风湿病及结缔组织病

()0 3354抗中性粒细胞桨抗体与儿童风湿性疾病肾损伤的关系/马宏…//中国实用儿科杂志一2000,15(3)一160一162 检测105例患儿于疾病急性期血清抗中性粒细胞浆抗体(ANCA),其血清ANCA阳性率为:系统性红斑性狼疮7忍3、过敏性紫癫巧/64、混合性风湿性疾病6/18。其中核周型即P一ANCA     

过敏性紫癜患儿PBMC表面粘附分子表达、血清抗中性粒细胞胞浆抗体检测情况研究

目的探讨过敏性紫癜(HSP)患儿外周血单个核细胞(PBMC)表面CD54、CD11a、CD11b、CD18表达和血清抗中性粒细胞胞浆抗体(ANCA)阳性率,以及两者在HSP发病中的关系。方法用标记的链霉卵白素-生物素免疫细胞化学染色法检测PBMC表面粘附分子(AMs)CD54、CD11a、CD11b、CD18的表达情况,用间接免疫荧光法(IIF)检测ANCA。结果HSP患儿PBMC表面表达CD54、CD11a、CD11b、CD18的阳性细胞百分率较对照组普遍上调,P<0.001;紫癜性肾炎(HSPN)患儿CD54表达较单纯HSP组升高;ANCA阳性检出率HSP组高于对照组,HSPN组高于单纯HSP无肾脏损害组,差异有显著性,P<0.01;ANCA阳性组的CD54、CD11a、CD11b较ANCA阴性组增高,差异有显著性,P<0.05或<0.01。结论AMs在HSP的发病中发挥一定的作用;HSP患儿血清ANCA阳性率升高,提示ANCA可能参与了HSP的致病过程;HSPN患儿ANCA阳性率明显升高,有助于诊断HSP是否伴肾脏受累;ANCA阳性者AMs高于ANCA阴性者,说明ANCA与AMs在HSP发病过程中可能起协同作用。     

抗中性粒细胞抗体在川崎病诊断中的价值

目的：探讨抗中性粒细胞抗体（ANCA)在川崎病诊断中的价值。方法：检测30例典型川崎病患儿和16 例不完全川崎病患儿急性期、恢复期血清ANCA,并随机选取25例健康儿童作为对照组。同时对川崎病患儿急性期行超声心动图(UCG)检查。结果：川崎病患儿急性期血清ANCA阳性率为65%,其中不完全川崎病组和典型川崎病组血清ANCA阳性率分别为69％、63％,均明显高于对照组(P＜0.01）。川崎病患儿恢复期血清ANCA阳性率显著低于急性期（33％ vs 65％,P＜0.05）。川崎病患儿急性期血清ANCA阳性率显著高于UCG的阳性检出率(P＜0.01)。ANCA阳性患儿冠脉病变发生率明显高于ANCA阴性患儿（43％ vs 13％,P＜0.05)。结论：血清ANCA可以作为一项早期诊断川崎病的参考指标和发生冠脉损害的一项实验室预警指标。     

过敏性紫癜患儿血清Tim-1水平及临床意义

目的 检测过敏性紫癜(HSP)患儿不同临床类型、不同临床疾病期血清中Tim-1水平,探讨Tim-1与HSP发生的关系.方法 不同临床类型HSP患儿共35例,分别在急性期和恢复期空腹采静脉血2ml,EUSA法测定其血清中Tim-1水平;20例无过敏性疾病史的健康体检儿童作为对照.结果 HSP患儿急性期血清中Tim-1含量较正常对照组显著增高(P＜0.001),HSP腹型和肾型急性期血清中Tim-1水平显著高于HSP单纯型(P＜0.01),而HSP腹型和肾型急性期血清中Tim-1水平差异无统计学意义.结论 Tim-1与HSP发生及临床类型有关,检测HSP患儿血清中Tim-1水平有助于判断HSP患儿的严重程度及复发可能.     

