儿童过敏性紫癜性肾炎与IgA肾病的临床和病理的对比研究

目的　对比研究过敏性紫癜性肾炎 (HSPN)和IgA肾病在临床、实验室检查及肾脏病理改变上的异同 ,探讨两者的关系。方法　对经肾活检证实的 31例IgA肾病及 12 0例过敏性紫癜性肾炎患儿进行详细的临床及病理对比分析。结果　 2 5 8%的IgA肾病发病年龄在 12岁以上 ,而HSPN中仅占 10 .0 % ,差异有显著意义 (P <0 0 5 )。二组患儿临床表现类型分布上相似 ,但HSPN肾外症状多 ,均有皮肤紫癜 ,5 9 2 %有胃肠症状 ,4 6 7%有关节痛 ,而IgA肾病仅 3 2 %有腹痛。在肾脏病理改变上 ,IgA肾病 35 5 %出现球性硬化、4 1 9%系膜硬化 ,HSPN分别为 3 1%及 6 3% ,但HSPN有 6 5 6 %出现内皮增生 ,IgA肾病仅 2 9 0 % ,差异均有非常显著意义 (P <0 0 1)。IgA肾病中 6 5 %合并有薄基底膜病变 ,HSPN未见薄基底膜病变。电镜下HSPN电子致密物稀疏、松散 ,沉积部位分布较为广泛 ,位于肾小球系膜、内皮下甚至基底膜内 ,而IgA肾病电子致密物成密集团块状 ,主要局限于系膜区及旁系膜区。HSPN患儿中 2 3例 (71 9% )肾小球免疫沉积物中含有IgG ,其中 6例以IgG为主 (4例 )或毛细血管壁有IgG线样沉积 (2例 ) ,而IgA肾病肾小球免疫沉积物中有IgG沉积的仅为 19 4 % ,其多为IgA伴IgM和 (或 )C3 沉积 ,未见以IgG沉积为主以及线样IgG沉积     

肾小球疾病156例的临床与病理

了解各种小儿肾脏疾病的临床表现和病理类型二者间的关系。方法对156例肾活检患儿的临床和病理的关系进行了分析。结果系膜增生性肾炎占68％,其临床表现为多种形式的肾脏疾病;IgA肾病22例,占14．1％;毛细血管内增生性肾炎占4．5％;新月体肾炎6例（3．8％）;其他型别的病理类型均较少见。结论系膜增生性肾炎是小儿肾脏疾病最常见的病理类型,临床主要见于紫癜性肾炎（34．9％）,其次见于肾病综合征（28．3％）,各种类型的肾小球肾炎（10．4％）。     

儿童IgA肾病72例临床与病理分析

目的探讨儿童IgA肾病(IgAN)的临床、病理特点及其相关关系。方法对本院2005年5月-2011年8月经肾穿刺活检确诊为IgAN的72例患儿的临床表现、临床分型、病理特点及免疫分型进行回顾性总结,并分析它们之间的相关关系。结果本组72例。男48例,女24例;年龄1岁5个月～17岁[(8.99±2.94)岁];入院时病程2 d～9 a(平均12.86个月)。临床以血尿起病者58例(包括38例肉眼血尿及5例伴水肿者),以单纯水肿起病者12例,以蛋白尿起病者2例。临床分型为肾病综合征型28例(38.89%)、孤立性血尿型19例(26.39%)、血尿和蛋白尿型13例(18.05%)、急性肾炎型10例(13.89%)、孤立性蛋白尿型2例(2.78%)。病理改变:系膜增生型肾小球肾炎40例,局灶增生型肾炎25例、毛细血管内增生型肾炎6例、新月体型肾炎1例。其中伴新月体形成者17例(占23.61%)。免疫组织化学可见多种免疫球蛋白沉积。沉积类型为满堂亮型1例、IgA+IgG+C32例、IgA型8例、IgA+IgM+IgG+C3型17例、IgA+IgM+C3型44例。结论 IgAN的临床表现形式多样,其病情轻重与起病形式无关。病理表现以系膜增生型肾小球肾炎为主,免疫球蛋白沉积以复合型为主。临床表现为肾病综合征型及血尿和蛋白尿型者病理较重,应尽早行肾穿,及时治疗。     

