环磷酰胺静脉冲击疗法治疗(氵弥)漫增殖型狼疮肾病疗效观察

狼疮肾是SLE重要并发症之一。如治疗不当常发展至终未性肾病(ESRD)。本文报告使用CTX静脉冲击疗法(CTX-PT)治疗(氵弥)漫增殖型狼疮肾病共7例。平均年龄7.6岁。CTX总量<150mg/kg。3例完全缓解,1例部份缓解,1例无效,经血浆置换术后缓解,1例发展为慢性肾功能衰竭,1例继续治疗中。作者结合LN临床及病理讨论CTX-PT的作用机制及疗效。     

狼疮相关性微小病变性肾病2例报告并文献复习

目的 提高临床医师对小儿狼疮性肾炎多样性的认识,加强对小儿狼疮相关性微小病变性肾病的了解.方法 回顾性分析本院收治的2例狼疮相关性微小病变性肾病患儿的临床资料、病理特点及诊疗经过,并结合文献进行分析.结果 2例患儿均为女童,年龄分别为12岁和13岁,病程为1-2个月.均符合典型的SLE诊断标准.狼疮活动的同时肾脏损害表现均以肾病综合征为主,但肾脏病理表现为微小病变.其病理表现为:光镜下2例患儿肾脏均未见明显改变;免疫荧光镜检例1显示阴性,例2仅见系膜区节段微量沉积,均无明显的肾小球毛细血管壁沉积;电镜均偶见少量的电子致密物沉积,但突出表现为弥散性肾小球上皮细胞足突融合.2例患儿经激素或联合免疫抑制剂治疗后尿蛋白均转阴.结论 临床医师在工作中应对狼疮活动并同时出现肾病蛋白尿的患儿进行认真分析,肾活检病理非常必要,对于认识新的狼疮性肾炎类型,提高临床医师对狼疮性肾炎的发病机制的了解,以及正确诊断、治疗极为重要.     

抗心磷脂抗体与儿童系统性红斑狼疮及狼疮性肾炎相关性研究

目的探讨抗心磷脂抗体(ACA)与儿童系统性红斑狼疮(SLE)及狼疮性肾炎(LN)的相关性。方法选取2005年4月至2013年10月住院治疗116例SLE患儿,收集并分析患儿的临床资料。结果 116例SLE患儿中,男20例、女96例;ACA-Ig M、Ig G或Ig A的阳性率分别为37.1%、35.3%、26.7%,阳性率差异无统计学意义(P>0.05)。116例SLE患儿中LN 75例,非LN 41例,LN与非LN患儿之间ACA-Ig M、Ig A阳性率差异无统计学意义(P均>0.05);而LN患儿ACA-Ig G阳性率(42.7%)高于非LN患儿(22.0%),差异有统计学意义(P=0.026)。男性和女性患儿间,LN及ACA各亚型比例的差异无统计学意义(P均>0.05)。结论各亚型ACA对于SLE的诊断价值无差别,但ACA-Ig G阳性提示易并发LN。     

儿童期系统性红斑狼疮与巨噬细胞活化综合征

在儿童,巨噬细胞活化综合征是风湿性疾病(特别是全身型幼年特发性关节炎)或炎症性疾病严重的、有时是致死性的并发症。然而,系统性红斑狼疮合并巨噬细胞活化综合征的临床报告多见于成人。文章重点对巨噬细胞活化等相关概念、感染致病、临床识别和治疗予以介绍。     

Immunological Profile in Children with Minimal Change Nephrotic Syndrome

ABSTRACT. Peripheral blood from patients with active stage of minimal change nephrotic syndrome (MCNS) was examined for concanavalin A (ConA)-inducible suppressor T cell activity, proliferative response to phytohemagglutinin (PHA) and in the autologous (AMLR) and allogeneic (MLR) mixed lymphocyte reaction, proportions of T cells with receptors for IgM (Tu) or IgG (Tγ) and the levels of serum immunoglobulin M, G and A. Six of 9 patients with MCNS studies showed deficiency of ConA-induced suppressor cell activity. In the AMLR, only one of 9 patients with MCNS demonstrated depressed proliferative response (p<0.05). In the allogeneic MLR, T cells from 5 of 9 patients with MCNS demonstrated poor proliferative response when stimulated with normal control non-T cells. Five of 9 patients with MCNS had depressed proliferative response to PHA. The proportion of total T cells, Tu cells and Ty cells in the patient group were comparable to healthy control group. Serum IgG was significantly decreased in 7 of 11 patients. This study demonstrates multiple immunological abnormalities in patients with MCNS that might play a role in its pathogenesis.     