儿童抗中性粒细胞胞浆抗体相关性小血管炎的临床病理特点分析

目的探讨儿童抗中性粒细胞胞浆抗体(ANCA)相关性小血管炎的临床表现、血清ANCA和肾脏病理检查特征以及其治疗转归情况.方法采用间接免疫荧光定性检查血清ANCA,以酶联免疫吸附分析(ELISA)定量测定患儿血清抗蛋白酶3(PR3)和抗髓过氧化物酶(MPO)抗体活性,并同时进行肾穿刺病理检查,对5例ANCA相关性小血管炎(ASV)患儿的临床表现、血清ANCA与肾活检结果及治疗转归情况进行分析.结果 (1) 5例ASV患儿占同期肾脏疾病住院患儿的0.25%,发病年龄在8～12岁之间,平均10岁6个月,均为女孩.(2) 5例ASV患儿血清胞浆型ANCA阴性,抗PR3定量都在正常范围内,环核型ANCA均阳性,抗MPO 98～242 kEU/L,平均154.5 kEU/L(正常<12.7 kEU/L).(3) 5例患儿均为ANCA相关性小血管炎中的显微镜下多动脉炎,肾脏病理改变均为广泛新月体形成,并有毛细血管袢纤维素样坏死,免疫荧光检查有不同程度的C3沉积,除1例IgA和IgM沉积较强外,免疫球蛋白沉积均较弱.电镜下显示沉积部位主要在内皮下,1例在GBM内也有沉积.(4) 5例患儿中有3例在确诊后1年内死亡,2例经甲泼尼龙和环磷酰胺冲击后,肾功能恢复至正常,分别随访1年和5年,留有血尿和少量蛋白尿.结论儿童ASV女孩占绝大多数,以环核型ANCA为主,较成人更易出现肾功能衰竭,预后也更为严峻,血清ANCA定性定量检查与肾活检是诊断儿童ASV的关键.     

过敏性紫癜患儿血管内皮生长因子变化的意义

目的探讨过敏性紫癜(HSP)患儿血清血管内皮生长因子(VEGF)水平的变化及其在HSP发病中的作用。方法采用双抗体夹心ELISA检测20例急性期HSP患儿、15例恢复期HSP患儿及15例正常儿童血清VEGF水平。结果急性期HSP患儿血清VEGF水平(533.85±127.63)ng/L,明显高于恢复期(160.47±37.39)ng/L及正常对照组(68.93±19.16)ng/L,差异具有显著性(F=158.86P<0.01),3组间作两两比较差异均具有显著差异(P均<0.01)。结论HSP患儿急性期血清VEGF水平明显升高,提示VEGF可能参与HSP的发病过程,可能在HSP发病机制中具有重要意义。     

过敏性紫癜患儿血清可溶性血管细胞黏附分子-1及可溶性细胞间黏附分子-1表达的意义

目的 检测过敏性紫癜(HSP)患儿血清可溶性血管细胞黏附分子-1(sVCAM-1)及可溶性细胞间黏附分子-1(sICAM-1)表达,并探讨其临床意义.方法 酶联免疫吸附法(ELISA)测定53例HSP患儿(其中急性期37例,恢复期16例)及25例健康儿童血清sVCAM-1、sICAM-1水平,分别比较其急性期和恢复期及有或无肾损害HSP患儿上述因子表达水平.结果 HSP急性期患儿血清sVCAM-1和sICAM-1水平明显高于恢复期和健康对照组(Pa＜0.01);肾损害组血清sVCAM-1、sICAM-1明显高于无肾损害组(Pa＜0.01).结论 sVCAM-1、sICAM-1参与HSP病理生理过程,与疾病分期相关;血清sVCAM-1、sICAM-1水平增高可作为儿童HSP肾损害的判断指标之一.     

血清半胱氨酸蛋白酶抑制剂C在过敏性紫癜患儿早期肾功能损害诊断中的价值

目的 探讨过敏性紫癜(HSP)患儿肾损害早期诊断的实验室指标,为HSP患儿肾损害的早期诊断提供依据.方法 选取45例血清Cr正常HSP患儿作为观察组,30例健康儿童作为健康对照组.抽血后采用酶联免疫吸附法(ELISA)测定血清半胱氨酸蛋白酶抑制剂C(Cyst-C).同时对观察组行尿常规检测.结果 HSP组血清Cyst-C显著高于健康对照组(P＜0.01),HSP患儿尿常规阳性率33.3%(15/45),而血清Cyst-C阳性率71.1%(35/45),二者间有统计学差异(P＜0.01).结论 通过检测HSP患儿血清Cyst-C,有助于发现血Cr正常的早期肾功能损害.     

