儿童Alport综合征临床与病理分析

目的：了解Alport综合征患儿的临床与病理特点。方法：回顾性分析我院2007年2月至2009年2月10例诊断为Alport综合征患儿的临床及病理资料。结果：男7例,女3例,年龄2岁至6岁7月,平均3岁2月。10例患儿中5例有明确家族史;X连锁显性遗传8例,常染色体隐性遗传2例。临床表现为孤立性血尿5例,血尿合并蛋白尿3例,大量蛋白尿1例,肾病综合征1例;肾组织病理示：8例光镜为系膜增生性肾小球肾炎,2例为局灶节段性肾小球硬化;免疫荧光均以IgM沉积为主;电镜下1例出现典型的肾小球基底膜病变。所有患儿肾脏IV型胶原α链分布异常。结论：Alport综合征临床表现多样。肾组织病理光镜下主要以系膜增生为主,免疫荧光以IgM沉积为主。电镜下基底膜病变不明显,需结合IV型胶原α链免疫荧光检测明确诊断。［中国当代儿科杂志,2010,12（3）：188-191］     

NADPH oxidase-derived reactive oxygen species involved in angiotensin Ⅱ-induced monocyte chemoattractant protein-1 expression in mesangial cells

Objective To investigate the origin of oxidative stress induced by angiotensin H (Ang Ⅱ ) in human mesangial cells and the role of reactive oxygen species ( ROS) in Ang Ⅱ -induced monocyte chemoattractant protein-1 (MCP-1) expression.Methods MCP-1 expression was determined by real time RT-PCR.ROS production was measured by DCFDA fluorescence.Nicotinamide adenine dinucleotide phosphate (NADPH) oxidase activity was examined by lucigenin chemiluminescence.p47phox and p67phox translocation was assayed by Western blot.Twenty-four male mice were randomly divided into three groups; the control,the Ang Ⅱ infusion [ Ang Ⅱ 400 ng/(kg±min) ],and the apocynin treatment.Ang Ⅱ was infused by subcutaneously osmotic minipump for 14 days.Urinary albumin and 8-isoprostane excretion were measured by ELISA.Results In cultured human mesangial cells,Ang Ⅱ induced the MCP-1 expression in a dose-dependent manner with 3.56 fold increase as compared with the control.Ang Ⅱ increased intracellular ROS production as early as 3 min with the peak at 60 min and was in a time and dose-dependent.Incubation with different dosages of Ang Ⅱ ( 1μmol/L,10μmol/L,and 100μmol/L Ang Ⅱ ) for 60 min,ROS production increased at 1.82,2.92,and 4.08 folds respectively.Ang Ⅱ-induced ROS generation was sensitive to diphenyleneiodonium sulfate (DPI,10 μmol/L) and apocynin (500μmol/L) ,two structurally distinct NADPH oxidase inhibitors.In contrast,inhibitors of other oxidant- producing enzymes,including the mitochondrial complex I inhibitor rotenone,the xanthine oxidase inhibitor allopurinol,the cyclooxygenase inhibitor indomethacin,the lipoxygenase inhibitor nordihydroguiaretic acid,the cytochrome P450 oxygenase inhibitor ketoconazole and the nitric oxide synthase inhibitor G-nitro-L- arginine methyl ester were without an effect Ang Ⅱ -induced ROS generation was inhibited by the ATI antagonist losartan (10μmol/L) but not the AT2 antagonist PD123319 (10μmol/L).Ang Ⅱ treatment induced translocation of cytosolic of p47 and p67 to the membrane.The antioxidants almost abolished Ang Ⅱ -induced MCP-1 expression.Ang Ⅱ infusion increased urinary and p67 translocation by 2.69-,2.97-,and 2.67-fold,respectively.Conclusions NADPH oxidase-derived ROS is involved in Ang Ⅱ-induced MCP-1 expression.Inhibition of NADPH oxidase alleviates Ang Ⅱ -induced renal injury.     

