后腹膜纤维化现状

后腹膜纤维化是一种病因未明的腹膜后纤维脂肪非特异性、非化脓性的炎症 ,以后腹膜广泛纤维化为特征 ,且临床上少见的、非特发性疾病。其病因可能与围绕腹主动脉发生的恶性肿瘤、炎症、放射线治疗等众多原因有关 (约 70 %病因仍然不明 )。该疾病临床表现缺乏特异性 ,诊断主要靠KUB、CT、MRI,药物和手术是治疗本病的有效方法。本文就其病因 ,发病机制 ,诊断及治疗作一综述     

特发性腹膜后纤维化并发肾积水

目的:探讨特发性腹膜后纤维化(IRF)并发肾积水的临床特征.方法:报告12例IRF并发肾积水患者的临床资料.12例均以腰、腹痛为首发症状.B超均发现肾积水及输尿管病变,3例发现腹膜后肿块.腹部CT均发现腹膜后肿块,对肿块的大小和部位均能明确.术前诊断准确率为83.3%.10例行输尿管松解 大网膜包裹或腹腔移位术,1例逆行置人输尿管支架管,1例肾穿刺造瘘.后2例情况好转后,仍行手术治疗.结果:10例随访无复发,1例因同侧肾积水复发行二次治疗,1例因对侧肾积水而行二次手术.结论:腰、腹痛患者应考虑到特发性腹膜后纤维化的可能;B超对肾积水敏感,腹膜后肿块依赖腹部CT检查,两者联合应用有利于诊断.输尿管松解 大网膜包裹或腹腔移位术是主要的有效的治疗手段.单侧腹膜后纤维化患者,随访应同时注意对侧发病的可能.     

腹膜后纤维化致肾积水

目的　提高腹膜后纤维化的诊治水平。　方法　报告收治的腹膜后纤维化致肾积水患者5例。　结果　原发性腹膜后纤维化4例,采用输尿管松解、带蒂大网膜包裹治疗,随访6～40个月,肾功能正常,肾积水基本消失。继发性腹膜后纤维化1例,因肾积水严重,行患肾切除,随访36个月,对侧肾功能正常。　结论　逆行肾盂造影是腹膜后纤维化重要的诊断手段,带蒂大网膜包裹术是有效的治疗方法。     

不同性别系统性红斑狼疮的临床特征分析

系统性红斑狼疮 (ＳＬＥ)是常见病 ,在我国发生率高达0 .1% ,肾脏损害是其显著的特点 ,其发病有着明显性别差异 ,男女的比例为 1∶5～ 12 〔1,2〕。据文献报道 ,男性ＳＬＥ的临床表现、治疗及预后均有别于女性患者〔2～ 4〕。作者比较分析了收集到的 2 40例女性 ,32例男性ＳＬＥ患者起病及进展期的临床表现、自身抗体分布以及首始起病时的病理分型 ,以探讨两性ＳＬＥ的临床特点。资料和方法1　病例来源　 1983年～ 1999年我院收治的住院患者 ,以及作者在进修期间收集到的住院患者 ,女性 2 40例 ,男性 32例 ,发病年龄 5岁～ 78岁 ,全部 2 72…     