过敏性紫癜患儿血清sE-选择素和sICAM-1水平的变化及其意义

目的研究过敏性紫癜(HSP)患儿血清sE-选择素和可溶性细胞间黏附分子1(sICAM-1)水平变化,探讨其在HSP发病机制中的作用。方法采用酶联免疫法检测60例HSP患儿、25例健康对照组儿童血清sE-选择素、sICAM-1水平。结果 HSP患儿血清sE-选择素和sICAM-1水平均高于对照组,差异有显著性(P<0.05)。sE-选择素水平在腹型组显著高于非腹型组(P<0.05);sICAM-1水平在不同临床表现组间差异无显著(P>0.05)。结论 HSP患儿血清sE-选择素、sICAM-1水平显著升高,sE-选择素水平在腹型组显著高于非腹型组,提示sE-选择素可能参与HSP急性期血管内皮细胞炎性以及脏器损伤的机制。     

过敏性紫癜患儿血清半胱氨酸蛋白酶抑制剂C和尿微量清蛋白检测的临床意义

目的：探讨血清半胱氨酸蛋白酶抑制剂C(Cyst-C)和尿微量清蛋白(mA1b)在过敏性紫癜（HSP）患儿早期肾损害中的检测意义。方法：选取48例血清肌酐正常的HSP患儿作为观察组,31例健康儿童作为对照组。均抽血后采用碱性苦味酸法检测血清肌酐（Scr）、酶联免疫吸附方法（ELISA)测定血清Cyst-C、免疫比浊法检测尿mA1b,同时对观察组做尿常规检测。并且随诊患儿1个月后复查血Cyst-C和尿mA1b含量。结果：观察组患儿急性期血清Cyst-C为2.24±0.81 mg/L,高于对照组的0.85±0.20 mg/L,急性期尿mA1b为20.04±10.32 mg/L,高于对照组的2.30±1.38 mg/L。两者比较差异有非常显著性意义（P＜0.01);HSP患儿急性期血清Cyst-C和尿mA1b与恢复期血清Cyst-C（1.70±0.30 mg/L）和尿mA1b（13.20±8.16 mg/L）比较,差异有非常显著性意义（P＜0.01);HSP患儿的尿常规异常率为33.3%(16/48),分别与尿mA1b异常率68.8%(32/48)、血清Cyst-C异常率72.9%(36/48)比较,差异有非常显著性意义（P＜0.01);HSP患儿的尿mA1b与血Cyst-C异常率两者相接近,差异无显著性。结论：血清Cyst-C联合尿mA1b检测是诊断HSP早期肾损害的灵敏指标,可作为临床早期诊断的可靠依据。［中国当代儿科杂志,2009,11（5）：346-348］     

Antineutrophil cytoplasmic antibodies (ANCA) in idiopathic pulmonary hemosiderosis

Four children were diagnosed with idiopathic pulmonary hemosiderosis (IPH), over a period of 4 years. Retrospectively, antineutrophil cytoplasmic antibodies (ANCA) were studied by indirect immunofluorescence (IIF) and ELISA in 18 sera from these patients, stored at -20°C. ANCA-positive sera, from 1/20 to 1/1, 200 dilution, were found in 3/4 of the patients, by IIF. The patient with the highest titre of ANCA died 3 months later during an acute crisis, the other two patients need a minimal dose of steroids. In one case only, a patient who is still without treatment, had no ANCA. The antibodies anti-myeloperoxidase and anti-pro-teinase-3 were negative or at border line levels. Rheumatoid factor, anti-nuclear (Hep-2), anti-endomysial, anti-reticulin and antibasement membrane antibodies were negative in all sera. The surviving patients were followed-up for more than 10 years with no systemic or renal disease appearences. The presence of serum ANCA may help to classify children with pulmonary haemorrhage and may have a prognostic value.     

