儿童系统性红斑狼疮并发假性肠梗阻8例临床研究

目的 探讨儿童系统性红斑狼疮(SLE)并假性肠梗阻(IPO)的临床特点及诊治方法.方法 回顾性分析中国医学科学院北京协和医院2010年1 月至2018年12月收治的16岁以下8例SLE并IPO患儿的临床资料,总结其发病特点、诊治方案和预后.结果 共8例纳入研究,其中男2例,女6例,发病年龄(13.6±1.7)岁(11～...     

儿童狼疮中枢神经系统的急症表现

目的：探讨儿童系统性红斑狼疮中枢审经系统急症的特征,方法：将狼疮脑病患儿与继发中枢神经系统损害狼疮患儿的ANA,dsDNA滴度,Sm阳性率及补体C3下降的阳性率进行比较,并对两组患儿的临床特征进行分析,结果：ANA、ds-DNA滴度及Sm阳性率,补体C3下降阳性率与狼疮脑病并不相关,脑电图有助于狼疮脑病的诊断。结论：儿童红斑狼疮中枢急症原发及继发性损害的鉴别诊断需结合临床及有关辅助检查综合分析。     

儿童肱动脉假性动脉瘤一例并文献复习

目的:报道1例儿童肱动脉假性动脉瘤的诊疗,并回顾相关文献总结此类疾病的病因、诊治及预后。方法:回顾性分析了2019年6月1例16天新生儿的左上肢肱动脉假性动脉瘤的临床资料。本例患儿出生后6d发现逐渐增大的左肘部包块。B型超声、左上肢MRI及CTA提示左肱动脉假性动脉瘤。经局部加压包扎治疗,包块持续增大并出现表面皮肤坏死...     

红霉素治疗急性假性结肠梗阻1例

患儿,男,2岁,因第1腰椎结核入院,在全身麻醉下行腰椎结核病灶清除、植骨术。手术过程顺利。术后d3患儿突然出现进行性腹胀、腹痛、呕吐。体检:体温37.9℃,脉搏118次/min,营养状态差。腹胀明显,叩诊鼓音,无压痛、反跳痛及腹肌紧张,肠鸣音存在。实验室检查:WBC 10.7×109/L,PLT26     

儿童狼疮中枢神经系统的急症表现

目的　 探讨儿童系统性红斑狼疮中枢神经系统急症的特征。 方法 　将狼疮脑病患儿与继发中枢神经系统损害狼疮患儿的ANA、dsDNA滴度、Sm阳性率及补体C3下降的阳性率进行比较 ,并对两组患儿的临床特征进行分析。 结果 　ANA、ds DNA滴度及Sm阳性率、补体C3下降阳性率与狼疮脑病并不相关 ,脑电图有助于狼疮脑病的诊断。 结论 　儿童红斑狼疮中枢急症原发及继发性损害的鉴别诊断需结合临床及有关辅助检查综合分析。     

儿童系统性红斑狼疮相关再生障碍性贫血2例报告并文献复习

目的 总结系统性红斑狼疮(SLE)相关的再生障碍性贫血(AA)的临床特点,以提高对该病的认识.方法 对收治的2例及文献报道的19例儿童SLE相关AA患者进行回顾性分析,并与单纯SLE及普通AA进行比较.结果 21例患者SLE和AA常不能同时诊断(占90.4%),SLE与AA的诊断相隔时间为-1～9年.实验室检查中抗ds-DNA抗体(42.8%)及补体降低(38.9%)的阳性率低于单纯SLE,外周血淋巴细胞比例升高(23.5%)及网织红细胞降低(17.6%)的发生率均低干普通AA,骨髓像、骨髓病理(100%)示骨髓造血不良,T细胞亚群中CD4+降低,CD8+升高,CD4/CD8倒置.治疗的有效率达85.7%,病死率约9.5%.结论 SLE相关AA临床表现无特异性;表现为外周血三系持续减少的患者应尽早作骨髓检查及免疫指标检测,以减少漏诊;其骨髓像、骨髓病理及T细胞亚群的特点与普通AA相似,但预后明显好于普通AA.     

以血小板减少性紫癜为首发表现的儿童系统性红斑狼疮13例诊治分析

目的 总结儿童系统性红斑狼疮的诊治经验.方法 回顾性分析2004年1月至2009年6月我院收治的13例以急性免疫性血小板减少性紫癜为首发症状的小儿系统性红斑狼疮的诊断、治疗及转归情况.结果 13例初诊患儿经临床表现及骨髓检查诊断为急性免疫性血小板减少性紫癜,经用激素及静脉丙种球蛋白治疗效果欠佳,动态随诊免疫指标及其他临床表现,并根据血液系统改变特点和对激素的反应加用其他免疫抑制剂如环孢素A、霉酚酸酯及环磷酰胺等.13例患儿分别于2～24个月确诊为系统性红斑狼疮,加用其他免疫抑制剂后治疗效果满意.结论 对于血小板减少的患儿尤其是青春期女孩要注意动态监测抗核抗体及其他免疫学指标,警惕系统性红斑狼疮的可能.     

