儿童紫癜性肾炎55例临床与病理分析

目的 探讨儿童紫癜性肾炎(Henoch-Sch(o)nlein purpura nephritis,HSPN)的临床和病理特点及其相关性,以指导治疗,改善预后.方法 收集2000年1月至2008年10月收治的55例紫癜性肾炎患儿的临床及病理资料,并且根据不同的条件分组进行网顾性分析.结果 临床分型中以血尿和蛋白尿最多,为22例(40.0%);肾病综合征型次之,16例(29.1%);单纯性蛋白尿或单纯性血尿型、急性肾炎型和急进性肾炎型占少数,分别为6例(10.9%)、4例(7.3%)和1例(1.8%).伴有消化道症状的紫癜性肾炎患儿,临床上较无消化道症状的患儿易有肾病水平蛋白尿(P<0.05).伴肉眼血尿患儿比镜下血尿者易出现肾病综合征(P<0.05).根据ISKDC标准,HSPN病理分级以Ⅱ级和Ⅲ级最多,分别为27例(49.1%)和16例(29.1%).免疫荧光检查显示IgA+IgG+IgM型为32例(58.2%),伴C3沉积41例(74.6%),病理分级主要在Ⅲ级以上.免疫沉积类型、C3沉积与病理分级有相关性(P<0.05).临床表现为单纯性血尿型或单纯性蛋白尿型的HSPN病理损害较轻,7例(58.3%)为Ⅱ级;血尿和蛋白尿型的病理分级以Ⅱ级和Ⅲ级多见,分别为12例(54.5%)和5例(22.7%);肾病综合征型的病理分级多见Ⅲ级以上,为10例(62.5%).结论 儿童紫癜性肾炎临床以血尿和蛋白尿型及肾病综合征型为主,病理分级以Ⅱ级和Ⅲ级最常见,免疫沉积物类型与C3沉积和病理分级有相关性.HSPN临床症状和病理损伤程度基本一致;消化道受累程度越重,出现蛋白尿的比例越高;肉眼血尿与肾脏损害的严重程度有关.     

血管紧张素转换酶基因多态性对过敏性紫癜的影响

目的 探讨过敏性紫癜(HSP)与血管紧张素转换酶(ACE)基因多态性间的关系。方法 选择70例HSP患儿,其中无肾脏损害32例,过敏性紫癜性肾炎(HSPN)38例。HSPN中单纯性血尿15例,蛋白尿23例。健康对照儿童100例。采用聚合酶链反应(PCR)检测ACE基因型。结果 1.HSP患儿与健康对照组ACE基因型分布比较无差异(P>0.05);2.非肾脏损害患儿32例与伴单纯性血尿HSPN患儿15例ACE基因型分布比较亦无差异(P>0.05);但与23例伴蛋白尿HSPN患儿ACE基因型分布比较,缺失型(DD),型者发生蛋白尿频率明显升高(P<0.05)。结论 HSPN蛋白尿发生与ACE基因多态性相关,DD型者发生蛋白尿机会明显增多。     

儿童紫癜性肾炎临床与病理相关性分析

目的：通过对95例紫癜性肾炎（HSPN）患儿临床表现及肾脏病理分析,阐明其临床及病理之间的联系。方法：对HSPN患儿进行临床分型及病理分级,对其进行综合分析。结果：①临床分型以肾病综合征型(27.4%)、蛋白尿＋血尿型(24.2%)多见,病理分级以Ⅲb(42.1%)最多见;②尿检正常者可见肾脏病理改变。尿检正常型、孤立性血尿或蛋白尿型以及血尿和蛋白尿型病理改变差异无显著性(P>0.05);③孤立性血尿或蛋白尿型以及血尿和蛋白尿型病例,病程越长病理分级也越重(P＜0.05);④免疫复合物沉积以IgA+IgG+IgM（58%)同时存在比例最高;病理分级越重,病程越短,IgA+IgG+IgM比例越高。结论：HSPN患儿临床表现为肾病综合征和肾炎型者病理改变相对较重,临床症状与病理不一定平行,尿检正常者病理改变也很明显,病程越长,病理改变呈加重趋势。免疫复合物沉积为IgA+IgG+IgM的病理改变相对较重。［中国当代儿科杂志,2007,9（2）：129-132］     

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。     

儿童紫癜性肾炎200例临床与病理分析

目的 探讨紫癜性肾炎(HSPN)患儿的临床表现与肾脏病理类型及转归的关系.方法 对200例HSPN患儿行经皮肾穿刺活检,同时测定免疫相关指标.根据不同的临床病理类型给予不同治疗方案,并对临床表现、实验室检查和组织学检查资料进行回顾性分析.结果 临床分型以血尿、蛋白尿型103例(51.5%)最多,肾病综合征型40例(20%)次之,急性肾炎型24例(12%)、单纯性血尿型或蛋白尿型20例(10%),急进性肾炎型8例(4%),慢性肾炎型5例(2.5%);肾脏病理分级以Ⅱ、Ⅲ级最多.有7例临床分型轻而病理分级为Ⅲ、Ⅳ级,有6例临床分型重,而病理分级为Ⅰ、Ⅱ级;免疫病理分型以 IgA + IgM沉积型最多(36%),其次为IgA + IgG + IgM沉积型(33%).随访0.5 ～ 5年,平均2.8年.结论 儿童HSPN临床症状的严重程度与病理损伤的程度不完全一致,病理分级与临床分型存在不平行性;病理级别Ⅲ级以下的治疗效果明显好于Ⅲ级以上,随着HSPN病理分级增高,临床治疗困难增加;皮质激素和免疫抑制剂环磷酰胺疗效肯定;经0.5 ～ 5年随访总体预后良好,有少部分患儿进入终末期肾病,尽快尽早治疗尤为重要.     

