血液净化治疗儿童重症系统性红斑狼疮多中心流行病学调查

目的了解我国目前血液净化治疗儿童重症系统性红斑狼疮(SLE)现状。方法中国医师协会儿科医师分会血液净化专家委员会讨论并制定调查表,以问卷调查的形式,收集2012年1月至2017年12月在参与研究的22家单位住院行血液净化治疗的127例重症SLE患儿病例资料,并进行统计学分析。结果共收集数据127例,其中男28例、女99例,年龄4~16岁,63例患儿应用血浆置换(PE)治疗180次,41例患儿应用DNA免疫吸附(DNA-IAS)治疗106次,11例患儿应用血液透析(HD)治疗112次,12例患儿应用血液灌流(HP)治疗32次。PE和DNA-IAS治疗后SLE疾病活动指数(SLEDAI)、ANA滴度、抗ds-DNA抗体、免疫球蛋白明显下降,补体回升;HD用于肾功能不全患儿治疗效果显著;HP能够清除炎症因子,使患儿临床症状缓解。PE、DNA-IAS、HD、HP的好转率分别为87.30%、87.80%、72.73%、75.00%。结论 PE和DNA-IAS疗法可迅速清除重症SLE患儿血液中的免疫性物质,缓解病情;HD治疗主要用于重度水肿及肾功能不全患儿;HP治疗能够有效清除炎症因子,缓解临床症状;对于重症SLE患儿,应根据病情的不同选择各异的血液净化疗法。     

儿童狼疮中枢神经系统的急症表现

目的：探讨儿童系统性红斑狼疮中枢审经系统急症的特征,方法：将狼疮脑病患儿与继发中枢神经系统损害狼疮患儿的ANA,dsDNA滴度,Sm阳性率及补体C3下降的阳性率进行比较,并对两组患儿的临床特征进行分析,结果：ANA、ds-DNA滴度及Sm阳性率,补体C3下降阳性率与狼疮脑病并不相关,脑电图有助于狼疮脑病的诊断。结论：儿童红斑狼疮中枢急症原发及继发性损害的鉴别诊断需结合临床及有关辅助检查综合分析。     

血浆置换与免疫吸附治疗儿童重症系统性红斑狼疮对照疗效分析

目的 比较血浆置换与免疫吸附治疗儿童重症系统性红斑狼疮的疗效,寻找治疗重症系统性红斑狼疮患儿较为优势的方法.方法 收集2007年3月至2013年3月中国医科大学附属盛京医院小儿肾脏风湿科收治的27例重症系统性红斑狼疮患儿,采用血浆置换治疗11例,采用免疫吸附治疗16例.监测患儿临床表现、系统性红斑狼疮疾病活动指数（systemic lupus erythematosus disease activity index,SLEDAI）、ANA滴度、免疫球蛋白IgG、血清离子及治疗费用、住院时间及不良反应,并进行组间比较.结果 两组患儿的年龄、性别、病程、治疗前SLEDAI评分均具有可比性.（1）血浆置换组11例患儿共血浆置换治疗26次,治疗后SLEDAI评分明显下降（19.00±3.77 vs 5.34±4.35）,ANA滴度明显下降（2 439.58±1 430.56 vs 303.54±169.32）,血清IgG水平明显下降[（8.35 ±5.67） g/L vs（4.04±2.23） g/L],差异均有统计学意义（P均＜0.05）.（2）免疫吸附组16例患儿共治疗44次,治疗后SLEDAI评分明显下降（18.25 ±4.62 vs4.25 ±2.23）,ANA滴度明显下降（2 560.39±1 563.78 vs 289.62±137.62）,血清lgG水平明显下降[（9.98±6.03） g/Lvs （3.23±1.37） g/L],差异均有统计学意义（P均＜0.05）.（3）经血浆置换与免疫吸附治疗后,两组患儿SLEDAI评分、ANA滴度、IgG水平下降的程度差异无统计学意义.（4）血浆置换及免疫吸附治疗前后血清钾、钠、氯、钙水平均无显著差异（P＞0.05）.（5）血浆置换组住院费用较免疫吸附组明显增加[（62881.6±8826.58）元vs （45 662.71±16 250.88）元],差异有统计学意义（P＜0.05）.（6）血浆置换组住院时间与免疫吸附组比较差异无统计学意义[（33.6±8.60）d vs （31.9±14.6）d]（P＞0.05）.结论 血浆置换与免疫吸附均为有效治疗重症系统性红斑狼疮的方法,但血浆置换费用较高,对降压药物血药浓度影响较大.     

儿童狼疮中枢神经系统的急症表现

目的　 探讨儿童系统性红斑狼疮中枢神经系统急症的特征。 方法 　将狼疮脑病患儿与继发中枢神经系统损害狼疮患儿的ANA、dsDNA滴度、Sm阳性率及补体C3下降的阳性率进行比较 ,并对两组患儿的临床特征进行分析。 结果 　ANA、ds DNA滴度及Sm阳性率、补体C3下降阳性率与狼疮脑病并不相关 ,脑电图有助于狼疮脑病的诊断。 结论 　儿童红斑狼疮中枢急症原发及继发性损害的鉴别诊断需结合临床及有关辅助检查综合分析。     

自身抗体检测在儿童自身免疫性疾病中的意义

目的 研究抗核抗体（ANA）、ANA荧光分型、抗可溶性抗原（ENA）抗体和抗双链DNA（ds-DNA）抗体检测在儿童自身免疫性疾病中的意义。方法 对住院患儿中ANA、抗ENA，或抗ds-DNA抗体至少1项阳性者共135例进行总结，分别计算其阳性预测值（PV）。结果 ANA阳性中自身免疫性疾病的PV=0．36，ANA荧光强度与PV成正比，ANA荧光分型中细颗粒型对于SLE有较高的PV；抗ENA抗体和抗ds-DNA抗体对自身免疫性疾病PV高于ANA。结论 ANA荧光强度，抗ENA抗体和抗ds-DNA抗体阳性对自身免疫性疾病有较高的诊断价值。     

