间歇白细胞去除法治疗儿童风湿性疾病疗效分析

目的探讨间歇白细胞去除法对系统性红斑狼疮等风湿性疾病的治疗效果.方法 20例患儿(其中系统性红斑狼疮8例、过敏性紫癜肾炎5例、幼年型类风湿关节炎3例、混合性结缔组织病2例,皮肌炎、硬皮病各1例)在常规治疗疗效不明显的情况下,加用间歇性白细胞去除法治疗,每次100～200mL,每周1～2次,病情稳定后改为 1～2个月1次.结果 20例中18例治疗后临床症状和体征均逐步明显好转, 各项实验室指标同步改善,保证了激素的安全减量.间歇白细胞去除法前后血象改变表明,除血小板有轻度降低外,白细胞、红细胞、血红蛋白均无明显改变.结论间歇白细胞去除法治疗儿童风湿性疾病,有良好的效果.     

雷公藤总苷加川芎嗪治疗紫癜性肾炎疗效分析

目的研究雷公藤总苷加川芎嗪治疗儿童紫癜性肾炎的疗效。方法紫癜性肾炎33例,随机分为治疗组17例、对照组16例,两组均口服雷公藤总苷1 mg/(kg.d),治疗组加用川芎嗪2~4 mg/(kg.d)静滴,2~3周。结果治疗组临床表现、血尿、蛋白尿、血凝恢复时间均明显短于对照组。结论雷公藤总苷加川芎嗪治疗紫癜性肾炎疗效优于单用雷公藤总苷。     

环磷酰胺冲击治疗紫癜性肾炎疗效观察

目的　探讨环磷酰胺 (CTX)冲击疗法治疗重症紫癜性肾炎 (HSPN)疗效。方法　选择 40例重症紫癜性肾炎患儿为对照组和观察组 ,观察组予CTX 8～ 1 0mg/ (kg·次 ) ,加入生理盐水 1 0 0mL中静滴 ,2d为 1个疗程 ,间隔 30d ,共用 4个疗程。对照组常规采用泼尼松 1 .5～ 2 .0mg/ (kg·d) ,双嘧达莫 1～ 2 g/ (kg·d)治疗。 结果　冲击治疗后 2 4h尿蛋白定量和血尿明显降低 ,冲击治疗前后有显著差异 (P <0 .0 1 )。结论　采用小剂量、长间隔CTX冲击方法治疗重症HSPN疗效好 ,且不良反应少。     

地塞米松冲击治疗过敏性紫癜34例

我科1985年以来用地塞米松冲击治疗过敏性紫癜34例,其中紫癜型肾炎12例,收到较好疗效。本文观察组34例,对照组36例,均为住院患者。2组年龄、性别相仿,年龄5岁～12岁。观察组合并紫癜型肾炎12例,对照组11例。观察组用维生素C及钙剂、地塞米松1mg～2mg/(kg·d)静点,3d后改隔日1次,9d为1疗程。对照组VitC 2g,钙剂1g,1次/d静点,10d 1疗程。诊断标准按实用儿科学标准。     

小儿新月体肾炎临床研究

目的了解小儿新月体肾炎的临床特点及诊断和治疗。方法对我科近3年来经肾脏病理确诊的16例新月体肾炎患儿发病年龄、病因、临床特点,实验室检查特点、肾脏病理改变、治疗和预后进行分析。结果小儿新月体肾炎以>10岁的儿童多见,最常见的原因为IgA肾病,其次为紫癜性肾炎和狼疮性肾炎,以急进性肾炎表现的较少。本组患儿均有肉眼血尿,大量蛋白尿、血清肌酐清除率(Ccr)逐渐下降、血尿素氮(BUN)逐渐上升的发生率分别为87.5%、75%、62.5%,病理有广泛的细胞性及细胞纤维性新月体形成,治疗主要是激素加免疫抑制剂,联合抗凝,抗血小板治疗及对症治疗。结论(1)小儿新月体肾炎以IgA肾病为最常见的病因,对肉眼血尿并大量蛋白尿持续存在的患儿建议早期肾病理检查,早期诊断,及时治疗,坚持随访尤为重要;(2)治疗采用激素加用免疫抑制剂,先强化后维持的治疗方案。     

静脉注射丙种球蛋白治疗早产儿重症感染50例

目的探讨静脉注射丙种球蛋白(IVIG)治疗早产儿重症感染的疗效。方法采用免疫透射比浊法检测50例重症感染出生1~5 d(胎龄28~36周)早产儿血清IgG、IgA、IgM。随机分为IVIG治疗组及对照组,各25例。均选用同类抗生素,IVIG组给予IVIG 1.0 g/(kg.d)在2~3 h内静脉滴注,连用2 d。结果早产儿50例血清IgG均值随胎龄增加而升高(r=0.99 P<0.01)。IVIG组治疗1周后IgG均值从8.2 g/L升至13.5 g/L(P<0.01);而对照组治疗后IgG均值为7.7 g/L。结论早产儿呈现生理性低丙种球蛋白血症,抗体缺乏是早产儿易发生感染的重要原因,早产儿重症感染用IVIG疗效最佳。     

