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1.
目的 探讨甲泼尼龙 (MP)冲击加口服复方环磷酰胺治疗危重狼疮的疗效。方法 总结我院近 5年收治危重狼疮 12例的临床资料 ,治疗组MP冲击加复方环磷酰胺 ;对照组MP冲击加静脉CTX冲击。结果 两组疗效无显著性差异 ,但对照组副作用大于治疗组。结论 MP冲击加复方环磷酰胺治疗危重狼疮疗效肯定 ,且副作用小于MP冲击加静脉CTX冲击  相似文献   

2.
儿童系统性红斑狼疮12例序贯治疗疗效观察   总被引:1,自引:0,他引:1  
目的应用系统性红斑狼疮活动指数(SLEDAI)和系统性红斑狼疮国际临床合作组与美国风湿病学会损害指数(DI)的方法指导儿童系统性红斑狼疮(SLE)患儿序贯疗法.方法12例儿童SLE采用序贯疗法.治疗前进行SLEDAI和DI评估.对SLEDAI和DI均存在肾脏损伤和(或)神经系统损伤的5例(Ⅰ组)采用大剂量甲基泼尼松龙(MP)冲击诱导[15~30(mg/kg)],连续或隔日,共3次,结束时口服泼尼松1mg/(kg·d)(平均40mg/d)3~4周后,予以环磷酰胺(CTX)静脉冲击治疗[8~12mg/(kg·d)],连续2d,每2周重复Ⅰ组,累计剂量≤150mg/kg,以后每3个月重复1组,全部重复剂量≤50mg/kg;同时给予羟基氯喹(HCQ)≤5mg/(kg·d).CTX维持治疗结束6~10个月后,口服雷公藤多甙(Tw)1~2mg/(kg·d)3~6个月.此间,泼尼松逐渐减量,以最小有效控制量长期维持.无肾脏损伤和(或)神经系统损伤的7例(Ⅱ组)均采用口服泼尼松1.5~2.0mg/(kg·d),临床症状控制后1个月逐渐减量,剂量减至1.0mg/(kg·d)时,加Tw(剂量同Ⅰ组,连续口服4~9个月),同时给予HCQ(同Ⅰ组),当泼尼松减量至0.5mg/(kg·d)时,可重复应用Tw3~4个月.随访期间进行DI评估.随访结束时年龄14~19岁,平均随访时间≥5年.结果Ⅰ组患儿SLEDAI>25分,5例均表现为严重的肾炎综合征或肾病综合征,其中2例同时伴中枢神经系统损伤(癫样发作和器质性脑综合征).Ⅱ组7例SLEDA17~16分.序贯治疗后,两组患儿SLEDAI明显改善,DI无增加.结论根据SLEDAI和DI结果,用不同的药物序贯疗法有较好的近期临床效果,远期疗效有待观察.  相似文献   

3.
目的:探讨狼疮肾炎(LN)患儿血中一氧化氮(NO)、脂质过氧化物(LPO)、超氧化物歧化酶(SOD)、谷胱甘肽过氧化物酶(GSH-px)和谷胱甘肽(GSH)的变化及甲基强的松龙(MP)与环磷酰胺(CTX)冲击治疗对它们的影响。方法:采用化学分析法检测18例活动期LN患儿MP和CTX冲击治疗前、后血中上述5项指标,对其中经治疗后达稳定期的16例LN患儿及健康儿童23例也检测了上述5项指标。结果:稳定期LN患儿仅GSH低于对照组(P<0.05),NO,LPO,SOD和GSH-px与正常对照组比较差异无显著性。活动期LN患儿5项指标与对照组及稳定期比较差异有显著性(P<0.05 或 0.01)。NO,LPO与抗ds-DNA抗体、24 h尿蛋白定量、尿素氮(BUN)、血肌酐(Scr)和血沉(ESR)呈显著正相关(P<0.01);与补体C3、C4、内生肌酐清除率(Ccr)呈显著负相关(P<0.05 或 0.01);而SOD,GSH-px和GSH、抗ds-DNA抗体、24 h尿蛋白定量、BUN、Scr和ESR呈显著负相关(P<0.05 或 0.01);与C3、C4、Ccr呈显著正相关(P<0.05 或 0.01)。肾功能不全组5项指标水平与肾功能正常组及对照组比较均有显著性差异(P<0.05 或 0.01)。结论:MP和CTX冲击治疗能显著降低NO,LPO水平,逽OD,GSH-px和GSH水平。NO,LPO,SOD,GSH-px和GSH可能参与了LN的发病过程,动态观察上述指标有助于判断LN的活动性、肾脏损害程度和治疗效果。  相似文献   