特发性腹膜后纤维化16例临床分析

目的 探讨特发性腹膜后纤维化(iRPF)临床特点、诊疗及转归,以提高临床诊断率,减少漏诊和误诊.方法 回顾分析在青岛大学附属医院住院确诊的16例iRPF患者,其中包括9例IgG4相关腹膜后纤维化患者.结果 iRPF多见于中老年男性,肾功能衰竭合并腰腹疼痛、体重减轻是最常见的临床表现.IgG4-RPF患者与非IgG4-RPF患者Hb、SCr、ESR、CRP组间比较差异无统计学意义.所有患者均存在CT或MRI下腹膜后软组织密度影;IgG4-RPF患者同时还可发现颌下腺、深部淋巴结、胰腺、胆管、胸腺、肾间质、甲状腺等多器官受累.病理学检查发现全部IgG4-RD患者不同受累器官均显示相同特点的改变,且其IgG4阳性的浆细胞计数、IgG4 +/IgG阳性浆细胞计数比例、席纹状纤维化例数较非IgG4-RPF患者有显著的统计学差异.8例行皮质类固醇和免疫抑制剂治疗;6例行药物及手术联合治疗;2例单纯手术治疗.所有接受皮质类固醇治疗的患者病情均缓解,其中2例停药后复发;但单纯外科治疗患者反复发作.结论 特发性腹膜后纤维化与IgG4-RD疾病谱有交集,但临床特点及普通影像学检查无法做出鉴别,需要进一步病理学检查.激素及免疫抑制剂对两者治疗均有效并可减少复发.     

腹膜后纤维化合并食管狭窄的临床分析

腹膜后纤维化(retroperitoneal fibrosis,RPF)为临床少见疾病,国外报道年平均发病率为1/20万～1/50万[1],合并食管狭窄更为少见.本文对1999年至2006年我院3例RFP合并食管狭窄的患者进行回顾性分析,现报道如下.     

腹膜后纤维化的诊断与治疗

目的 探讨腹膜后纤维化致肾积水的病因、诊断及治疗。方法 分别采用将输尿管移于腹腔内、带蒂大网膜包裹以及回肠代输尿管等方法治疗腹膜后纤维化致肾积水5例。结果 随防1－3年,肾功能正常,积水明显好转。结论 静脉尿路造影（IVU）,逆行造影（RGP）以及CT是诊断的主要手段,治疗应根据粘边输尿管的范围、部位、有无管腔闭塞而采取不同的手术方法。     

后腹膜纤维化现状

后腹膜纤维化是一种病因未明的腹膜后纤维脂肪非特异性，非化脓性的炎症，以后腹膜广泛纤维特征，且临床上少见的，非特发性疾病。其病因可能与围绕腹主动脉发生的恶性肿瘤，炎症，放射线治疗等众多原因有关（约70％病因仍然不明）。该疾病临床表现乏特异性，诊断主要造KUB、CT、MRI，药物和手术是治疗本病的有效方法。本文就其病因，发病机制，诊断及治疗作一综述。     

腹膜透析相关性腹膜纤维化及治疗

腹膜透析是终末期肾病的重要替代疗法，其所导致的腹膜纤维化也越来越引起人们的重视。本文就腹膜纤维化的机制及治疗作一综述。     

Pneumococcal infection in patients with systemic lupus erythematosus

Objective

Our study aimed to analyze the risk factors associated with the occurrence and severity of pneumococcal infection (PI) in systemic lupus erythematosus (SLE) patients.

抗核抗体阴性的系统性红斑狼疮的探讨

目的 :探讨抗核抗体 (ANA)阴性的系统性红斑狼疮 (SLE)的特点。方法 :总结 1991年～ 1998年 85 3例住院SLE病人 ,发现ANA阴性者有 139例 ,对ANA阴性者的临床及实验室资料进行分析 ,并与ANA阳性者比较。结果 :ANA阴性者光过敏明显增加 ,肾炎、关节炎则明显减少 ,雷诺氏现象、脱发也较少 ;抗SS -A抗体较多见 ,抗ds-DNA明显少于ANA阳性者 ,抗Sm、抗核糖核蛋白抗体、类风湿因子阳性率较低。结论 :ANA阴性的SLE是SLE的一种亚类 ,其特点是皮肤光过敏多见 ,抗SS -A阳性率较高。     

90例系统性红斑狼疮患者死亡原因分析

目的分析系统性红斑狼疮（ＳＬＥ）患者的死因，目的在降低其病死率。方法对４６５例ＳＬＥ患者中的９０例死者死因进行分析。结果最主要死因是ＳＬＥ本身４０例（４４４４％），其次是感染３０例（３３３３％），心血管疾病１２例（１３３３％）。ＳＬＥ与感染致死较常发生于年轻患者。ＳＬＥ死亡多发生在诊断后的头三年。结论ＳＬＥ死亡最主要原因是ＳＬＥ本身，故对ＳＬＥ活动应积极治疗，但不能过度，以免引发感染而死亡。     

