227例小儿原发性肾小球疾病232次肾活检结果分析

本文报告4年来我院227例小儿原发性肾小球疾病(PGD)肾活检结果,其中5例作肾活检复查。临床表现为肾病综合征103例,单纯性血尿或/和蛋白尿97例,急性肾炎16例,迁延性肾炎3例,慢性肾炎3例,家族性肾炎5例。病理表现共10类,其中微小病变(MCN)13例(5.7%),系膜增生性肾炎(MsFGN)140例(61.7%),局灶性肾炎(FGN)12例(5.3%),膜性增生性肾炎(MPGN)9例(4%),膜性肾炎(MN)2例(0.9%),局灶节段性肾小球硬化(FSGS)9例(4%),新月体性肾炎(CrGN)1例(0.4%),硬化性肾小球肾炎(SCGN)3例(1.3%),弥漫毛细血管内增生性肾炎(EnPGN)15例(6.6%),IgA肾病(IgAGN)23例(10.1%)。5例肾活检复查结果:2例由MCN转为FSGS,1例MCN转为MsPGN,1例MsPGN转为兼有FSGS,1例由FGN转为MsPGN。     

原发性肾病综合征患儿肾组织中p18的表达及意义(英文)

目的：肾脏固有细胞的异常增生是肾小球硬化发展的病理基础,细胞增生又受细胞周期调控物质的调节。本研究通过探讨肾病综合征患儿肾组织细胞周期调节蛋白p18的表达水平及其与肾脏固有细胞增生之间的关系,为抑制肾脏固有细胞的异常增生,延缓肾脏病的慢性进展开辟新的途径。方法：以39例原发性肾病综合征患儿［8例微小病变(MCD),15例系膜增生性肾小球肾炎(MsPGN),7例膜增生性肾小球肾炎(MPGN),9例局灶节段性肾小球肾炎(FSGS)］肾活检石蜡包埋肾组织及6例肾肿瘤肾切除病人的正常肾组织作为研究对象,用免疫组织化学方法检测了p18在肾组织的表达水平,分析其与肾病综合征的病理类型及肾组织中增殖细胞核抗原(PCNA)的表达的关系。结果：肾病综合征组肾小球内PCNA阳性细胞百分率(28.6%±3.4%)明显高于对照组(10.8%±3.4%)(P<0.05),p18阳性细胞百分率(35.8%±4.0%)明显高于对照组(6.1%±1.9%)(P<0.05);肾病综合征组肾小管-间质内的PCNA阳性细胞百分率(68.3%±11.6%)明显高于对照组(12.6%±2.6%)(P<0.05)。不同病理类型肾病综合征患儿的肾小球内PCNA阳性细胞百分率存在显著差异,分别为MCD 23.6%±4.6%,MsPGN 40.2%±5.1%,MPGN 27.5%±3.6%,FSGS 34.6%±5.1%(均P<0.05);不同病理类型肾病综合征患儿的肾小球内p18阳性细胞百分率存在显著差异,分别为MCD 25.2%±4.3%,MsPGN35.7%±7.1%,MPGN 37.7%±4.0%,FSGS 40.1±6.4%(均P<0.05)。肾小球内p18的阳性细胞百分率和PCNA阳性细胞百分率成正相关(r=0.6632,P<0.05)。结论：肾病综合征患儿肾组织p18表达水平增加可能起促进异常增生的肾脏固有细胞消退的作用。［中国当代儿科杂志,2004, 6(5): 373-376］     

儿童肾病综合征肾组织病理光镜和免疫荧光分型的关系

目的 探讨肾组织光镜病理和免疫荧光病理的相关性.方法 回顾性分析410例原发性肾病综合征行经皮肾活检患儿的肾组织病理资料.结果 410例患儿肾组织光镜病理类型分布为:轻微病变(MCNS)133例(32.4%),系膜增生性肾小球肾炎(MsPGN)229例(55.9%),膜增生性肾小球肾炎(MPGN)5例(1.2%),膜性肾小球肾炎(MN)7例(1.7%),局灶节段硬化(FSGS)6例(8.8%).肾组织免疫荧光病理类型分布为:IgA型41例(10%),IgM型66例(16.1%),IgA+M+G+C3型 9例(2.2%),补体为主型8例(2%),无免疫复合物型286例(69.8%).MCNS、MsPGN、FSGS均以无免疫复合物型多见,MPGN以补体为主型多见,MN型以IgA+M+G+C3型多见.5种不同肾组织光镜病理类型中的免疫荧光类型分布差异有统计学意义(χ2 ＝ 87.673,P ＜ 0.01).结论 5种肾组织光镜病理的免疫荧光病理存在差异.     