4.
目的 观察甲基泼尼松龙(MP)联合环磷酰胺(CTX)静脉冲击治疗重症紫癜性肾炎(HSPN)的疗效.方法 对临床表现为血尿和蛋白尿型、急性肾炎综合征型、肾病综合征型、急进性肾炎型,病理改变为Ⅲ级、Ⅳ级、Ⅵ级的18例莺症HSPN患儿,给予MP+CTX静脉冲击治疗,随访5~36个月.结果 冲击治疗后9例完全缓解,6例部分缓解,3例无效.总有效率为83.33%;治疗后24 h尿蛋白定量、尿系列微量蛋白、血尿素氮、血肌酐明显下降,治疗前后相比差异有统计学意义(P<0.05).结论 MP+CTX静脉冲击治疗重症HSPN近期疗效好,不良反应少,是临床治疗重症HSPN的重要手段.  相似文献   

5.
经颅多普勒超声诊断儿童狼疮脑病   总被引:1,自引:0,他引:1  
目的探讨经颅多普勒超声(TCD)在儿童狼疮脑病(NPLE)诊断中的意义.方法54例系统性红斑狼疮(SLE)患儿分为三组无NPLE组即SLE组(15例);NPLE组(20例);NPLE亚临床组(19例).应用TCD仪,常规探查颅内双侧大脑前、中、后动脉和颈内动脉、椎动脉及基底动脉共11条动脉;探测参数有脑动脉平均峰流速(Vm)、左右大脑半球血流速度对称性、频谱形态、血流声频、血管波动指数(PI);对NPLE组中16例TCD异常者做了动态TCD观察.结果TCD异常表现为Vm增高或下降、左右半球血流速度不对称、涡流频谱、粗糙血流声频、PI下降等.SLE组正常12例,异常3例;NPLE组正常2例,异常18例;NPLE亚临床组正常0例,异常19例.NPLE组及NPLE亚临床组TCD异常率高于SLE组(x2=32.13,P<0.01),而前两者间差异无显著性.x2检验显示NPLE组和NPLE亚临床组PI下降、Vm增高或下降、双半球血流速度差、涡流频谱、粗糙血流声频异常率均比SLE组高,P均<0.01;NPLE组与NPLE亚临床组各项参数差异均无显著性(P>0.05).结论TCD简便、无创、价优,有助于长期随访观察SLE患儿病情,为NPLE患儿脑血管早期功能性变化的检测提供了一项较敏感和特异的方法.  相似文献   

6.
目的总结对系统性红斑狼疮(SLE)并发乳糜性腹水的诊断及治疗经验。方法报道中山大学附属第一医院儿科首例SLE并发乳糜性腹水患儿的诊治经过并进行文献复习。结果患儿诊断为SLE、狼疮性肾炎(LN)、溶血性贫血。LN表现为肾病综合征型,肾穿刺活检示LNV+III(A/C),伴有持续严重腹水,腹水检查提示为乳糜性腹水,先后给予甲泼尼龙(MP)、环磷酰胺(CTX)冲击及饮食治疗,狼疮指标好转,腹水消退。查阅相关文献,其发病机制可能与SLE对淋巴网状系统的影响,低蛋白血症致肠壁水肿,淋巴管通透性增加,脂质代谢异常等因素有关。结论 SLE并发乳糜性腹水属罕见病例,对SLE合并难治性腹水,建议行腹腔穿刺腹水检查排除乳糜腹。治疗原则是以原发病变的治疗为主,配合饮食控制等保守治疗。  相似文献   