Rituximab therapy for juvenile-onset systemic lupus erythematosus

Rituximab (RTX), an anti-CD20 monoclonal antibody, has been proposed for use in the therapy of systemic lupus erythematosus (SLE). We present the initial long-term experience of the safety and efficacy of rituximab for treatment of SLE in children. Eighteen patients (mean age 14 ± 3 years) with severe SLE were treated with rituximab after demonstrating resistance or toxicity to conventional regimens. There was a predominance of female (16/18) and ethnic African (13/18) patients. All had lupus nephritis [World Health Organization (WHO) classes 3–5] and systemic manifestations of vasculitis. Clinical disease activity of the SLE was scored with the SLE-disease activity index 2K (SLEDAI-2K). Patients were followed-up for an average of 3.0 ± 1.3 years (range 0.5 to 4.8 years). B-cell depletion occurred within 2 weeks in all patients and persisted for up to 1 year in some. Clinical activity scores, double-stranded DNA (dsDNA) antibodies, renal function and proteinuria [urine protein to creatinine ratio (Upr/cr)] improved in 93% of the patients. Five patients required multiple courses of RTX for relapse, with B-cell repopulation. One died of infectious endocarditis related to severe immunosuppression. In conclusion, our data support the efficacy of rituximab as adjunctive treatment for SLE in children. Although rituximab was well tolerated by the majority of patients, randomized controlled trials are required to establish its long-term safety and efficacy. The authors declare that they have no conflict of interest.     

Renal infarction in a child with systemic lupus erythematosus

Although patients with systemic lupus erythematosus (SLE), especially those with antiphospholipid antibodies, have a high incidence of arterial and venous thrombotic manifestations, renal infarction has been rarely reported in these patients and is probably underestimated. A 9-year-old boy with renal infarction, diagnosed by computed tomography and scintigraphy, is described. Initially he complained of severe flank pain; he had no urinary abnormalities and his blood pressure was normal. No evidence of systemic disease was found. He responded well to antibiotic treatment without the need for immunosuppressive therapy. In subsequent years he presented a spectrum of clinical symptoms, including fever, malaise, arterial hypertension headache, and mononeuritis multiplex, accompanied by an increased erythrocyte sedimentation rate and transitory proteinuria. This suggested vasculitis involving peripheral vessels as well as the central nervous system. Treatment with oral prednisone and azathioprine led to remission. Four years after the renal infarction, the child presented with recurrence of systemic disease. The diagnosis of SLE was established, with positive antiphospholipid antibodies. The sudden appearance of severe unexplained flank pain should alert the clinician to a possible underlying renal vessel thrombosis. Renal venous thrombosis is probably much more common; however, renal arterial thrombosis and infarction in association with SLE with positive antiphospholipid antibodies should be added to the differential diagnosis.     

Acute acalculous cholecystitis in a child with systemic lupus erythematosus

A 10-year-old boy with systemic lupus erythematosus (SLE) developed abrupt right upper quadrant pain and vomiting during the course of his active disease. Antiphospholipid antibody was negative and the C3 level was low. Abdominal sonography showed cholecystitis with sludge balls in the gallbladder. He was treated by high-dose prednisolone with ceftriaxone and metronidazole IV for 3 days but due to poor response, cholecystectomy was performed and no stone was identified. Histopathologic examination showed vasculitis in the medium-sized arteries of the gallbladder wall. He was doing well at the 9-month follow-up after the operation. This report describes the first pediatric case of SLE with acalculous cholecystitis caused by vasculitis of the gallbladder.     

