儿童紫癜性肾炎临床分型 病理分级及预后相关性分析

目的探讨儿童紫癜性肾炎(HSPN)临床、病理与预后关系。方法研究对象为2008年5月至2013年4月收住于中国医科大学附属第一医院HSPN患儿40例,对其临床、病理及预后等资料进行统计学分析。结果 40例病理分级中I级14例(35.0%)、Ⅱ级13例(32.5%)、Ⅲa级6例(15.0%)、Ⅲb级3例(7.5%)、Ⅴ级1例(2.5%)、Ⅵ级3例(7.5%)。其中临床分型血尿和蛋白尿型最多,为25例(62.5%),临床分型越重,病理分级亦越重(P0.05)。皮疹出现次数与病理分级有相关性(χ2=5.1673,P0.05)。单纯Ig A沉积型22例(55.0%)、Ig A+Ig G型8例(20.0%)、Ig A+Ig M型5例(12.50%),Ig A+Ig G+Ig M型5例(12.50%)。HSPN肾小管间质病变(+)级32例,(++)级4例,(+++)级3例,(++++)级1例,肾小管间质病变与病理分级显著相关(r=0.50119,P0.05)。随访临床治愈30例(75.0%)、轻微尿异常8例(20.0%)、活动性肾脏病2例(5.0%),无肾功能不全患儿。免疫复合物沉积类型与病理分级分布差异无统计学意义(χ2=0.8225,P0.05)。HSPN患儿病理分级、肾小管间质病变程度和预后均为正相关(r=0.55826、0.56697,P0.05)。结论儿童HSPN皮疹反复发作多次,应警惕肾脏损伤。病理分级、小管-间质损害程度与预后正相关,尽早肾活检对了解肾脏病变情况十分必要。     

IgA肾病患儿血管紧张素Ⅱ变化的临床意义

目的探讨血管紧张素Ⅱ(AngⅡ)在IgA肾病(IgAN)中的临床意义。方法选择2008年5月至2012年12月间经肾活检诊断为原发性IgAN的30例患儿(IgAN组)及同期健康体检的30例儿童(对照组);以ELISA法检测并比较IgAN组与对照组的血和尿AngⅡ水平;以免疫组织化学法检测IgAN组肾组织中AngⅡ表达,分析IgAN组肾组织中的AngⅡ与各临床指标间的相关性。结果 IgAN组的尿AngⅡ水平高于对照组,差异有统计学意义(P0.05)。IgAN组的尿AngⅡ水平与尿微量白蛋白水平呈正相关(r=0.37,P=0.046);不同病理分级IgAN患儿的尿及肾组织AngⅡ表达差异均有统计学意义(P0.05)。IgAN患儿的病理分级与尿AngⅡ水平及肾组织AngⅡ表达呈显著正相关(r=0.69,0.79,P均=0.000)。结论 IgAN患儿尿AngⅡ水平升高,并与病理分级和尿微量白蛋白水平正相关,检测尿AngⅡ可能对评定IgAN疾病进展及判断预后有实用价值。     

IgA肾病患者肾组织单核细胞趋化蛋白-1表达的意义

目的 探讨原发性IgA肾病(IgAN)患者肾组织单核细胞趋化蛋白-1(MCP-1)的表达变化.方法 选择经皮肾组织穿刺活检确诊为IgAN的患者40例.根据肾脏病理Lee氏分级(Ⅰ～Ⅴ级)将纳入研究的患者分为2组:A组20例,病理分级为Ⅰ～Ⅲ级;B组20例,病理分级为Ⅳ～Ⅴ级.对照组20例标本选取手术切除的肾肿瘤、肾囊肿患者远离病变组织的正常肾组织.同时将肾组织的肾小管和肾间质按照Katafuchi标准分为无间质病变组21例,轻度间质病变组8例,中度间质病变组19例和重度间质病变组12例.均采用免疫组织化学方法测定其肾组织中MCP-1的表达(以灰度值反映),观察其肾组织切片的染色强度及染色透光度,灰度值大则MCP-1表达少,反之则表达多.结果 根据肾脏病理Lee分级分组,各组灰度值比较:B组灰度值(68.08±2.37)与A组灰度值(74.50±3.27)比较、B组与对照组灰度值(81.98±3.21)比较、A 组与对照组比较,差异均有统计学意义(Pa<0.01);根据Katafuchi标准分组,无间质病变组、轻度间质病变组、中度间质病变组及重度间质病变组灰度值分别为82.03±3.13、76.44±2.01、71.49±1.69、66.54±1.23,各组比较差异均有统计学意义(Pa<0.01).结论 MCP-1可反映原发性IgAN患者肾组织的病理损害程度,且表达水平与肾组织损害程度有关.     