儿童原发性肾病综合征α-辅肌动蛋白4mRNA表达的临床意义

目的探讨在儿童原发性肾病综合征中α-辅肌动蛋白4(α-actinin-4)mRNA表达及其意义。方法经肾活检获取34例原发性肾病综合征患儿(病例组)的肾组织,其中局灶节段硬化性肾小球肾炎(FSGS)21例、微小病变(MCNS)6例、系膜增生性肾小球肾炎(MsPGN)7例;另外,12例对照组标本取自肾肿瘤切除术后肿瘤周边的正常组织。通过实时荧光定量PCR(quantitative real-time PCR)的方法检测每份肾组织中α-actinin-4 mRNA表达,同时分析FSGS患儿中mRNA表达与24 h尿蛋白、内生肌酐清除率(Ccr)的相关性。结果 FSGS组α-actinin-4 mRNA表达明显高于对照组,差异有统计学意义(P0.05),而MCNS组和MsPGN组α-actinin-4 mRNA表达与正常对照组的差异均无统计学意义(P0.05)。在FSGS组中,表现为肾炎型肾病(伴发镜下血尿)患儿的α-actinin-4 mRNA表达明显高于表现为单纯性肾病的患儿,差异有统计学意义(P0.05);FSGS患儿α-actinin-4mRNA表达量与24 h尿蛋白及Ccr无相关性(P0.05)。结论在儿童原发性肾病综合征中,α-actinin-4 mRNA表达量增高可能是促进肾小球足细胞骨架修复的因素之一;该骨架分子的mRNA表达变化与病程及病理类型相关。     

47例Alport综合征临床与病理分析

目的 分析不同年龄组Alport综合征患儿的临床与病理特点.方法 回顾性分析我院1990年1月-2007年1月47例住院并且明确诊断为Alport综合征患儿的临床及病理资料.结果 男32例,女15例,男女比例2.1:1,年龄15个月～13岁,平均9.0岁.47例患儿中39例有明确家族史,其中X连锁显性遗传37例;常染色体隐性遗传2例.28例(59.3%)患儿首发症状为肉眼血尿或镜下血尿,14例(29.8%)患儿为蛋白尿、水肿.Alport综合征临床表现为孤立性血尿11例(23.4%)、血尿合并蛋白尿17例(36.2%)、肾病综合征14例(29.8%)、肾功能不全5例(10.6%);孤立性血尿型、血尿合并蛋白尿型见于研究病例中的所有年龄组儿童,而肾病综合征型、肾功能不全型仅见于7～13岁年龄组儿童.肾组织病理显示,33例(70.2%)光镜改变为系膜增生性病变(MsPGN),13例(27.6%)表现为局灶节段性肾小球硬化(FSGS),1例(2.1%)表现为膜增生性肾小球肾炎(MPGN).免疫荧光多以IgM沉积为主19例(40.4%),以IgA沉积为主9例(19.1%),以IgG沉积为主9例(19.1%),免疫荧光阴性10例(21.4%).电镜下39例出现典型的肾小球基底膜病变,8例显示基底膜变薄.47例患儿中46例肾脏和(或)皮肤Ⅳ胶原a链分布异常.结论 Alport综合征男性患儿发病率高于女性.不同年龄组Alport综合征肾脏表现有明显差异,血尿伴随疾病始终,但随着病程延长,尿蛋白量逐渐增加.Alport综合征肾脏病理光镜下无特征表现,主要以系膜增生为主,小年龄组患儿电镜下GBM病变不典型,需结合Ⅳ胶原a链免疫荧光检测明确诊断.     

泌尿生殖系统疾病

032205小儿孤立性血尿的病理分析/张桂菊一//临床儿科杂志一2003,21(l)一40一41 45例中薄基底膜肾病13例(28.9%),馆A肾病n例(24.5%).系膜增生性肾小球肾炎8例(17.8%),Al-port综合征6例(13.3%),肾小球轻微病变4例(8 .9%),局灶节段性硬化2例(4.4%).IgM肾病l例(2.2%)。提示:遗传性肾小球基底膜病占小儿单纯性血尿的第l位.但有明确家族史的不足50%。IgA肾病也是主要原因之一,表现为孤立性血尿的IgA肾病病理变化相对较轻。表2参9(蔡虹蔚) 032206输尿管硬镜下气囊扩张术治疗肾孟输尿管连接部狭窄24例/刘敬斌//实用儿科临床杂志一2003 .18(l)一…     

环磷酰胺冲击治疗儿童激素耐药型肾病综合征的疗效

目的 探讨激素耐药型肾病综合征(SRNS)患儿大剂量环磷酰胺(CTX)冲击治疗的疗效及影响疗效的相关因素.方法 总结本院2004年12月～2009年12月收治的资料完整并进行CTX静脉冲击治疗的38例SRNS患儿的病例资料及随访情况,并就其缓解情况及与临床分型、病理类型、临床指标之间的关系及药物不良反应进行回顾性分析.结果 1.本组完全缓解18例(47%),部分缓解11例(29%),总有效率76%;部分缓解患儿均发生于疗程6个月内,延长疗程无累积缓解率增加;未缓解患儿1例在起病5 a内进展至终末期肾病.2.SRNS患儿单纯型肾病组缓解率高于肾炎型肾病组.3.SRNS病理改变以非微小病变肾病为主,即以系膜增生性肾小球肾炎(MsPGN)、局灶节段性肾小球硬化(FSGS)为主;其中MsPGN缓解率较高,而FSGS缓解率相对较低.4.不良反应发生率为53%,最常见不良反应为一过性胃肠道反应,占36%,其次为轻度脱发(10%)、白细胞降低(7%),无严重感染、出血性膀胱炎发生.结论 CTX冲击联合激素治疗SRNS疗效肯定;临床类型、病理类型等因素可能与疗效及预后有关,单纯型肾病、MsPGN有效率较高,FSGS患儿CTX冲击疗效较差,发展为终未期肾病的风险较高.     