小儿急进性肾炎和新月体肾炎的再认识:附43例临床与病理分析

目的 探讨小儿急进性肾炎和新月体肾炎的病因、临床以及病理特点及两者之间的关系。方法 回顾总结1987年至2002年临床诊断急进性肾炎或病理诊断新月体肾炎的43例住院患儿的临床资料。结果 患儿以学龄儿童多见，60．5％为原发性肾脏疾病，绝大多数表现为浮肿、少尿、高血压、肉眼血尿及肾病水平蛋白尿。部分(24．0％)急进性肾炎的肾脏病理为非新月体肾炎，包括毛细血管内增生性肾小球肾炎、Ⅳ型膜增殖性狼疮性肾炎、增生硬化性肾小球肾炎及局灶节段性肾小球硬化：而部分(32．1％)新月体肾炎的临床表现为非急进性肾炎，包括肾病综合征(肾炎型)、急性肾炎综合征及慢性肾脏疾病。予正规治疗的25例患儿中，44．0％的患儿肾功能恢复，44．0％的患儿肾功能好转。结论 急进性肾炎是临床诊断，新月体肾炎是病理诊断，两者并非完全一致；积极行肾穿刺活检，有助于明确肾脏病理类型，指导治疗；早期诊断、及时治疗有助于改善急进性肾炎或新月体肾炎患儿的预后。     

儿童肾脏疾病临床与病理753例分析

目的分析和总结儿童肾脏疾病的临床资料和病理类型,探讨肾穿刺活检术在儿童肾脏疾病中的重要性。方法回顾性分析1995—2015年在山东大学附属省立医院小儿肾脏风湿免疫科接受经皮肾穿刺活检的753例肾脏疾病患儿临床资料和病理资料。结果 753例经皮肾穿刺活检患儿中,原发性肾小球疾病428例(56.84%)、继发性肾小球疾病306例(40.64%)、先天性遗传代谢性肾脏疾病17例(2.26%)、肾小管间质性疾病2例(0.27%)。临床诊断最常见的为原发性肾病综合征。原发性肾小球疾病中最常见的临床表现和病理类型分别为原发性肾病综合征和IgA肾病,继发性肾小球疾病中常见的临床表现和病理类型分别为紫癜性肾炎和系膜增生性肾小球肾炎。先天性遗传代谢性肾脏疾病中最常见的为薄基底膜肾病和Alport综合征。8例重复肾活检患儿的病理类型及临床表现均出现改变。结论儿童肾脏疾病以原发性肾小球疾病为主,最常见的临床诊断和病理类型分别为原发性肾病综合征和系膜增生性肾小球肾炎。重复肾穿刺活检有助于及时掌握患儿病理类型转化,调整治疗方案。     

儿童C3肾小球肾炎6例临床与病理分析

目的分析6例儿童C3肾小球肾炎的临床表现、病理特征及治疗反应,旨在提高儿科医生对此病的认识和处理。方法对2010年9月至2016年6月在吉林大学第一医院小儿肾病科确诊为C3肾小球肾炎且有完整临床资料的6例患儿的临床表现、病理改变、疗效及预后进行回顾性分析。结果(1)临床表现及实验室检查:以血尿为首发症状2例,以血尿和蛋白尿为首发症状4例;其中表现为肾炎综合征3例,肾病综合征1例;6例患儿补体C3均降低;补体C4均正常;(2)病理特点:6例患儿免疫荧光均可见补体C3强阳性沉积;光镜下表现为系膜增生性肾小球肾炎5例,表现为毛细血管内增生性肾小球肾炎1例;电镜下3例可见电子致密物沉积,3例未见电子致密物沉积;(3)治疗及预后;均予肾炎常规治疗,2例有新月体形成者加用糖皮质激素治疗。随访6~42个月,预后较好。结论本组C3肾小球肾炎患儿以血尿和(或)蛋白尿为主要表现,免疫荧光见C3强阳性沉积,光镜表现多以系膜增生性肾小球肾炎为主,电镜可表现为电子致密物沉积,短期预后较好。     