肿瘤患者弓形虫抗体调查

本文对１７８例肿瘤患者和２３８例健康人，采用间接血凝进行弓形虫抗体检测。结果患者组阳性２７例，感染率为１５．１６％；健康人阳性者７人，感染率为２．９４％。     

食管炎患者真菌感染的特点与药敏分析北大核心CSCD

目的探讨食管炎患者真菌感染的病原菌特点以及药敏学分析,为临床治疗提供参考依据。方法将2014年3月-2015年2月确诊为食管炎合并真菌感染的59例患者作为研究组,同期选取65例患者作为对照组,对两组患者的临床资料进行对比分析,并对研究组患者进行真菌检测和药敏分析,数据采用SPSS 19.0软件进行统计分析。结果研究组患者在患有糖尿病、应用广谱抗菌药物及强酸抑制药明显多于对照组,差异有统计学意义(P<0.05);研究组患者共分离出59株真菌,以白色假丝酵母菌、热带假丝酵母菌和克柔假丝酵母菌为主,分别占5.59%,28.81%和20.34%;对制霉菌素和两性霉素B的药物敏感性较好,其敏感率均为100.00%。结论对食管炎患者真菌感染进行真菌检测和药敏分析,以保证临床用药的规范性和合理性,有效降低了真菌继发性感染的发生,对真菌性食管炎的预防治疗工作起到积极作用。     

微小膜壳绦虫卵的孵化观察

微小膜壳绦虫 (Hymenolepis nana)又称短小绦虫 ,是鼠体内常见的寄生虫 ,亦可寄生于人体。该虫发育中除需昆虫(蚤、甲虫 )作为中间宿主外 ,也可在人、鼠体内直接孵出幼虫进而发育为成虫 ,造成人自体内重复感染。尤其在免疫力低下的人体内 ,可大量繁殖。关于该虫在终宿主体内是如何孵化 ,导致大量感染的报道较小。现将笔者试验观察到的一些现象报道如下。方法及观察结果1　解剖小鼠肠管 ,从小肠上段向下逐段剪开 ,发现虫体后挑出 ,放入生理盐水中 1～ 3d备用。用小镊子从虫体末端夹取孕节少许 ,置玻片上 ,涮出虫卵在光镜下观察。结果如下。1…     

儿童毛细血管内皮细胞增生性紫癜性肾炎的临床病理及预后分析

Objective To investigate the clinicopathological characteristics and prognosis of Henoch-Schonlein purpura nephritis with diffused endothelial cell proliferation (DEP-HSPN) in children. Methods Data of 8 DEP-HSPN cases in Nanjing Children's Hospital within recent ten years were retrospectively reviewed. The clinicopathological features, efficacy and prognosis were compared between DEP-HSPN cases and 48 cases of non-DEP-HSPN. Non-DEP-HSPN cases were divided into two groups according to the clinical classification or the pathological classification.Results (1) In DEP-HSPN, HSP developed nephritis within 4 to 15 days after the initial onset of purpuric rashes. Hematuria was present in all the 8 patients. The main clinical manifestation of DEP-HSPN was nephritic-nephrotic syndrome (4 cases), nephrotic level proteinuria (3 cases) and acute nephritic syndrome (1 case). Four cases had macrohematuria. Six cases had abdominal symptoms and two cases had arthritis. Pathology of all the cases showed grade Ⅲ-b lesion with diffused endocapillary proliferation and segmental necrotizing lesion of the capillary wall, always accompanied with intraglomerular inflammatory cell infiltration. Crescent was found in 4 cases. (2)Compared to non-DEP-HSPN grades Ⅲ, DEP-HSPN showed a shorter course of disease.Macrohematuria, heavy proteinuria, nephritic-nephrotic syndrome, and segmental necrotizing lesion of capillary wall were more common in DEP-HSPN. Compared to non-DEP-HSPN with nephrotic level proteinuria, DEP-HSPN had a lower rate of crescent. (3) Methylprednisolone pulse therapy in early stage, then prednisone combined with cyclophosphamide were used in the treatment of DEP-HSPN.After an average follow-up period of seven months, one patient showed complete remission, five showed persistent microhematuria, and two showed persistent microhematuria accompanied with minor proteinuria. No significant difference of prognosis was found between DEP-HSPN and nonDEP-HSPN. Conclusions DEP-HSPN has an acute onset. The main clinical manifestation of DEP-HSPN is nephritic-nephrotic syndrome and nephrotic level proteinuria, always accompanied with macrohematuria. Immunosuppressant treatment in the early stage of disease is effective for a short-term outcome.     