7.
Sun L  Xu H  Liu HM  Zhou LJ  Cao Q  Shen Q  Fang XY 《中华儿科杂志》2011,49(11):819-824
目的 分析儿童狼疮性肾炎(LN)的预后及其影响因素.方法 对101例LN的患儿进行回顾性分析.分为肾活检组与未肾活检组;病理分型包括A组(Ⅰ+Ⅱ型),B组(Ⅲ+Ⅳ型),C组(V型)3组;诱导期药物环磷酰胺(CTX)组与霉酚酸酯(MMF)组;临床结局缓解组(完全缓解和部分缓解)和无效组(治疗无效和死亡).用药不依从定义为诱导期CTX冲击间期大于45d或疗程不足,或MMF或其他免疫抑制剂自行停止服用1周以上.用SPSS 11.0软件中的Life-Tables分析累积生存率.结果 (1)LN患儿3年和5年的累积生存率为93.59%和87.80%;3年和5年的肾脏累积生存率为100%和91.12%.(2)单因素分析显示诱导期疾病缓解与是否肾活检、不同病理类型、不同药物诱导、治疗是否依从和发病时蛋白尿量5个因素有关;维持期疾病缓解与前4个因素有关.(3)多因素分析显示维持期与疾病缓解相关的主要因素为治疗是否依从(x2=9.818,P =0.002).用药依从性差主要发生在未行肾活检组(x2=9.569,P=0.002).(4)Ⅲ和Ⅳ型LN中,无用药不依从发生,MMF和CTX在此组中疗效无明显差异(P =0.405).结论 LN儿童疾病缓解的主要影响因素为免疫抑制剂的用药依从性.未行肾活检组患儿疾病缓解率低可能与病理类型不确定及用药依从性差有关.在Ⅲ和Ⅳ型LN中,MMF和CTX的诱导缓解疗效无差异.对尿液检查轻度异常的LN患儿应坚持行肾活检病理检查,可指导治疗、改善预后.  相似文献   

8.
目的总结儿童狼疮性肾炎(LN)的临床特征并分析其治疗效果及远期预后,探讨LN患儿进展至终末期肾脏病(ESRD)的危险因素。方法回顾性分析2003年1月至2019年12月于中山大学附属第一医院治疗并随访的343例LN患儿的一般情况、临床表现、实验室检查、治疗、随访(截至12月31日)和预后情况等临床资料。根据肾活检病理的不同分型比较其完全缓解率;根据诱导治疗6个月后缓解情况分为完全缓解组和部分缓解组比较其复发情况;按复发情况分为无复发组、蛋白尿型复发组和肾炎型复发组;按起病时血肌酐有无升高分为血肌酐正常组和血肌酐升高组。组间比较采用χ2检验,采用Kaplan-Meier生存分析计算患儿存活率及肾脏存活率,ESRD的危险因素采用COX比例风险回归模型进行分析。结果 343例LN患儿中男68例(19.8%)、女275例(80.2%),发病年龄为13.0(11.0,16.0)岁。LN患儿的肾脏表现中蛋白尿305例(88.9%)、血尿245例(71.4%);肾外表现中贫血273例(79.6%)、皮疹183例(53.4%)和发热165例(48.1%)。212例(61.8%)患儿起病时为重度狼疮活动...  相似文献   

9.
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。  相似文献   

10.
大剂量静脉注射免疫球蛋白辅助治疗狼疮肾炎的疗效观察   总被引:11,自引:1,他引:10  
目的 观察大剂量静脉注射免疫球蛋白(IVIG)治疗狼疮肾炎(LN)的疗效。方法 在激素联合环磷酰胺的基础上对观察组29例LN患儿加用IVIG治疗,并与对照组33例LN患儿比较。结果 观察组在降低LN疾病活动性指数计分、血尿及蛋白尿消失、抗核抗体(ANA)及抗ds-DNA阴转率和降低院内感染率方面明显优于对照组,未见明显副作用。结论 IVIG配合激素及环磷酰胺治疗LN是一种有效、安全的方法。  相似文献   