儿童IgA肾病临床表现与病理分析

目的探讨IgA肾病临床表现与病理的关系。 方法对1996—2005上海交通大学附属儿童医院住院的77例患儿,进行肾组织活检确诊为原发性IgA肾病。参照Lee修改的Meadow病理分级标准将IgA肾病分级。 结果血尿蛋白尿33例,孤立性血尿22例,肾病综合征12例,急性肾炎9例,孤立性蛋白尿1例。病理分级：Ⅰ级11例,Ⅱ级14例,Ⅲ级47例,Ⅳ级5例。 结论呈孤立性血尿的IgA肾病病理改变相对较轻,随着蛋白尿的增多,肾损害逐渐加重。     

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目的探讨IgA肾病临床表现与病理的关系。方法对1996—2005上海交通大学附属儿童医院住院的77例患儿,进行肾组织活检确诊为原发性IgA肾病。参照Lee修改的Meadow病理分级标准将IgA肾病分级。结果血尿蛋白尿33例,孤立性血尿22例,肾病综合征12例,急性肾炎9例,孤立性蛋白尿1例。病理分级:Ⅰ级11例,Ⅱ级14例,Ⅲ级47例,Ⅳ级5例。结论呈孤立性血尿的IgA肾病病理改变相对较轻,随着蛋白尿的增多,肾损害逐渐加重。     

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目的探讨儿童紫癜性肾炎(HSPN)的临床、病理分型分级与预后的关系。方法回顾性分析2000年1月至2008年10月在新疆维吾尔自治区人民医院儿科住院的55例HSPN患儿的临床、病理特点,分析对远期预后影响的因素。结果临床表现为血尿和蛋白尿者22例(40.0%),肾病综合征16例(29.1%),单纯性血尿或蛋白尿、急性肾炎和急进性肾炎者分别为12例(21.8%)、4例(7.3%)和1例(1.8%)。肾脏病理表现为系膜增生、肾小球硬化、新月体形成,国际小儿肾脏病研究组分级多见Ⅱ级和Ⅲ级,分别为27例(49.1%)和16例(29.1%)。临床分型与病理分级有相关性(P<0.05),临床分型越严重,肾脏病理损害越重,预后不佳。病理分级与预后有相关性(P<0.05),病理分级越重,预后越不佳。肾小管-间质病理分型与病理分级有相关性(P<0.01),随着肾小管-间质病变程度加重,肾脏病理损伤的程度越重,患儿预后不良。结论儿童紫癜性肾炎的临床表现、病理分级与远期预后密切相关,临床表现重,病理分级高,预后差。     

IgA肾病97例的临床和病理分析

目的探讨儿童IgA肾病的临床与病理特点的关系。方法对2005年1月-2010年6月经本院肾脏病理室确诊为IgA肾病的97例患儿的临床表现,病理特点及相关实验室检查进行回顾性分析。结果 97例患儿中男女比例为2.61,发病年龄(10.6±2.9)岁。临床表现以肾病综合征最多见(40.2%),其次为孤立性血尿(30.9%),病理类型以轻度系膜增生性IgA肾病最多见(29.9%),组织分级以Ⅲ级改变为主(52.6%),免疫荧光分型以IgA+IgM型多见(45.4%)。将临床表现由轻到重分为孤立性血尿、血尿蛋白尿、肾病及肾炎综合征3组,病理改变分为Ⅰ～Ⅱ级、Ⅲ级、Ⅳ～Ⅴ级3个级别,行双向有序χ2检验,差异无统计学意义(χ2=4.081,P=0.395);将免疫病理分为单纯IgA沉积型与复合沉积型2组,其临床表现不同,差异有统计学意义(χ2=8.421,P=0.015)。结论儿童IgA肾病以学龄期多见,男性多于女性。临床表现与免疫病理分型相关,单纯IgA沉积者临床表现较轻。临床分型与组织分级间未发现显著联系。     

儿童紫癜性肾炎临床与病理分析

目的探讨儿童紫癜性肾炎(HSPN)临床及病理特点。方法回顾性分析2009年1月至2014年1月住院的242例HSPN患儿的临床、病理特点。结果 HSPN临床分型最常见为血尿和蛋白尿型(135例,55.79%);病理分型主要集中在Ⅱ和Ⅲ型。不同临床分型HSPN患儿的病理分型分布差异有统计学意义(H=30.46,P0.001);尿检正常型的病理分型最轻,孤立性血尿或蛋白尿型的病理分型以Ⅱ型较多,血尿和蛋白尿型以Ⅲa型较多,而肾病综合征型以Ⅲb型较多。肾穿时病程长短在不同临床(H=0.02,P0.05)及病理分型(H=2.08,P0.05)间的差异均无统计学意义;肾脏免疫复合物沉积在不同病理分型间差异无统计学意义(P0.05);血Ig A、补体C3水平以及血小板计数在不同病理分型和临床分型间的差异也均无统计学意义(P均0.05)。结论 HSPN患儿病理分型与临床分型相关。     

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.