TGF-β1在儿童原发性IgA肾病肾损害中的临床意义

目的 探讨转化生长因子-β1（TGF-βl）在儿童原发性IgA 肾病（IgAN）患儿肾损害中的临床意义。方法 选择2008 年5 月至2012 年10 月间经肾活检诊断为原发性IgAN 的30 例儿童为研究对象,另30例健康儿童作为对照组。采用酶联免疫吸附实验（ELISA）法检测其血和尿中TGF-β1 的浓度,免疫组织化学法检查肾组织中 TGF-β1 蛋白的表达,并进行血、尿和肾组织中的 TGF-β1 三者之间及其与各临床指标间的相关性分析。结果 原发性 IgAN 患儿血、尿的 TGF-β1 均高于对照组,差异有统计学意义（Pr=0.557,P=0.001）;肾脏组织中 TGF-β1 的表达与肾组织病理分级密切相关,且随着其病理分级的增加而增加（r=0.682,Pr=0.038, P=0.844）。结论 IgAN 患儿尿TGF-β1 的值与IgAN 病理分级的严重程度相关,临床检测尿TGF-β1 可能对评定慢性肾病进展及判断预后有重要的实用价值。     

儿童IgA肾病临床与病理的关系

目的探讨儿童IgA肾病(IgAN)的临床特点及其与病理的关系。方法对肾活检确诊为IgAN 21例进行临床分型、病理分级及免疫分型,并分析之间的相互关系。结果本组IgAN发病率男童多于女童(2.5∶1.0),临床表现为单纯性肉眼血尿14例(66.7%),血尿伴蛋白尿4例(19.1%),肾病综合征1例(4.7%),肾炎综合征2例(9.5%),病理改变以Ⅲ级为主,免疫球蛋白沉积以复合型为主。结论随着对无症状血尿、蛋白尿者肾活检的增多,小儿IgAN的诊断有逐年增加趋势。IgAN临床表现多样,几乎包括肾小球疾病的所有类型,且临床与病理有一定关系。单纯血尿者病理改变相对较轻,预后较好;蛋白尿者病理改变较重,应早期诊断,早期治疗。     

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目的了解伴新月体形成的原发性IgA肾病(IgAN)患儿临床与病理特点。方法选择2000年1月至2011年1月在温州医学院附属育英儿童医院经肾活检确诊为原发性IgAN且病历资料完整的患儿78例,根据是否伴新月体形成分为伴新月体形成的IgAN组(C组)和不伴新月体形成的IgAN组(NC组),并比较两组结果。结果 78例原发性IgAN患儿中男性57例,女性21例;年龄平均(9.32±3.16)岁。C组33例(42.3%),NC组45例(57.7%)。与NC组相比,C组肾活检前病程更短[(2.15±4.06)个月对(9.87±19.09)个月,P<0.05],肉眼血尿发生率更高(30/33对25/45,P<0.01),24h尿蛋白定量更多[(93.08±82.75)mg/(kg·d)对(44.92±68.44)mg/(kg·d),P<0.05],且表现为大量蛋白尿者更多(19/31对10/42P<0.01),肾功能损害者较多(8/33对2/45,P<0.01)。在肾脏病理改变上,C组中-重度系膜增生、球囊粘连、毛细血管内皮增生均显著多于NC组(P均<0.01)。结论儿童伴新月体形成的原发性IgAN临床表现、病理改变较重,临床上应提高对此类型IgAN的认识,争取早期肾活检,早期诊断,积极治疗,在急性期控制疾病进展以改善预后。     

原发性免疫球蛋白A肾病55例临床病理分析

目的探讨儿童原发性免疫球蛋白A肾病(IgAN)的临床、病理特征及预后。方法对1996~2005年经肾活检确诊为原发性IgAN的患儿55例进行详尽的临床病理分析。本组男35例,女20例,发病年龄2~16岁,平均9岁,占同期肾活检的10.5%。结果临床表现为肾病综合征占30.9%、孤立性血尿占25.5%、血尿蛋白尿占23.6%、急性肾炎综合征占18.2%、慢性肾炎综合征占1.8%;病理分级以Ⅲ级多见(61.8%),其次为Ⅳ级(21.8%)和Ⅱ级(12.7%),Ⅰ级仅占3.6%;免疫病理分型IgA IgM IgG( C3)型占45.5%,IgA IgM( C3)型30.9%,IgA单独沉积21.8%,满堂亮者1.8%。双向有序χ2检验表明临床表现的严重程度与病理分级间存在线性关联,伴蛋白尿者病理改变较重;且临床表现与免疫病理分型间也具有一定相关性,孤立性肉眼血尿患儿中,以IgA型较多见,而表现为肾病综合征患儿中,IgA IgM IgG( C3)型最多见。对其中24例平均随访39个月,除1例孤立性血尿尿检无改变,1例血尿蛋白尿蛋白尿好转血尿无改善外,其他患儿均明显好转,仅有轻微血尿或微量蛋白尿。结论儿童原发性IgAN的临床表现与病理特征存在一定程度关联。临床表现为肾病综合征及肾炎综合征者病理改变较重,以Ⅲ、Ⅳ级为主,而孤立性血尿者病变较前者轻。     