11.
目的:分析系统性红斑狼疮疾病活动指数2000(SLEDAI-2000)和英国狼疮评估组2004(BILAG-2004)两种评分系统在评估狼疮性肾炎(LN)患儿肾脏病变活动度方面的可行性。方法:收集159例系统性红斑狼疮(SLE)合并LN患儿的临床资料,应用 BILAG-2004和 SLEDAI-2000两种临床评分系统判断其临床疾病活动度。进行两种评分与24 h尿蛋白定量、病理指数的相关分析。用ROC曲线对两种评分系统进行评价。结果:24 h尿蛋白定量分级以中量蛋白尿居多(31.5%),病理类型以弥漫性LN(Ⅳ型)最多,占46.0%;24 h尿蛋白定量与两种评分均呈正相关(r值分别为0.36和0.39,均P<0.05);LN患者Ⅰ~Ⅳ型的活动指数(AI)值与SLEDAI-2000评分均呈正相关(r值分别为0.86、0.88、0.84和0.77,均P<0.05),与BILAG-2004评分亦均呈正相关(r值分别为0.88、0.98、0.86和0.89,均P<0.05);SLEDAI-2000评分与Ⅱ型LN患者AI评分相关性最好,其次为Ⅰ型;BILAG-2004评分与Ⅱ型LN患者AI评分相关性最好,其次为Ⅳ型;BILAG-2004评分系统的曲线下面积(AUC)为0.93,SLEDAI-2000 AUC为0.88。结论:BILAG-2004和SLEDAI-2000评分系统均可以评估LN患者的肾脏病变活动度。BILAG-2004评分系统结果更加可靠,更加全面。  相似文献   

12.
This study was done to determine whether intravenous methylprednisolone therapy given concomitantly with low-dose daily, oral prednisone would be as effective as highdose daily prednisone in the treatment of patients with active systemic lupus erythematosus (SLE) nephritis.Thirteen patients with active SLE nephritis were started on 2 mg/kg prednisone per day, considered the high prednisone phase. Therapy was continued until remission was achieved. Prednisone administration was then tapered to less than 0.5 but more than 0.2 mg/kg per day. On later relapse, these patients received three doses of methylprednisolone (20 mg/kg per dose) on alternate days and continued on the same daily dose of prednisone (<0.5 >0.2 mg/kg per day) prior to pulse therapy; this was the methylprednisolone phase. The 13 patients were studied in both phases, serving as their own controls.After 1 month of therapy, no significant differences were observed between treatment phases as to improvement in clinical and laboratory findings. A significant increase in the serum concentration of C3 and C4 was seen both in the highdose prednisone and methylprednisolone phases, while the serum concentration of anti-ds DNA antibody significantly decreased.Methylprednisolone therapy seems as effective as highdose prednisone in patients with relapse of SLE nephritis. Because side effects are minimal, methylprednisolone administration may be tried as the therapy of choice for these patients.Abbreviation SLE systemic lupus erythematosus  相似文献   

13.
小儿狼疮性肾炎88例临床分析   总被引:1,自引:0,他引:1  
目的探讨小儿狼疮性肾炎(LN)的临床特点。方法对88例小儿LN和200例成人LN进行了比较分析。结果小儿组男性比例较高,初发症状以发热、消瘦、乏力、水肿、肺部感染等多见;肾脏受累率两组差异不大,但儿童组受累的严重程度较成人重,且从发病至出现肾脏损害的平均时间较短;小儿贫血、血小板减少较多见,狼疮细胞阳性率高;感染为小儿LN死亡的主要原因。结论小儿LN的早期诊治很重要  相似文献   