儿童狼疮性脑病临床特征

目的探讨儿童狼疮性脑病(NPSLE)的临床特点、辅助检查及治疗。方法对11例NPSLE患儿的临床资料进行分析。结果11例NPSLE患儿均有不同程度的头痛症状,意识障碍及失语各1例,抽搐、记忆力减退和共济失调各3例,出现病理反射及脑膜刺激征各4例。血清学均呈狼疮活动表现。3例脑脊液常规无异常,寡克隆抗体明显升高。其中10例行头颅CT检查,异常9例;10例患儿行脑电图检查,正常1例,且与CT无交叉。经个体化综合治疗,11例患儿神经精神症状均有不同程度的改善。结论系统性红斑狼疮累及神经系统症状最常表现为头痛。目前NPSLE主要根据临床表现作出诊断,血清学、影像学及脑电图检查有助于狼疮性脑病的诊断。对NPSLE的治疗强调个体化的综合治疗。     

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??Objective??To observe the incidence??clinical types??and prognosis of neuropsychiatric systemic lupus erythematosus??NPSLE?? in children??and to probe the relationship between the onset and the relacted factors. Methods??
The clinical data of 87 children with SLE treated in Children’s Hospital of Shanghai from Jan. 2002 to Dec. 2015 were analyzed retrospectively. The patients were divided into NPSLE group and non-NPSLE group. Collect the data of the patients??including the course of the disease??activity index??SLEDAI scores????blood sedimentation??the values of serum complement and the positive rate of dsDNA??anti-ribosomal P-protein autoantibody and anticardiolipin antibody in autoantibodies??and make a comparison and statistical analysis. Results??Nervous system injury occurred in 23 cases??with an incidence rate of 26.4%??and the average course of the disease was 3??11 months. Most of 23 cases had headache. Most of the patients presented SLE activity in serology. Totally 39 cases of all patients had abnormal cranial imaging findings. Electroencephalogram was performed in 21 patients??which presented 12 abnormalities. There were no statistical differences between patients with and without NPSLE in the disease course??blood sedimentation??the values of serum complement??the positive rate of dsDNA or activity index. Clinical symptoms were improved in all 39 patients after treatment??but 2 patients died of heart failure in follow-up. Conclusion??Neuropsychiatric manifestation in children with SLE is common??and it can happen at any stage of the disease. NPSLE in children presents different kinds of symptoms. Early diagnosis and treatment can improve the short-term prognosis. Psychiatric symptoms may also occur during therapy.     

Serum levels of vascular endothelial growth factor in children and adolescents with systemic lupus erythematosus

The angiogenic cytokine vascular endothelial growth factor (VEGF) may have a role in the pathogenesis of collagen diseases. We aimed to assess its serum levels in children and adolescents with systemic lupus erythematosus (SLE) and to elucidate its correlation with clinical features, laboratory parameters, and the overall disease activity. This study comprised 25 children and adolescents with SLE and 30 healthy controls. Disease activity was evaluated by SLE disease activity index (SLEDAI) score. Laboratory investigations included complete blood count, erythrocyte sedimentation rate (ESR), urine analysis, 24-h total urinary protein, assay of serum creatinine, ANA, anti-DNA, complement component C3, lupus anticoagulant, and VEGF. Serum levels of VEGF were significantly increased in SLE patients (579.5 +/- 184.7 pg/ml) when compared with controls (113.2 +/- 30.8 pg/ml) (p < 0.0001). VEGF serum levels were significantly increased in patients having renal involvement and neurologic symptoms than those who did not have them (p < 0.0001, p < 0.005, respectively). Serum levels of VEGF were higher in patients with antiphospholipid syndrome, vasculitis, and skin symptoms than those without, but the difference did not reach statistical significance. Meanwhile, they were similar in patients with and without arthritis (p > 0.05). VEGF serum levels were not correlated to age; inversely correlated to platelet count, serum C3 level; and positively correlated to ESR. SLEDAI score was positively correlated to VEGF serum level (r = 0.86, p < 0.0001). VEGF may be relevant to SLE pathogenesis. Its concentration seems to be a marker of SLE activity, which could help in disease monitoring and planning of treatment.     