婴幼儿227例肾穿刺及临床病理学特点

目的 分析婴幼儿肾脏疾病临床病理的特点及婴幼儿肾穿刺安全进行的方法和意义.方法 对临床诊断为肾脏疾病的227例婴幼儿进行经皮肾穿刺活检,肾脏病理组织分别进行光镜、免疫荧光和电镜观察.光镜标本做苏木精-伊红(HE)、过碘酸雪夫反应(PAS)、六胺银(PASM)和Masson染色,免疫荧光检测其肾组织中的IgG、IgM、IgA、C3、C4、C1q、Fibrin,204例标本同时行电镜检查.结果 所有患儿肾穿刺均获成功,术后无明显并发症.有肾穿刺适应证的227例婴幼儿肾脏疾病中最常见的是肾病综合征(38.3%)、孤立性血尿(37.0%)和急性肾炎综合征(9.3%),继发性肾脏疾病相对较少(5.3%).肾脏病理类型中最常见的是系膜增生性肾小球肾炎(62.6%)、IgA肾病(8.4%)和局灶节段性肾小球硬化(5.7%).87例肾病综合征病理类型最常见的是系膜增生性肾小球肾炎(50例)、微小病变(11例)和局灶节段性肾小球硬化(9例);84例孤立性血尿病理类型最常见的是系膜增生性肾小球肾炎(68例)和IgA肾病(9例).结论 在不盲目扩大适应证的基础上,安全有效地进行婴幼儿肾穿刺,可以提高婴幼儿肾脏疾病的诊治水平.     

高原地区肾脏疾病患儿肾脏活体组织穿刺术及病理分型

目的通过对高原地区和平原地区肾小球疾病患儿进行肾脏活体组织穿刺术,探讨高原地区与平原地区肾脏疾病患儿病理类型分布是否存在区别。方法采用随机对照研究方案,将高原地区22例肾病患儿和平原地区22例肾病患儿进行肾活体组织穿刺术,分析高原地区和平原地区儿童肾病病理类型分布特点。其病理分型标准参照WHO 1982年的分类及改良的1995年肾小球病病理学分类标准。结果 1.高原地区肾小球疾病患儿与平原地区肾小球疾病患儿病理类型分布上有区别,高原地区以继发性肾小球疾病为多发,占所有肾活检病例的57.7%;而平原地区以原发性肾小球疾病为多见,占所有肾活检病例的85.0%。2组病理类型比较差异有统计学意义(P<0.001)。2.高原地区9例原发性肾小球疾病患者中,系膜增生性肾小球肾炎和IgA肾病各2例,各占9.5%;微小病变型肾病、局灶-节段性病变、毛细血管内增生性肾小球肾炎、系膜毛细血管性肾小球肾炎、新月体性肾小球肾炎各1例,各占4.7%;本组病理类型中无硬化性肾小球肾炎。高原地区继发性肾小球疾病病理类型中,以紫癜性肾炎为多见,其次为乙型肝炎病毒相关性肾炎,狼疮性肾炎。结论高原地区与平原地区肾病患儿病理类型分布存在区别;高原地区继发性肾小球疾病患病率高于平原地区继发性肾小球疾病患病率。     

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。     

儿童紫癜性肾炎和IgA肾病临床病理对比分析

目的：探讨儿童紫癜性肾炎（HSPN）和IgA肾病在临床和肾脏病理上的异同。方法：回顾性分析103例HSPN患儿和61例IgA肾病患儿的临床资料和肾脏病理资料。结果：HSPN患儿与IgA肾病患儿在年龄、性别及病程方面比较差异无统计学意义（P>0.05）。与HSPN患儿比较,IgA肾病患儿临床分型更重,且更易出现肉眼血尿,差异有统计学意义（P<0.05）。IgA肾病患儿的血肌酐及胆固醇水平较HSPN患儿增高,差异有统计学意义（P<0.05）。光镜病理显示HSPN患儿较IgA肾病患儿易出现纤维蛋白原相关抗原沉积,而IgA肾病患儿较HSPN患儿易出现补体C3沉积,且间质纤维化、肾小管管型、肾小管炎症细胞浸润在IgA肾病患儿更常见（P<0.05）。结论：儿童HSPN和IgA肾病在临床表现、肾脏病理等方面存在较大差异,这些差异不支持两者为同一疾病的假说。     