14.
目的观察大剂量短疗程泼尼松(Pred)疗法对儿童急性免疫性血小板减少症(ITP)的疗效。方法 162例ITP患儿根据治疗方法不同随机分为大剂量静脉丙种球蛋白+甲基泼尼松龙组(IVIG+MP)、静脉丙种球蛋白组(IVIG)、甲基泼尼松龙组(MP)与Pred组。IVIG+MP组41例,采用IVIG(1g/kg,共1次)+MP[10 mg/(kg.d),每3天减半量,共9 d]冲击治疗,继之口服Pred[1.5~2.0 mg/(kg.d)],并逐渐减量维持治疗;IVIG组39例,采用丙种球蛋白(1 g/kg,共1次)冲击治疗,继之口服Pred[1.5~2.0 mg/(kg.d)]并逐渐减量维持治疗;MP组40例,采用MP[10 mg/(kg.d),每3天减半量,共9 d]冲击治疗,继之口服Pred[1.5~2.0 mg/(kg.d)]并逐渐减量维持治疗;Pred组42例,采用口服Pred[4 mg/(kg.d),共4 d]治疗后停药,无减量维持治疗。比较各组治疗前后血小板数、治疗有效率、不良反应发生率及药费支出。结果各治疗组治疗前后血小板数及治疗有效率差异无显著性,IVIG+MP组、IVIG组、MP组治疗不良反应发生率及药费支出均高于Pred组。结论大剂量短疗程Pred疗法治疗儿童急性ITP能有效提升血小板计数,有效率与IVIG及MP冲击治疗相比差异无显著性,不良反应少,花费低。  相似文献   

15.
A 12-year-old girl with signs and symptoms of lupus erythematosus and nephritis had, by renal biopsy, glomerular capillary loop deposits identical in location and composition to those described in children with the dense deposit lesion of membranoproliferative glomerulonephritis, Type II. Following of prednisone therapy, serologic and clinical abnormalities resolved. The patient has experienced excellent growth and no recurrence of disease while receiving alternate-day prednisone therapy. The dense deposit lesion has not previously reported in patients having signs and symptoms of lupus glomerulonephritis.  相似文献   

16.
Among 68 children with severe dilated cardiomyopathy, 43 (aged 10 months to 15 years) presented with active myocarditis, diagnosed by endomyocardial biopsy. They were divided into four treatment groups: I, controls: 9 patients submitted to conventional treatment (digitalis, diuretics, and vasodilators) for 8.1±0.7 (SD) months; II, prednisone: 12 patients received conventional therapy plus prednisone; III, azathioprine: 16 patients submitted to conventional therapy plus prednisone and azathioprine; IV, cyclosporine: 13 patients treated with conventional therapy plus prednisone and cyclosporine. Immunosuppressive therapy was maintained for a mean of 8.4±1.2 months. They were submitted to noninvasive (electrocardiogram, chest radiograph, Doppler echocardiogram, and radioisotopic scintigraphy) and invasive (hemodynamic) studies. In the control group only 2 of 9 patients showed clinical and hemodynamic improvement and 1 of 4, histologic regression of the myocarditis. Among patients submitted to conventional therapy plus prednisone, 3 of 12 presented clinical and hemodynamic improvement; 2 of 5 also showed histologic regression of inflammatory process. By contrast, patients treated with azathioprine or cyclosporine associated with prednisone had significantly better results: 13 of 16 and 10 of 13 patients, respectively, had clinical and hemodynamic improvement; all 6 patients in the azathioprine group and all 4 patients in the cyclosporine group had histologic regression of the myocarditis. Two patients in the prednisone group, one in the azathioprine group, and one in the cyclosporine group died during treatment, in cardiogenic shock. In our experience immunosuppressive therapy with azathioprine or cyclosporine associated with prednisone improves the prognosis of children with active myocarditis and severe ventricular dysfunction.  相似文献   