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??Objective??To analyse the positron emission computed tomography??PET/CT?? findings of neuropsychiatric systemic lupus erythematosus??NPSLE?? in children. Methods??Patients diagnosed with NPSLE treated at Department of Pediatrics??Peking Union Medical College Hospital from April 2012 to February 2014 were recruited. PET/CT using F-18-labelled fluorodeoxyglucose??18F-FDG?? was performed in patients with NPSLE??and the results were analyzed. Results??A total of 19 patients received 18F-FDG PET/CT and 13 of them were females. Age was from 7to14 years and median age was 11.3 years. The most common neuropsychiatric manifestation was headache??8/19???? followed by seizure??7/19???? cognition dysfunction??2/19????psychiatric disease??1/19?? and ataxia??1/19??. All patients under MRI examination??and only 4 of them were abnormal. 18F-FDG PET/CT revealed significant glucose hypometabolism in all patients. The temporal lobe??17/19?? was the most susceptible brain regions??followed by the parietal lobe??16/19????occipital lobe??14/19?? and frontallobe??10/19??. In contrast??glucose hypermetabolism was detected in basal ganglia??8 patients?? and thalamus??3 patients??. Conclusion??PET reveals significant glucose hypometabolism with or without local hypermetabolism in patients with NPSLE.     

Intracranial venous thrombosis and pulmonary embolism with antiphospholipid syndrome in systemic lupus erythematosus

Abstract : The presence of antiphospholipid antibodies is associated with arterial and venous thrombosis. A 14-year-old girl, with systemic lupus erythematosus (SLE), developed headache and cough and was found to have intracranial venous sinus thrombosis with secondary pulmonary embolism associated with antiphospholipid syndrome. Clinical and radiological improvement occurred with anticoagulation therapy. Because SLE is commonly associated with antiphospholipid antibodies, thromboembolic events should be considered in the differential diagnosis of both cough and headache in children with SLE.     

小儿系统性红斑狼疮的特点

目的了解小儿系统性红斑狼疮(SLE)的特点。 方法对85例SLE儿的临床资料进行分析。 结果85例中起病年龄10a以上者66例(77．6％),男女比为16．1,12例(14．1％)家族中有结缔组织病史。最常见的表现是抗核抗体(ANA)阳性(91．8％)、血沉增快(90．6％)、肾脏受累(82．3％)、发热(82．3％)、低补体血症(81．2％)、蝶形红斑(69．4％)、关节症状(62．3％)及血液系统损害(62．3％)。18例(21．2％)起病时表现为单一系统损害。6例(7．0％)肾损害始终为唯一临床表现。经激素联合免疫抑制剂治疗,随访0．5～23a,临床缓解或病情波动者49例,无1例进入慢性肾功能衰竭,死亡9例,失访27例。 结论本病临床表现多样,肾损害发生率高,早期正规治疗疗效显著。     

A 10 year review of systemic lupus erythematosus in Singapore children

A 10 year retrospective analysis of the clinical features and survival of 24 Singapore children with systemic lupus erythematosus was made. The female to male sex ratio was 11:1. The median age at diagnosis was 9.3 years (range: 3.5–17.6 years), and the median duration of follow-up was 3.6 years (range: 3 months - 10 years). The common modes of presentation were prolonged fever and malar rash (both 46%). Renal involvement (71 %) was frequent. There were six deaths, three from chronic renal failure, two from infection, and one from carditis. The overall survival at 5 years was 0.800 (s.e.m. = 0.090), and at 10 years 0.698 (s.e.m. = 0.103). The survival for lupus nephritis was 0.727 at 5 years (s.e.m. = 0.116), and 0.586 at 10 years (s.e.m. = 0.130). Although the 5 year survival rate is comparable with other series, there were more deaths after the first 5 years, and morbidity from the disease as well as from therapy was considerable.     

Dysfunction of the Monocyte-Macrophage System in the Idiopathic Minimal Change Nephrotic Syndrome

ABSTRACT. We studied the function of phagocytes and the distribution of lymphocyte subpopulations in 23 patients with Idiopathic Minimal Change Nephrotic Syndrome. All the patients were in relapse at the time of the study. The latter was performed before specific therapy was started. Our control group consisted of 26 normal children who were studied while undergoing routine analysis prior to plastic surgery. Polymorphonuclear leukocytes from the patients showed no alterations in their ability to ingest and to kill Candidas. On the contrary, peripheral blood monocytes had a normal phagocytic function with a decreased candidacidal activity when compared to normal controls (p<0.001). No correlation was found between serum immunoglobulin levels and the monocyte lytic function. The absolute number of B lymphocytes was significantly increased     

