C-反应蛋白测定的意义

目的　 了解全身炎症反应综合征 (SIRS)患儿在不同阶段血清C 反应蛋白 (CRP)测定的意义。 方法 　随机选择 6 0例符合SIRS诊断的患儿 ,分别于急性期和恢复期行血清CRP测定。同时选择同期住院无SIRS的非危重病儿 30例作为对照组。观察CRP水平的改变与病情严重程度的关系及对MODS发生率的影响。 结果 　SIRS患儿血清CRP水平在急性期显著升高(92 8mg/L) ,急性期与对照组 (15 1mg/L)比较有显著差异 (P <0 0 1)。SIRS患儿急性期A、B二组MODS的发生率分别为10 5 %、40 9%。 结论 　CRP在SIRS的发生、发展中起重要作用 ,在SIRS急性期CRP显著增高者提示病情危重。对预测MODS的发生 ,早期防治MODS提供理论依据     

N末端脑利钠肽原在川崎病患儿中的变化及其意义

目的探讨N末端脑利钠肽原(N-terminalpro-brainnatriureticpeptide,NT-proBNP)在川崎病(Kawasakidisease,KD)患儿中的变化及其临床意义。方法分别测定13例确诊为KD患儿急性期的血浆NT-proBNP水平,并测定了其中的8例恢复期患儿血浆NT-proBNP水平,同时以9例小儿上呼吸道感染急性期为对照组的血浆NT-proBNP水平。分别测定KD患儿在急性期及恢复期的外周血白细胞计数(whitebloodcell,WBC)、C反应蛋白(C-reactiveprotein,CRP)及血浆心肌肌钙蛋白(cardiactroponinI,cTnI)水平,常规超声心动图检测左心室舒张末期内径(leftventricularenddiastolicdiameter,LVDd)、射血分数(leftventricularejectionfraction,LVEF)、舒张期二尖瓣血流频谱速度及冠状动脉病变情况。结果KD患儿急性期血浆NT-proBNP水平为(691±86)ng/L,而对照组小儿血浆中NT-proBNP水平为(47±10)ng/L,两组相比差异具有统计学意义(P<0·001)。有8例KD患儿同时测定了急性期及恢复期血浆NT-proBNP水平。急性期患儿血浆NT-proBNP水平为(636±89)ng/L,在恢复期血浆NT-proBNP水平显著降低,为(164±35)ng/L(P<0·01)。通过直线回归分析,KD患儿急性期血浆NT-proBNP水平与左心室EF、LVDd均无相关关系,同时与患儿舒张期二尖瓣血流频谱E峰及A峰比值也无相关关系。但是,KD患儿急性期血浆NT-proBNP水平与患儿急性期CRP、WBC呈显著正相关(r分别为0·615及0·547,均P<0·05),同时还与急性期患儿的心肌肌钙蛋白水平呈显著正相关(r=0·611,P<0·05)。结论血浆NT-proBNP水平在KD急性期显著升高,而在疾病恢复期其水平明显降低。KD患儿急性期血浆NT-proBNP水平的升高可能与急性期KD患儿的心肌炎症及炎性因子的升高有关。     

窦组织细胞增生伴巨大淋巴结病一例

患儿男,4岁,回族。因发热伴颌下包块1个月于2005年9月5日入院。入院前曾给予多种抗生素治疗无效,高热不退,体温39～41%。在外院曾对颌下包块进行活检,未明确诊断。入院查体:腋温40℃,脉搏110次/min,一般情况尚好。双侧颌下均触及多个包块,左侧最大者7cm×6cm×5cm,右侧最大者6cm×6cm×5cm,表面光滑,质韧,触痛,无粘连。腋下可扪及数个花生米大小的淋巴结,活动欠佳,无粘连。双肺呼吸音粗糙;心脏无异常;肝肋下3.5cm,质韧,无触痛;脾肋下3cm,腹软,未触及包块。血常规:WBC 32×10~9/L,N 28.7×10~9,Hb 103×10~9/L,余正常。ESR58 mm/h,CRP 43μg/ml,补体 C_3 2050 mg/L(正常值90～1800 mg/L),C_4 429 mg/L(正常值100～400 mg/L),CD_345.95%(正常值50.5%～68.6%),CD_4 24.72%(正常值35.1%～44.7%),CD_8及 CD_4/CD_8正常,IgG、IgA、IgM 正常。TORCH 抗体[即一组先天性或围产期的非细菌性感染     

儿童噬血细胞综合征合并重症中枢神经系统病变1例救治经验

患儿，男，13岁，因反复发热11 d入院。患儿于11 d前无明显诱因出现发热，弛张高热，热峰40℃，每日2~3次，伴畏寒、寒颤，咽痛，轻咳，服退热药物体温降至正常2~3 h后又反复。到当地医院住院治疗，血常规检查示白细胞计数（WBC）26×10^9/L，中性粒细胞百分比（N%）87.9%，淋巴细胞百分比（L%）8.7%，血红蛋白（Hb）132 g/L，血小板（PLT）205×10⁹/L；C反应蛋白（CRP）111.46 mg/L，降钙素原（PCT）1.29 ng/mL，肺炎支原体抗体1：160。予头孢哌酮舒巴坦、阿奇霉素、奥司他韦等抗感染治疗，复查血常规示：WBC 10×10⁹/L，N% 83.24%，L% 8.7%，Hb 124 g/L，PLT 241×10⁹/L；CRP 39.25 mg/L，PCT 3.03 ng/mL。发热无好转，来我院急诊，予头孢曲松静脉滴注10 min后出现颜面部多发红色皮疹伴瘙痒，停止头孢曲松输注并口服西替利嗪后皮疹消退，以"发热查因"收住院。     

高频通气在新生儿疾病中的应用

1　病案摘要女 ,G3 P3 ,孕 35w+ 1。因母亲化脓性阑尾炎术后宫缩增加 ,行剖宫产娩出 ,出生体重 3kg,Apgar评分 8～ 9分。生后不久即出现气促 ,呼吸 80～ 90次 /min ,并逐渐出现吸凹 ,青紫 ,呻吟不止 ,于生后 4 5h收入院。入院T 37℃ ,神志清 ,反应差 ,早产儿貌 ,前囟平软 ,皮肤青紫 ,呻吟不止。两肺呼吸音粗 ,呼吸 6 0次 /min ,三凹症 (+) ,心率 138次/min ,律齐 ,心音有力 ,全腹平软 ,四肢活动可 ,肢端冷。辅助检查 :血常规Hb2 12 g/L,RBC 5 1× 10 12 /L ,WBC 18 5× 10 9/L ,N 0 73,BPC 375× 10 9/L ,CRP <8mg/L。血气分析 …     

风疹并血小板减小性紫癜及颅内出血1例

患儿,女,2岁,发烧、头面及周身皮疹2天,全身出血点6h入院。查体:T36.5℃,R25次/min,P102次/min,额面部、周身皮肤散在的淡红色斑丘疹并有针尖大小的鲜红色的出血点、淤点及淤斑。抽搐,颈抵抗,巴氏征(+),心肺无异常。WBC8.0×10~9/L,RBC3.8×10~(12)/L,Hb11.0g/L,血小板45×10~9/L,BT5.5min,脑脊液:潘氏反应(+),细胞总数500×10~6/L,有核细胞10×10~6/L,分类:单核细胞0.98,多核细胞     

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.