17.
儿童特发性膜性肾病临床病理特点及治疗探讨   总被引:1,自引:0,他引:1  
目的 了解儿童特发性膜性肾病(IMN)的临床病理特点,探讨其治疗方案.方法 回顾性分析25例病理确诊的IMN患儿的临床病理特点,总结其不同治疗方法 的疗效.结果 儿童IMN占同期所有肾穿刺活检(简称肾穿)患儿的3.81%.25例IMN中男9例,女16例;起病年龄2~14岁,平均(9.4±3.4)岁;肾穿时病程0.4~11.0个月,中位数2.5个月.临床表现为肾病综合征肾炎型21例(84%),肾小球肾炎4例(16%).全部患儿均伴血尿,其中肉眼血尿7例,高血压4例,并发血栓2例,肾功能不全1例.病理分期IMNⅡ期21例(84%).伴中重度小管间质损害者6例,伴局灶节段硬化2例.22例肾病综合征及肾病水平蛋白尿患儿中,21例首选糖皮质激素治疗,其中20例符合评价激素疗效标准:激素敏感1例(复发后转为激素耐药),19例为激素耐药(95%).后续治疗包括继续单纯激素减量隔日治疗8例,其中完全缓解5例,部分缓解3例;激素联合免疫抑制剂治疗12例,该12例连同首选联合免疫抑制剂治疗1例、激素治疗5周联合免疫抑制剂治疗1例,共14例.结论 本组患儿IMN临床表现以肾病综合征为主,均伴有不同程度血尿.绝大多数初治激素耐药,但部分病例减量隔日治疗过程中获缓解,联合免疫抑制剂治疗及疗效尚需进一步临床验证.
Abstract:
Objective To investigate the clinicopathological feature and treatment of idiopathic membranous nephropathy(IMN)in children.Method A retrospective analysis of 25 cases of biopsyproven IMN seen between January 2004 and December 2009.Result The incidence of IMN was 3.81% in all the children patients who underwent renal biopsy.Of 25 patients with IMN,nine were boys and sixteen were girls.The mean age at onset was(9.4±3.4)years with a range of 2-14 years.Renal biopsies were performed at a median 2.5 months(range 0.4-11 months)after onset.The clinical manifestations included nephrotic syndrome(NS)nephritic type in 21 cases(84%)and glomerulonephritis in 4 cases.All patients presented with hematuria,and 7 had macroscopic hematuria.Hypertension was noted in 4 patients.Two patients were complicated with thrombosis.One patient was in a chronic renal insufficiency(CRI)state.According to the MN staging criteria,21 cases were in stage Ⅱ IMN(84%).Six patients showed moderate or severe tubulointerstitial lesion.Focal segmental glomerulosclerosis(FSGS)was found in two patients.Of the 22 patients with NS and nephrotic proteinuria,21 cases were treated with prednisone initially and in 20 of them the efficacy of corticosteroid therapy was evaluated:one of them was steroid sensitive(became steroidresistant after relapse)and all the others were steroid-resistant(95%).The subsequent treatment:eight of them were treated with prednisone followed by a taper to alternate-day therapy.Five of them had complete remission and three partial remission.Twelve cases were treated with combined therapy of prednisone and immunosuppressive agents. Of these 12 cases together with one case who received initially combined treatment with prednisone and immunosuppressive agent and one case treated with prednisone initially for five weeks then with combined therapy contained another immunosuppressive agent,totally 14 cases,5 had complete remission,2 partial remission,3 did not achieve remission,and 3 had unknown response.Conclusion Of the patient cohort,the predominant presenting feature was nephrotic syndrome,and with different degree hematuria.Almost all of them were steroid resistant,but followed by a taper to alternate-day therapy,some could achieve remission.The effect of a combination of prednisone and immunosuppressive agent is needed to be further proven in children.  相似文献   

18.
患儿为12岁2个月的青少年女性,反复腹痛、呕吐2年余,关节痛3个月,诊断为复发性急性胰腺炎,多次入院治疗,胰腺炎原因不明。实验室检查发现空腹血清三酰甘油多次显著升高,免疫球蛋白升高、抗核抗体阳性,予对症支持治疗后症状好转。3个月前患儿出现四肢关节肿痛伴活动障碍,检查发现蛋白尿、血尿,抗双链DNA抗体阳性,进一步肾脏穿刺检查,病理及冷冻切片免疫荧光提示弥漫性狼疮性肾炎(Ⅳ型)。最终诊断为系统性红斑狼疮、狼疮性肾炎Ⅳ型、复发性急性胰腺炎。胰腺炎高度可疑与系统性红斑狼疮有关。予硫酸羟氯喹、甲泼尼龙琥珀酸钠及环磷酰胺治疗,四肢关节肿痛症状改善。后改为醋酸泼尼松片口服,定期予以环磷酰胺冲击治疗及贝利优单克隆抗体泵注等治疗,目前关节疼痛好转,仍有蛋白尿及血尿。临床上需要警惕以胰腺炎为首发表现的系统性红斑狼疮,对不明原因胰腺炎需进行相关免疫学指标检查,密切监测其他系统的症状,以免延误诊断。[中国当代儿科杂志,2022,24(8):917-922]  相似文献   

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