儿童C1q肾病的临床病理特点及治疗

目的 探讨儿童C1q肾病的临床、病理特点及治疗方法。 方法 回顾性分析本院8年来经肾活检确诊的23例C1q肾病患儿临床、病理和预后资料。 结果 C1q肾病占同期肾活检的原发性肾小球疾病的4.78%。23例患儿中,男15例,女8例;年龄10个月~12岁5个月,平均发病年龄（5.0±3.4）岁;肾病综合征（NS） 18例（2例伴镜下血尿）,肾病水平蛋白尿4例（1例伴镜下血尿）,单纯镜下血尿1例。1例NS起病前曾服用2周中药,发病时同时并发急性肾功能不全。3例患儿有肾脏病家族史,其中2例（肾病水平蛋白尿）为姐弟,父亲亦有蛋白尿,基因检测证实为家族性Denys-Drash综合征并发C1q肾病。1例患儿（NS）姐姐亦有大量蛋白尿（未行肾活检）。所有患儿起病时血压均正常,补体正常,抗核抗体、抗dsDNA抗体、抗Sm抗体及乙肝两对半均阴性。18例NS中13例激素耐药（72.2%）,4例激素依赖,1例激素敏感。光镜下,13例为微小病变（MCD）（其中1例伴间质性肾炎）;6例为系膜增生性肾小球肾炎（MsPGN）;4例为局灶节段性肾小球硬化（FSGS）。另9例患儿伴有不同程度的小管萎缩和间质纤维化。免疫荧光下,所有患儿均见系膜区弥漫性C1q≥2+沉积,其中伴IgG沉积18例,IgM沉积18例,IgA沉积8例,C3沉积11例,6例患儿呈“满堂亮”表现。除4例患儿电镜下未见肾小球外,其余19例中4例系膜区见电子致密物沉积。12例激素耐药（包括2例肾病水平蛋白尿者）及3例激素依赖患儿在激素治疗基础上加用静脉CTX冲击;3例激素耐药者加用环孢素A（CsA）口服;1例激素依赖患儿给予足量激素重新诱导;1例单纯镜下血尿患儿及2例Denys-Drash综合征并发C1q肾病患儿仅给予血管紧张素转换酶抑制剂（ACEI）治疗。其中1例患儿CTX冲击满疗程无效后换用CsA治疗;1例患儿CTX冲击满疗程无效后换用FK506治疗。23例患儿中,1例失访,1例治疗时间<3个月未纳入随访对象,2例Denys-Drash综合征目前不能通过药物治疗好转未纳入疗效统计,余19例中,15例完全缓解（78.9%）,2例部分缓解（10.5%）,2例无效（10.5%）。NS患儿总缓解比例94.4%（17/18）,肾病水平蛋白尿患儿总缓解比例50.0%（2/4）。病理为MCD者总缓解比例100.0%,MsPGN者缓解比例83.4%,FSGS缓解比例50.0%。随访末所有患儿血压、肾功能均正常,自身抗体均阴性,补体水平均正常。 结论 C1q肾病罕见,临床以NS或肾病水平蛋白尿为主,且往往激素耐药或激素依赖;病理以MCD为主,也可表现为MsPGN或FSGS。加用其他免疫抑制剂治疗后,MCD和MsPGN者多可获缓解,但FSGS预后欠佳。     

瘦素在儿童肾小球疾病中的变化

目的:探讨儿童肾小球疾病(肾病综合征、急性肾小球肾炎、紫癜性肾炎)血清瘦素(leptin)的变化。方法:对93例肾小球疾病患儿(排除肥胖患儿)清晨空腹测血清leptin,其中肾病综合征33例、急性肾小球肾炎31例、紫癜性肾炎29例;并测24小时尿蛋白定量、血胆固醇、血尿素氮及肌酐、尿N-乙酰-β-氨基葡萄糖苷酶(NAG)。设正常对照组26例均为健康体检儿童,清晨空腹测血清leptin。分别比较3组疾病与正常对照组之间的血leptin差异,3组疾病之间血清leptin的差异;分析血清leptin与尿蛋白定量、血胆固醇、血尿素氮及肌酐、尿NAG酶之间的关系。结果:3种疾病患儿的血清leptin都较正常对照组明显升高,有统计学意义(P<0.05),且以肾病综合征和急性肾小球肾炎组升高最明显(P<0.01);血清leptin与尿蛋白定量、血胆固醇呈正相关(P<0.05),与血尿素氮及肌酐、尿NAG酶无相关性(P>0.05)。结论:儿童肾病综合征、急性肾小球肾炎、紫癜性肾炎的血清leptin均有不同程度的升高,说明血清leptin水平可作为临床判断肾脏滤过吸收功能状态的一个间接指标,对临床治疗具有一定指导价值。