系膜增生性肾炎患儿肾组织血小板源性生长因子及其受体表达的临床研究

目的研究系膜增生性肾炎患儿肾组织血小板源性生长因子(PDGF)及其受体(PDGFR-α、β)的表达及意义.方法采用免疫组化染色法检测弥漫性系膜增生性肾小球肾炎(MsPGN)、局灶性节段性肾小球硬化(FSGS)肾活检组织中PDGF、PDGFR的表达.结果PDGF在MsPGN中的表达明显高于正常肾和FSGS,PDGFR-α、β的表达高于正常肾(P＜0.01);PDGF在FSGS中的表达与正常肾相比无差异,但PDGFR-α、β表达升高(P＜0.01);MsPGN与FSGS的PDGFR-α、β表达无明显差异(P＞0.05).结论肾组织PDGF及其受体的过度表达可能在MsPGN的发病中起重要作用.     

儿童无症状血尿431例病因分析及随访

目的探讨儿童无症状血尿的病因和转归?方法回顾性分析2001年1月至2014年12月就诊的431例无症状血尿患儿的病因、临床特点及预后?结果 431例患儿中,男197例、女234例,初诊年龄(5.52±2.77)岁(8个月~17岁);持续镜下血尿425例,发作性肉眼血尿6例。肾小球源性血尿315例(73.1%),其中临床诊断为孤立性血尿286例,急性肾小球肾炎5例;病理诊断肾小球轻微病变13例,Ig A肾病4例,系膜增生性肾小球肾炎4例,薄基底膜肾病3例。非肾小球源性血尿136例(31.5%),其中左肾静脉压迫综合征113例,特发性高钙尿症17例,肾结石4例,泌尿道感染1例,左肾缺如1例。286例孤立性血尿患儿随访(3.05±2.69)年(0.5~13.5年),145例(50.7%)血尿消失,其中110例在初诊后3年内消失;24例伴有血尿家族史的患儿中仅有6例血尿消失;所有孤立性血尿患儿随访结束时肾功能都维持良好?结论儿童无症状血尿发病年龄跨度大,以肾小球源性血尿居多;儿童孤立性血尿大多在初诊后3年内消失,伴有血尿家族史者血尿持续时间可能更长;儿童期孤立性血尿预后良好,需长期随访。     

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。     

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.     

�¶�֢��ϵ�ϰ���ע��ȱ�ݶද�ϰ������ڵ�ת��

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.     

How well are we doing? – Metabolic control in patients with diabetes

OBJECTIVE: To compare the present level of metabolic control in children and adolescents with insulin-dependent diabetes mellitus (IDDM) attending Brisbane paediatric diabetes clinics with published overseas data. METHODOLOGY: Blood HbA1c concentrations, population characteristics, current treatment practices and short-term complications were recorded in all patients, aged 19 years and under, attending the diabetes clinics of the two Brisbane Children's Hospitals or the private practice of one of the authors (MJT) in the first quarter of 1998. RESULTS: Two hundred and sixty-eight patients were assessed (M/F 142/126). Ages ranged from 1 to 19 years (mean 11. 2 years); duration of IDDM was 0-16 years (mean 4.4 years); and 141 (53%) were pubertal. Of those aged less than 13 years, only 4% had more than two injections daily. Insulin doses (U/kg/day) rose with increasing age. Larger doses were required in regimens involving more than two injections per day than those involving one to two injections per day. Ketoacidosis or severe hypoglycaemia in the last 3 months were reported in eight (2.7%) and 17 (6.3%) of patients, respectively. Mean HbA1c (+/- SD) was 8.6 +/- 1.4% (range 5.2-14.0%), with 33% of children having a HbA1c concentration < 8%. HbA1c concentrations were significantly related (P < 0.05) to insulin dose and to duration of diabetes, but not to severe hypoglycaemia, ketoacidosis, age, frequency of injections, or number of clinic visits per year. Mean HbA1c concentration was significantly higher (P < 0.05) in those children in puberty (8.7 +/- 1.5%) than in those not in puberty (8.5 +/- 1.2%). CONCLUSION: Only 33% of patients had a HbA1C concentration less than 8% and 6.3% had a severe hypoglycaemic episode in the 3 months. These results are similar to published overseas data.