Anti-neutrophil cytoplasmic antibodies in Japanese children with ulcerative colitis

Objective: To investigate the diagnostic value of anti-neutrophil cytoplasmic antibodies (ANCA) in the diagnosis of ulcerative colitis (UC) in Japanese children.
Methodology Serum samples from 23 children with UC (17 Japanese, 6 non-Japanese), 27 children with Crohn's disease (CD) (10 Japanese, 17 non-Japanese), 10 children with other diarrhoeal diseases, and 33 normal, healthy adult volunteers were assayed for ANCA using an indirect immunofluorescence technique.
Results ANCA were detected in 6/17 (35%) UC patients and 0/10 (0%) CD patients in Japanese children, and in 3/6 (50%) UC patients and 3/17 (18%) CD patients in non-Japanese children. The difference in prevalence between Japanese and non-Japanese children with UC was not statistically significant ( P >0.05). ANCA were not found in other diarrhoeal patients and volunteers.
Conclusions Although ANCA have been reported to be useful in the diagnosis of UC in adults, they may be of limited use in Japanese children. This might reflect the heterogeneity of UC.     

儿童急性T淋巴细胞白血病微小残留病检测的临床意义

目的：探讨急性T淋巴细胞白血病（T-ALL）患儿微小残留病（MRD）监测的临床指导价值。方法采用多参数流式细胞术对2006年1月1日-2008年12月31日在北京儿童医院治疗的60例T-ALL患儿在治疗不同时间点进行追踪监测,分析不同MRD水平患儿的临床特征及预后。结果诱导治疗第33 d MRD≥1×10-4组患儿易复发,P＝0.03;诱导治疗第33 d MRD＜1×10-4组患儿预计5年无事件生存率（EFS）为100％,而MRD≥1×10-4组患儿5年EFS为（62.5±7.1）％,两组之间差异有显著性（P＝0.018）。结论监测T-ALL患儿MRD水平在评估早期治疗反应、监测复发以及估计预后中具有重要临床价值。     

伴抗中性粒细胞胞浆抗体阳性狼疮性肾炎患儿临床病理特征分析

目的 分析伴抗中性粒细胞胞浆抗体（ANCA）阳性狼疮性肾炎（LN）患儿的临床及病理特征。方法 回顾性选择2003年1月至2019年12月于中山大学附属第一医院诊断的LN患儿,依据血清ANCA结果分为ANCA阳性组（n=59）和ANCA阴性组（n=454）,比较两组患儿临床表现、组织病理学、治疗缓解与预后情况。结果 ANCA阳性组白细胞降低并伴红细胞沉降率增快（P < 0.05）。两组患儿血肌酐、尿蛋白、尿红细胞差异无统计学意义（P > 0.05）。308例患儿行肾脏穿刺活检,光镜结果提示ANCA阳性组细胞纤维性新月体比例高于ANCA阴性组（P < 0.05）,免疫复合物沉积比例低于ANCA阴性组（P < 0.05）。两组患儿治疗缓解率、生存情况差异无统计学意义（P > 0.05）。结论 ANCA阳性LN患儿肾脏病理损害更重,与临床表现不完全对应平行,及时行肾脏穿刺活检至关重要。     

Diversity in presenting manifestations of systemic lupus erythematosus in children.

OBJECTIVE: To describe the diversity in presenting manifestations of systemic lupus erythematosus (SLE) in children. STUDY DESIGN: Initial clinical and laboratory manifestations of 39 children, who fulfilled >/=4 American College of Rheumatology criteria for SLE, were retrospectively analyzed. RESULTS: Median age at onset was 12 years. The male to female ratio was 1:18.5, and racial/ethnic backgrounds were white 41%, black 33%, and Hispanic 26%. Initial manifestations included musculoskeletal 74%, cutaneous 72%, constitutional 67%, neurologic 28%, renal 28%, lymphadenopathy 15%, and Raynaud's phenomenon 10%. Laboratory abnormalities at presentation to our clinic included elevated erythrocyte sedimentation rate 87%, anemia 72%, lymphopenia 59%, leukopenia 31%, proteinuria or cellular casts 44%, low C(3) or C(4) level 77%, antinuclear antibodies 97%, and anti-double-stranded DNA 95%. One third (33%) presented with features not initially suggestive of SLE. Six patients presented with unusual manifestations including parotitis, quadriplegia, chorea, severe abdominal pain, persistent cough, and dizziness. However, 85% of patients with atypical manifestations had abnormal complete blood count or urinalysis results at presentation. CONCLUSION: Presenting manifestations of SLE in children are diverse. A detailed history, thorough review of systems, complete physical examination, complete blood count, urinalysis, and a high index of suspicion help to make the correct diagnosis of SLE in patients with atypical presentations.     