儿童狼疮性肾炎的性别和临床免疫学特征

目的探讨儿童狼疮性肾炎的性别、临床和免疫学特征。方法将71例女性，6例男性红斑狼疮患者的临床、血清免疫学指标和肾脏损害进行比较。结果女性红斑狼疮患者关节炎、白细胞减少比男性常见，而男性盘状红斑较常见，且肾损害比女性严重。结论红斑狼疮临床表现、血清免疫学特征及发病率与性别有关，但男性狼疮性肾炎预后可能较差。     

Liver disease in association with neonatal lupus erythematosus

Abstract  This report describes a patient with neonatal lupus erythematosus (NLE) in whom there was multisystem involvement including neonatal hepatitis. The hepatitis, defined pathologically as a giant cell hepatitis, presented with severe cholestasis, was unrelated to other known causes of neonatal hepatitis and resolved spontaneously by 6 months of age. Both mother and infant were positive for Sjogren syndrome A + B antibodies (SS-A [Ro] and SS-B [La]) as well as having high titres of antinuclear antibody (ANA). Three of the four cases described in the one previous report of this association were also ANA positive. This antibody may be a marker for the development of the hepatitis. Maternal and infant ANA status should be determined in cases labelled as idiopathic neonatal hepatitis to exclude undiagnosed maternal SLE as a cause of neonatal cholestasis.     

吗替麦考酚脂治疗增殖性狼疮性肾炎的Meta分析

目的 评价吗替麦考酚脂(MMF)治疗增殖性狼疮性肾炎（PLN）的疗效和安全性。方法 检索Cochrane图书馆、PubMed、EMBASE、中国生物医学文献光盘数据库、中国期刊全文数据库和中国维普数据库等中关于MMF治疗PLN疗效和安全性的RCT文献,检索时间均从建库至2009年6月30日,由3名系统评价员进行文献资料提取和质量评价。评价指标为终末期肾病(ESRD)发生率、病死率、完全缓解率、部分缓解率、总缓解率、复发率和不良反应（闭经、感染、带状疱疹、WBC减少和胃肠道反应等）发生率。采用RevMan 5．0软件进行Meta分析。结果 共检索到868篇文献,符合纳入标准的14篇RCT文献（n＝1 032）进入Meta分析。文献质量评价7篇为B级,7篇为C级。Meta分析结果显示：①MMF组完全缓解率（OR＝2．16,95％CI：1．31～3．55）、总缓解率（OR＝1．51,95％CI：1．10 ～2．07）显著高于环磷酰胺（CTX）组;MMF组闭经发生率（OR＝0．16,95％CI：0．05～0．50）、感染发生率（OR＝0．38,95％CI：0．23～0．61）和WBC减少发生率（OR＝0．43,95％CI：0．27～0．71）均显著低于CTX组;两组病死率、ESRD发生率、部分缓解率、胃肠道反应发生率和带状疱疹发生率差异均无统计学意义。②MMF组病死率、ESRD发生率 、完全缓解率、部分缓解率、总缓解率、复发率、SCr倍增发生率 、胃肠道反应发生率、带状疱疹发生率与CTX/硫唑嘌呤（AZA）组差异均无统计学意义;MMF组闭经发生率（OR＝0．20,95％CI：0．07～0．61)、感染发生率（OR＝0．46,95％CI：0．22～0．99)和WBC减少发生率（OR＝0．12,95％CI：0．03～0．52) 显著低于CTX/AZA组。结论 现有的证据显示,MMF疗效与CTX或CTX/AZA相近,安全性方面优于CTX或CTX/AZA。有关PLN患儿MMF的疗效需进一步的RCT研究予以明确。     

Failure of clinical and laboratory characteristics to differentiate mesangial proliferative from minimal-change nephrotic syndrome

Twelve clinical and laboratory characteristics of nephrotic syndrome were compared in 24 children with biopsy-proven mesangial proliferative glomerulonephritis (MesPGN) and 17 children with biopsy-proven minimal-change nephropathy (MCNS). The objective of the study was to determine if these characteristics alone, without renal biopsy, could be used to differentiate the two histopathologic entities. Sex, urinary protein level and IgM immunofluorescence were found to be significantly different in the two groups. Discriminant analysis produced two formulae which gave a discriminant rate of 79% for MesPGN and 76% for MCNS. We conclude that the clinical and laboratory characteristics studied could not differentiate MesPGN from MCNS.     

Nephrotic syndrome in Kawasaki disease

Abstract Renal manifestations in Kawasaki disease are rare, and nephrotic syndrome in this disorder has not been described previously. We report a 3 month old infant with Kawasaki disease, presenting with nephrotic syndrome which responded to steroid therapy. The patient, however, developed coronary aneurysms and died of acute myocardial infarction.