儿童狼疮脑病39例报告

为探讨儿童狼疮脑病的临床特点及预后，通过长期随访，对１３０例系统性红斑狼疮（ＳＬＥ）患儿中的３９例狼疮脑病的临床资料进行了分析和总结。结果，狼疮脑病的发生率为３０％。其中８５％（３３／３９）的患儿在确诊ＳＬＥ第１年内出现中枢神经系统（ＣＮＳ）受损，１８％（７／３９）以ＣＮＳ损害作为ＳＬＥ首发症状。最常见ＣＮＳ异常是癫痫发作、头痛、昏迷、急性器质性脑病综合征样表现。随访的２５例中，坚持按医嘱服泼尼松者１２例，病情持续稳定；间断服药、自行加减泼尼松剂量１１例，病情波动，其中６例间断发热、皮疹、关节肿痛，５例遗留ＣＮＳ后遗症；自行停止治疗的２例均死亡。提示，ＣＮＳ受累是儿童ＳＬＥ常见的严重并发症，临床表现具多样性，故应注意鉴别诊断。早期诊断和治疗，长期随访和坚持按医嘱服药，可望获得良好预后。     

儿童系统性红斑狼疮合并鼻眶脑型毛霉菌病1例报告并文献复习

目的　提高对儿童系统性红斑狼疮（PSLE）并鼻眶脑型毛霉菌病（ROCM）的认识。 方法　总结2019年12月河北医科大学附属燕达医院儿科收治的1例PSLE并发ROCM的病例资料,并复习相关文献。结果　PSLE并发ROCM罕见,诊断困难,治疗效果欠佳。结论　对于PSLE合并ROCM应加强认识,早期诊断和多学科治疗有助于降低病死率。     

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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。     

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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.     

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。     

Recurrent antiphospholipid-related deep vein thrombosis as presenting manifestation of systemic lupus erythematosus

Antiphospholipid antibodies (aPL) are frequently associated with thrombotic disorders in the so-called antiphospholipid syndrome. Together with anticardiolipin antibodies (aCL), lupus anticoagulant (LA) is the main diagnostic tool for aPL detection. Since LA determination is based on the finding of prolonged clotting time in vitro, concomitant anticoagulant therapy may significantly interfere with its detection. We report a case of a boy in whom recurrent aPL-related thrombosis heralded for several months the onset of systemic lupus erythematosus (SLE). Abnormally increased in vitro clotting times at the time of the second thrombotic event led to the suspicion of the presence of LA activity. However, this latter finding was difficult to interpret since the patient was already on heparin treatment at the time of our first observation. Thus, LA was assayed using a commercial kit in which a heparin neutralizer is included (Staclot LA). Two consecutive samples from the patient were compared with eight patients on anticoagulant therapy for non-aPL-related thrombotic events and 20 healthy controls. The study showed that, taking into account the concomitant anticoagulant treatment, Staclot LA was positive only in the propositus, raising the suspicion of a possible aPL-related origin of the thrombotic event. This issue was definitively confirmed in a subsequent follow-up. Conclusion The present report shows that aPL-related deep vein thrombosis can be the earliest clinical manifestation of pediatric SLE, and that Staclot LA may have a role in LA detection during the course of anticoagulant treatment. Received: 6 October 1998 / Accepted: 15 September 1999     

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.     

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??Abstract??Objective??To observe the efficacy and safety of Rituximab ??Roche Pharmaceuticals RTX?? trade name?? rituximab?? treatment for children with systemic lupus erythematosus??and try to find a useful alternative therapeutic approach to those with bad response to traditional therapies. Methods??Produced by Roche Pharmaceuticals??CD20 monoclonal antibody rituximab was used by intravenous injection. Initial dose was 188 mg/m2?? 2 weeks later the second injection was given at 375 mg/m2. Thirty minutes before each injection 5mg Dexamethasone and 10mg Promethazine were given to prevent drug allergy. Results??Twelve cases of SLE in children were performed flow cytometry detection of CD20+ 4 weeks after administration??10 cases were 0??85.71%?? in B cell depletion?? and gradually rose after 6??8 months. IgG?? IgM and IgA plasma had no significant difference from the baseline. Clinical manifestations and clinical signs in 12 cases of children were evaluated??and the average score dropped from 16.0±2.95 to 8.67±1.83. ANA?? Anti-ds-DNA?? C3 and C4 had different degrees of improvement. In 6 cases of lupus nephritis children with urinary protein was significantly improved after 6 months of Rituximab treatment??4 cases of elevated serum creatinine and blood urea nitrogen was also returned to normal after 6 months of treatment. One case had severe pulmonary infection. Conclusion??Significant effects of CD20+ monoclonal antibody ??rituximab?? treatment are shown for children with SLE and lupus nephritis?? especially in severe children. This provides a new alternative treatment for those with poor tolerance to traditional Prednisone and cyclophosphamide treatment and with poor clinical effect. However?? infection problem can not be ignored. The Rituximab replacement treatment for children with SLE still need further study.     

Mizoribine as an effective combined maintenance therapy with prednisolone in child-onset systemic lupus erythematosus

BACKGROUND: Systemic lupus erythematosus (SLE) is one of the major collagen diseases in childhood. However, the pathogenesis of this disease still remains unknown. The disease is known as a chronic inflammatory disease. Since oral and intravenous corticosteroid therapy has been introduced into the treatment of SLE, the prognosis of patients has improved significantly. However, it has now become clear that there are limitations in the effectiveness, as well as adverse reactions when corticosteroids therapy is administered for a long-term period. Therefore, we have been attempting to improve the maintenance therapy of child-onset SLE. METHODS: We have proposed and tested a new type of combination therapy using prednisolone (PSL) and mizoribine (MZR) in pediatric patients with SLE for maintenance therapy after the induction of remission. RESULTS: Our results showed that this combination therapy is more effective than the previous regimen. In addition, no significant side-effects were observed in our study. CONCLUSION: This combination therapy is still not perfect. Efforts should be continued to establish an optimal therapy regimen for child-onset SLE.