系统性红斑狼疮妊娠子痫前期的识别和防范

系统性红斑狼疮(SLE)是一种多发生于青年女性的累及多系统多器官的自身免疫性疾病。近年来,关于妊娠期SLE的诊治进行了系统的规范,使一些妊娠期合并SLE的孕产妇有较好的妊娠结局。但是,在这些人群中不良母婴结局的发生率仍然很高,包括妊娠丢失、胎儿宫内生长受限、胎儿死亡和子痫前期(PE)等。SLE妊娠合并PE与妊娠合并SLE活动的临床处理不同,但由于二者常常难以鉴别甚至合并存在,故如何准确诊断从而正确治疗对于减少孕产妇不良妊娠结局尤其重要。     

系统性红斑狼疮患者白细胞糖皮质激素受体测定及其意义

目的 为了解系统性红斑狼疮(SLE)患者糖皮质激素受体(GCR)变化及其与糖皮质激素(GC)疗效关系。方法 用放免法测定患者血浆GC水平,用放射配体结合法测定其治疗前、后外周血白细胞GCR。结果 SLE患者GC水平正常而GCR明显降低;GCR相对高者(>2647.40位点/细胞),GC疗效较好,低于2151.35位点/细胞疗效较差。结论 GCR降低可能参与SLE的病理发生过程;GCR水平可指导GC用药及预测疗效。     

Retrospective study of plasma exchange in children with systemic lupus erythematosus

A retrospective review of patients was conducted to evaluate the short-term effect of therapeutic plasma exchange (TPE) in children with active systemic lupus erythematosus (SLE). Between April 1984 and March 2003, 22 children underwent 112 TPE sessions. The median number of daily, double-volume sessions was 6 (range 1–16). Plasma exchange was performed by centrifugation, using 4.5% albumin with fresh-frozen plasma as required. All children were receiving standard immunosuppressive therapy. The predominant clinical features that led to TPE were categorised into systems and outcome was evaluated according to clinical improvement or lack of it. There was benefit for central nervous system and pulmonary manifestations (10 cases, 11 episodes). Of 8 children treated for renal manifestations, 6 achieved good outcomes. Two children became dialysis dependent despite treatment; however, both had been successfully treated with TPE before. Five children received more than one course of TPE. No child died during the procedure and major complications were few. Our data show that TPE can be used as adjunctive therapy in childhood SLE with few complications. Outcome is difficult to measure but in our experience, during the acute phase of illness, TPE appears to be of benefit.This work was presented in part as a poster at the 11th International Vasculitis and ANCA Workshop, Prague, Czech Republic, October 2003     

Clinical manifestations and analyses of the cytotoxic T-lymphocyte associated-4 gene in two Japanese families with systemic lupus erythematosus

Although still incompletely understood, the etiology of systemic lupus erythematosus (SLE) is considered to involve both genetic and environmental factors. We encountered two boys with severe SLE from unrelated families and analyzed the gene that encodes cytotoxic T-lymphocyte-associated (CTLA)-4, a protein important in T-cell activation and immune tolerance. Abnormal function of the gene may participate in causation of autoimmune disease, including SLE. In family 1, a boy showed serious cardiovascular complications associated with heart failure, and his mother also had clinically active SLE, including nephritis. A boy in family 2 developed severe renal complications and peripheral vasculitis accompanied by disseminated petechiae in the lower extremities. His paternal grandfather had died from fibrinous pneumonia caused by SLE. They showed high SLE Disease Activity Index (SLEDAI) score. Analysis of the CTLA-4 gene indicated that the boy in family 1 and his mother and the boy in family 2 possess a GG genotype in CTLA-4 exon 1 at +49 together with a 106-bp fragment length of the 3′ untranslated region (UTR) in exon 4. No association with disease activity was found for polymorphism of the promoter region in exon 1 at −318 in either family. Disorders of the CTLA-4 gene, especially a GG genotype in exon 1 at +49 and/or 106-bp fragment length of the 3′UTR in exon 4, may be involved in early development of SLE in Japanese children, such as the boys described here.