肾病综合征患儿血清α1—抗胰蛋白酶的变化及其临床意义

本文对40例肾病(NS)患儿(单纯性32例,肾炎性8例)在发病极期及恢复期血清α_1抗胰蛋白酶(α_1-AT)变化特点及临床意义进行探讨,结果40例患儿在发病极期(单纯性NS为1.84±0.63g/L,肾炎性NS为1.80±0.72g/L)与对照组(3.13±0.9g/L)比较,前者血清α_1-AT值显著降低(P<0.01),但两型之间无明显差异(P>0.05);在恢复期与对照组比较,两组无明显差异(P>0.1)。动态监测血清α_1-AT值的变化,对判断病情及指导治疗方面具有实际意义,当α_1-AT值显著降低时应给予正规激素等药物积极治疗,如持续减低,提示对激素耐药可考虑加用其它免疫抑制剂。     

小剂量肝素防治过敏性紫癜性肾炎肾损害的临床研究

目的观察小剂量肝素防治过敏性紫癜性肾炎肾损害的有效性。方法采用随机方法将61例过敏性紫癜(HSP)患儿分为肝素治疗组(35例),对照组(26例)。2组患儿均给予一般综合治疗包括脱敏(钙剂、扑尔敏)、改善血管脆性(复方芦丁、维生素C)、H2受体阻滞剂甲氰咪呱静脉输注等对症治疗直至临床症状消失。肝素组在以上综合治疗基础上加用小剂量肝素钠按80~100U/(kg·次) 10%葡萄糖50~100mL静脉输注,1次/d,连续7d或小剂量肝素钙按10U/(kg·次),皮下注射,2次/d,连续7d。动态观察尿常规变化,治疗开始前、治疗结束时肝素治疗组患儿血凝4项检测并在开始治疗前、开始治疗后1个月、3个月、6个月对尿常规正常者给予β2微球蛋白(β2-MG)、尿微量白蛋白(AIb)检测。结果随访6个月时肝素治疗组较对照组肾损害发生率明显降低,统计学显示(P<0.01)有显著性差异。尿常规始终正常者有28例存在尿β2-MG和或尿AIb异常。结论肝素可有效预防或降低过敏性紫癜性肾炎肾损害的发生率,尿β2-MG和尿AIb可作为HSP早期肾损害的敏感指标。     

糖皮质激素在儿童危重症手足口病中的应用

目的分析2011年本院收治的重症手足口病(HFMD)患者的临床特点、治疗情况,观察应用糖皮质激素的效果。方法将56例重症HFMD患儿随机双盲分为激素治疗组与非激素治疗组,2组病例均采用抗病毒、对症综合治疗,激素治疗组在此治疗基础上加用激素。结果 2组痊愈出院49例,遗留肢体活动障碍3例。其中激素治疗组1例,非激素治疗组2例;激素治疗组死亡1例。2组疗效比较无明显差异。结论重症HFMD患者应用糖皮质激素冲击疗法的效果并不显著。     

多靶点免疫抑制剂治疗儿童激素耐药型肾病综合征前瞻性研究

目的探讨多靶点免疫抑制剂治疗儿童激素耐药型肾病综合征(SRNS)疗效及安全性。方法选择2013年9月至2015年10月安徽省儿童医院等5家医疗机构的SRNS患儿48例,在综合治疗基础上随机分为两组:(1)观察组:给予环孢素(CSA)[3~4 mg/(kg·d)]联合霉酚酸酯(MMF)[20 mg/(kg·d)]多靶点治疗;(2)对照组:给予CSA[4~6 mg/(kg·d)]。治疗期间严密监测各组不良反应,分别于治疗后2周、1个月、3个月、6个月测定患儿CSA浓度、尿蛋白/尿肌酐、肝肾功能、血常规、尿常规等。结果治疗期间观察组CSA平均血药浓度为(88.76±16.94)μg/L,对照组为(152.96±19.20)μg/L(P0.001)。治疗1、3个月,观察组尿蛋白/尿肌酐与对照组同时间尿蛋白/尿肌酐相比下降(P0.05)。治疗1、3个月,观察组血浆白蛋白与对照组同时间血浆白蛋白相比升高(P0.05)。治疗期间两组尿β2-微球蛋白相比差异无统计学意义(P0.05)。观察组总缓解率为88%,对照组总缓解率为87%。观察组在治疗2周、1个月时缓解率优于对照组(P0.05)。治疗期间主要的副反应有感染、胃肠道反应、多毛、高血压、白细胞下降。观察组在治疗期间不良反应发生率明显低于对照组(P0.05)。结论 CSA联合MMF可以使SRNS患儿早期即可得到缓解,也可与单用CSA一样维持长期缓解,但副反应更小。     

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.