Anti-neutrophil cytoplasmic antibodies in childhood systemic lupus erythematosus

The prevalence and antigen specificity of anti-neutrophil cytoplasmic antibodies (ANCA) in sera from 23 children with active systemic lupus erythematosus (SLE) were studied utilizing indirect immunofluorescence and IgG and IgM ELISA using crude neutrophil extract and purified proteinase 3, myeloperoxidase, lactoferrin, cathepsin G and elastase. ANCA were present in 69% of SLE children and consisted of IgM and IgG antibodies of variable specificities, but did not correlate with organ involvement or disease activity. It remains unclear whether they have pathogenic significance or are epiphenomena in the category of polyclonal B-cell activation. However, their presence is entirely compatible with SLE even though they have hitherto been commonly associated with other systemic vasculitides.     

CD58在儿童急性B淋巴细胞白血病中的表达及其用于微小残留病检测的可行性研究

目的 检测淋巴细胞功能相关抗原-3(CD58)在儿童急性B淋巴细胞白血病(B-ALL)中的表达情况,并探讨其用于儿童B-ALL微小残留病(MRD)检测的可行性。方法 选取2014年1~9月确诊为B-ALL的87例患儿为研究对象,同期选取20例非肿瘤、非血液病且骨髓涂片细胞形态正常的住院患儿作为对照组。应用四色流式细胞术(FCM)检测对照组及B-ALL患儿初诊时、诱导化疗15 d时骨髓标本的CD58表达水平。在诱导化疗33 d时,采用实时荧光定量聚合酶链反应(RT-PCR)和FCM对B-ALL患儿进行MRD检测。结果 87例B-ALL患儿骨髓CD58平均荧光强度(MFI)(91±33)高于对照组(14±6)(P< 0.01);其中44例B-ALL患儿CD58强表达。在诱导化疗过程中,诱导化疗15 d时B-ALL患儿骨髓CD58的表达量(105±22)与初诊时(107±26)比较差异无统计学意义(P> 0.05)。对44例CD58强表达B-ALL患儿进行MRD检测,FCM检测MRD阳性患儿9例,阴性患儿35例;RT-PCR检测MRD阳性患儿11例,阴性患儿33例,42例(95%)检测结果一致,两种检测方法检测MRD差异无统计学意义(P> 0.05)。结论 CD58在B-ALL患儿中高表达且表达稳定,可以作为B-ALL患儿MRD检测的指标。     

甲巯咪唑与抗中性粒细胞胞浆抗体阳性血管炎的关系

目的 研究抗甲状腺药物甲巯咪唑(MMI)与抗中性粒细胞胞浆抗体(ANCA)阳性血管炎的关系.方法 对33例Graves病患儿在服药前及服药后检测血ANCA.同时对我院近年来诊治的2例毒性弥漫性甲状腺肿(Graves病)患儿在服用MMI期间出现ANCA阳性血管炎进行临床病理分析.结果 2例患儿在服用MMI第5～6年出现抗中性粒细胞胞浆抗体阳性血管炎,临床出现血尿、肾功能减退,肾活检示肾血管炎改变,急性肾血管坏死伴新月体形成.经免疫抑制剂等治疗临床缓解;33例Graves病患儿服药前血ANCA均为阴性.有3例患儿分别在服用MMI第2、3个月及2年时出现ANCA阳性,阳性率9%(3/33).临床出现镜下血尿,服药后ANCA阳性率高于服药前(x2=5.3,P<0.05).3例患儿经对症治疗后镜下血尿消失,血ANCA复查转阴性.结论 MMI与ANCA阳性血管炎可能有关,可产生从轻到重的肾脏受累临床表现,采用不同的治疗方法可使症状消失.     

过敏性紫癜患儿血清高迁移率族蛋白B1表达及意义

目的 测定过敏性紫癜(HSP)患儿血清高迁移率族蛋白B1(HMGB1)水平,分析血清HMGB1与实验室指标的相关性,初步探讨HMGB1与HSP发病及肾损害的关系.方法 纳入2017年12月至2019年12月在重庆医科大学附属儿童医院初诊为HSP患儿83例,其中45例无肾脏受累(A组)、38例合并肾脏损害(B组),恢复期...