Pubertal growth and development and prenatal and lactational exposure to polychlorinated biphenyls and dichlorodiphenyl dichloroethene

OBJECTIVES: Polychlorinated biphenyls (PCBs) and dichlorodiphenyl dichloroethene (DDE) are ubiquitous toxic environmental contaminants. Prenatal and early life exposures affect pubertal events in experimental animals. We studied whether prenatal or lactational exposures to background levels of PCBs or DDE were associated with altered pubertal growth and development in humans.Study design: Follow-up of 594 children from an existing North Carolina cohort whose prenatal and lactational exposures had previously been measured. Height, weight, and stage of pubertal development were assessed through annual mail questionnaires. RESULTS: Height of boys at puberty increased with transplacental exposure to DDE, as did weight adjusted for height; adjusted means for those with the highest exposures (maternal concentration 4+ ppm fat) were 6.3 cm taller and 6.9 kg larger than those with the lowest (0 to 1 ppm). There was no effect on the ages at which pubertal stages were attained. Lactational exposures to DDE had no apparent effects; neither did transplacental or lactational exposure to PCBs. Girls with the highest transplacental PCB exposures were heavier for their heights than other girls by 5.4 kg, but differences were significant only if the analysis was restricted to white girls. CONCLUSIONS: Prenatal exposures at background levels may affect body size at puberty.     

Prevention and treatment of overweight and obesity in children and adolescents

Increasing numbers of obese children and adolescents all over the world demand an investment in the primary and secondary prevention of obesity and overweight in this age group. The goal of preventive measures in children is to avoid the negative short- and long-term health problems associated with obesity. Primary prevention aims at establishing a healthy, active lifestyle and keeping children and adolescents within a range of body weight which is considered to be healthy. Constant availability and affordability of palatable and energy-dense food in the affluent society of the western world demands preventive strategies. Universal or public health prevention seems to be the most suitable form because several other cofactors of morbidity and mortality of affluent societies can also be prevented. However, in most European countries there is a lack of awareness of the necessity of prevention programmes, not only among the general population but also among the medical society. More awareness and consciousness to the problem of obesity must be generated in order to lead to effective therapeutic programmes. For those children and adolescents who are already obese, secondary prevention is mandatory. Therapeutic intervention programmes for the obese aim at long-term weight maintenance and normalisation of body weight and body fat. They have to modify eating and exercise behaviour of the obese child and establish new, healthier behaviour and lifestyle. Treatments programmes must include behavioural components in order to permanently change nutrition and physical exercise of the obese children and adolescents. However, long-term results of treatment programmes in European countries are scarce and the reported results, even of multidisciplinary regimens, are not impressive. CONCLUSION: In most European countries there is an urgent need not only for a growing awareness of the problem of obesity in children and adolescents but also for development of new comprehensive approaches in treating this group.     

Prevention and treatment of overweight and obesity in children and adolescents

Increasing numbers of obese children and adolescents all over the world demand an investment in the primary and secondary prevention of obesity and overweight in this age group. The goal of preventive measures in children is to avoid the negative short- and long-term health problems associated with obesity. Primary prevention aims at establishing a healthy, active lifestyle and keeping children and adolescents within a range of body weight which is considered to be healthy. Constant availability and affordability of palatable and energy-dense food in the affluent society of the western world demands preventive strategies. Universal or public health prevention seems to be the most suitable form because several other cofactors of morbidity and mortality of affluent societies can also be prevented. However, in most European countries there is a lack of awareness of the necessity of prevention programmes, not only among the general population but also among the medical society. More awareness and consciousness to the problem of obesity must be generated in order to lead to effective therapeutic programmes. For those children and adolescents who are already obese, secondary prevention is mandatory. Therapeutic intervention programmes for the obese aim at long-term weight maintenance and normalisation of body weight and body fat. They have to modify eating and exercise behaviour of the obese child and establish new, healthier behaviour and lifestyle. Treatments programmes must include behavioural components in order to permanently change nutrition and physical exercise of the obese children and adolescents. However, long-term results of treatment programmes in European countries are scarce and the reported results, even of multidisciplinary regimens, are not impressive. Conclusion In most European countries there is an urgent need not only for a growing awareness of the problem of obesity in children and adolescents but also for development of new comprehensive approaches in treating this group.