IgA肾病患儿血清IgA1低糖基化水平的变化及临床意义

目的 探讨IgA肾病(IgAN)患儿血清IgA1低糖基化水平的变化及临床意义.方法 肾活检证实为IgAN的26例患儿按临床表现分为孤立性血尿组(10例)、血尿蛋白尿组(6例)和肾病综合征组(10例);按病理分级分为病理Ⅰ加Ⅱ级组(5例)、Ⅲ级组(11例)和Ⅳ加Ⅴ级组(10例).同期门诊体检的健康儿童20例作为健康对照组.以蚕豆凝集素(VVL)亲和ELISA检测VVL与血清IgA1的结合力,间接反映血清IgA1糖基化程度(结合力增高说明IgA1呈低糖基化).采用SPSS 11.0软件进行分析,组间比较采用t检验、单因素方差分析和LSD-t法,相关分析采用Pearson法.结果 IgAN患儿血清IgA1低糖基化水平显著高于健康对照组(P=0),在不同临床表现和病理分级组中的差异无统计学意义(P>0.05).IgA1低糖基化水平与患儿起病年龄、尿红细胞计数、24 h尿蛋白定量、血清清蛋白(Alb)、胆固醇(Chol)、BUN、Cr水平和内生肌酐清除率(Ccr)无相关性.采用VVL亲和ELISA测定其血清IgA1与VVL结合力反映IgA1低糖基化水平诊断IgAN的灵敏度为88.5%,特异度为90.0%,与肾穿病理诊断的符合率为88.5%,阳性预测价值为92.0%,阴性预测价值为85.7%.结论 IgAN患儿血清存在IgA1低糖基化,其低糖基化水平与临床表现和病理改变无关.采用VVL亲和ELISA测定血清IgA1与VVL结合力反映IgA1低糖基化水平这一方法 在诊断儿童IgAN方面显示出很好的有效性和实用性,对临床无创性诊断IgAN有一定的应用价值.     

表现为孤立性血尿的儿童IgA肾病临床与病理分析

目的 探讨表现为孤立性血尿的儿童IgA肾病（IgAN）的临床、病理特点及肾穿的必要性。方法回顾性分析52例表现为孤立性血尿的儿童IgAN的临床与病理特点。其中男36例，女16例；平均年龄10、2岁。结果发作性肉眼血尿占78．8％，Lee病理分级Ⅲ级占78．8％，有肾脏不良病理表现占48％。发作性肉眼血尿与镜下血尿Lee病理分级、肾脏不良病理表现百分率、肾脏免疫球蛋白沉积类型、IgA沉积强度比较差异均无显著性（P=0．227，0．845，0．276，0．957）。肾脏免疫荧光Fg、C1q阳性者肾脏不良病理表现百分率分别高于Fg、C1q阴性者（P=0．013，0．048）。结论 表现为孤立性血尿儿童IgAN以发作性肉眼血尿多见，Lee病理分级以Ⅲ级多见，常有肾脏不良病理表现，血尿与肾脏病理严重程度不成正比，即使是表现为镜下血尿者也应及早行肾活检。肾脏Fg、C1q沉积可能与肾脏不良病理表现有关。     

原发性局灶节段性肾小球硬化症患儿21例的临床、病理特点与预后分析

目的观察原发性局灶节段性肾小球硬化症(FSGS)患儿的临床表现、肾脏病理特点及其与预后的关系。方法收集重庆医科大学附属儿童医院2003年6月-2008年6月经肾活检确诊为原发性FSGS的21例患儿的临床病理资料,分析其临床表现、实验室指标和肾脏病理特点,随访患儿的预后,并对影响预后的相关因素进行分析。结果21例FSGS患儿临床诊断以肾病综合征最多[15例(71%)],病理分型以非特殊型最多[8例(38%)],病理类型与临床表现无对应关系(P>0.05)。21例中2例失访,余19例随访4～51(22.84±12.13)个月,完全缓解10例(53%),显著缓解4例(21%),部分缓解4例(21%),无效1例(5%)。肾小管间质病变与FSGS的预后相关(P<0.05),是FSGS预后的危险因素(OR>1)。结论1.原发性FSGS患儿临床表现以肾病综合征最多,非特殊型是原发性FSGS的主要病理类型,不同病理类型与临床表现无对应关系。2.原发性FSGS患儿短期预后良好,应加强长期预后的随访观察。3.肾小管间质病变是影响预后的危险因素。     

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.