心力衰竭患儿血清β3受体自身抗体水平对离体成鼠心肌细胞的负性变力作用

目的 探讨心力衰竭患儿血清抗β3肾上腺素受体(β3AR)自身抗体水平对离体成年大鼠心肌细胞收缩效应的影响.方法 采用ELISA筛查心力衰竭和健康儿童血清抗β3AR自身抗体,利用亲和层析法提纯抗β3AR自身抗体阳性血清免疫球蛋白G(IgG);利用动缘探测系统观察提纯的抗β3AR自身抗体阳性IgG对离体成年大鼠心肌细胞收缩效应的影响.结果 50例心力衰竭患儿血清抗β3AR自身抗体的阳性率和平均几何滴度为分别30%(15/50例)和1:(48.32±2.58),明显高于健康儿童[12%(6/50例),P＜0.05;1:(13.72±1.49),P＜0.001];与对照组比较,该抗体可降低成年大鼠心肌细胞最大收缩幅度/初长度(3.86±0.31)%、最大收缩速率[(-0.47±0.07) μm/s]和最大舒张速率[(0.17±0.02) μm/s](Pa＜0.001).该作用不能被纳多洛尔阻断,但可被布拉洛尔或β3AR细胞外第二环合成抗原阻断.结论 心力衰竭患儿血清抗β3AR细胞外第二环自身抗体的阳性率和平均几何滴度均明显高于健康儿童,且该自身抗体具有类似β3AR激动剂样负性变力效应.     

严重脱发和骨髓抑制:是系统性红斑狼疮加重吗？

1 病历摘要患儿,女,15岁。首次住院情况:因反复发热、脱发、皮疹和关节疼痛于2003年10月住院。实验室检查:外周血 WBC减少,伴明显淋巴细胞减少;尿沉渣镜下血尿和轻微蛋白尿;血清抗核抗体(ANA)、抗双链DNA(ds-DNA)抗体阳性,抗核糖体RNP(rRNP)抗体弱阳性,补体(C)3和C 4 明显减低。肾组织活检病理:系膜细胞及基质中度弥漫性增生,节段性加重,伴内皮细胞局灶节段性增生,内皮下及     

大剂量静脉注射免疫球蛋白辅助治疗狼疮肾炎的疗效观察

目的　观察大剂量静脉注射免疫球蛋白(IVIG)治疗狼疮肾炎(LN)的疗效。方法　在激素联合环磷酰胺的基础上对观察组29例LN患儿加用IVIG治疗，并与对照组33例LN患儿比较。结果　观察组在降低LN疾病活动性指数计分、血尿及蛋白尿消失、抗核抗体(ANA)及抗ds-DNA阴转率和降低院内感染率方面明显优于对照组，未见明显副作用。结论　IVIG配合激素及环磷酰胺治疗LN是一种有效、安全的方法。     

白介素-13与γ-干扰素在急性毛细支气管炎患儿血清中的表达及其临床意义

目的探讨白介素-13(IL-13)、γ-干扰素(IFN-γ)在急性期毛细支气管炎患儿血清中的表达及与病情轻重之间的相关性。方法用酶联免疫吸附(ELISA)法测定2005-02—2005-11于遵义医学院附属医院治疗的42例急性期毛细支气管炎患儿(其中轻症组22例,重症组20例)和16名健康婴儿的血清IL-13、IFN-γ的质量浓度。结果急性期毛细支气管炎患儿血清IL-13的质量浓度[(6.88±2.12)ng/L]明显高于对照组[(5.48±1.28)ng/L,P<0.01]。急性期毛细支气管炎患儿IFN-γ质量浓度[(10.71±2.44)ng/L]明显高于对照组[(9.20±1.54)ng/L,P<0.05];其中轻症组明显高于重症组和对照组(P<0.05),而重症组与对照组差异则无显著性(P>0.05)。结论急性期毛细支气管炎患儿血清IL-13质量浓度明显增高,提示IL-13参与了毛细支气管炎的发病过程,但所测质量浓度不能反映病情严重程度;IFN-γ在轻症毛细支气管炎组质量浓度明显增高,而重症组不增高,这可能与急性重症毛细支气管炎患儿IFN-γ产生受抑制有关。     

急性毛细支气管炎患儿血清白细胞介素13、γ-干扰素表达的意义

目的 探讨急性毛细支气管炎患儿血清IL-13、IFN-γ表达及其临床意义.方法 用酶联免疫吸附法(ELISA)检测42例急性期毛细支气管炎患儿(其中轻症组22例,重症组20例)和20名健康婴儿血清IL-13、IFN-γ水平.采用方差分析和成组t检验,检测各组间差异.结果 1.急性毛细支气管炎患儿血清IL-13[(6.88±2.12 )ng/L]明显高于对照组[(5.48±1.28 )ng/L](P＜0.05).2.急性期毛细支气管炎患儿IFN-γ[(10.71±2.44 )ng/L]明显高于对照组[(9.20±1.54)ng/L](P＜0.05);轻症组明显高于重症和对照组(P＜0.05),而重症与对照组则无显著性差异(P＞0.05).结论 1.IL-13参与毛细支气管炎的发病过程,但其水平不能反映病情严重程度;2.IFN-γ水平在轻症毛细支气管炎组明显增高,而重症组不增高,可能与急性重症毛细支气管炎患儿IFN-γ产生受抑制有关.     

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.