小儿系统性红斑狼疮伴贫血的临床分析

目的 :了解小儿系统性红斑狼疮 (SLE)伴贫血的发生率、临床及实验室检查特征、误诊情况。方法 :对10 3例SLE患儿进行回顾性分析 ,将其中 78例伴有贫血的患儿分为两组 ,一组为自身免疫性溶血性贫血组 (AIHA组 ) ,另一组为非AIHA组 ,进行临床症状及实验室检查的比较。结果 :10 3例SLE中贫血发生率为 75 .7%(78/ 10 3) ;AIHA组和非AIHA组在发病年龄、性别上无显著性差异 ;在临床表现上 ,伴AIHA的SLE常以贫血为首发症状或主要表现 ;实验室检查上AIHA组血中IgG、补体C3 、球蛋白 (Glb)、总蛋白 (TP)降低比非AIHA明显。结论 :贫血是SLE常见的表现 ;SLE伴AIHA者 ,其免疫功能紊乱更明显 ,狼疮活动程度高 ;以AIHA的表现为首发症状或主要表现的SLE患者 ,易误诊。     

迟发性狼疮性肾炎的临床及病理特点

目的:探讨迟发性系统性红斑狼疮(late onset systemic lupus erythematosus)即年龄≥50岁系统性红斑狼疮患者的临床、实验室检查及肾脏病理特点。方法:回顾分析我院肾脏科1995年1月~2014年1月20例年龄≥50岁系统性红斑狼疮(systemic lupus erythematosus,SLE)患者(命名为迟发组)的临床表现、自身抗体、尿常规、肾功能、肾穿刺病理类型及活动性指数和慢性指数,与对照组(年龄18岁~49岁)(命名为非迟发组)135例SLE患者的上述指标进行对比。结果:(1)迟发组SLE男女比例为1∶2.33,显著低于非迟发组的男女比例(1∶10.25)。(2)迟发组SLE患者面部皮疹、光过敏的发生率低于非迟发组(P0.05)。(3)迟发组SLE患者乏力、体重下降、口眼干燥非特异临床表现高于非迟发组(P0.01)。(4)实验室检查示:迟发组ds-DNA阳性率及低补体血症发生率明显低于非迟发组(P0.01)。(5)迟发组狼疮性肾炎(lupus nephritis,LN)病理分型前三位分别为LN-Ⅳ型、LN-Ⅲ型、LN-Ⅴ型/LN-Ⅳ+Ⅴ型,与非迟发组LN各病理分型发病率类似。迟发组肾脏病理的慢性化指数高于非迟发组(P0.01),两组肾脏病理活动性指数差异无统计学意义。结论:迟发组SLE患者其临床表现及实验室检查有其自身特点,典型的临床表现少见,特异性抗体阳性率偏低,在临床诊断中应给予足够的重视,减少误诊和漏诊。     

疑诊抗磷脂抗体相关性肾病一例

由于抗磷脂抗体相关性肾病(antiphospholipid antibody-associated nephropathy,APLN)缺乏特异的临床及组织病理学表现,其临床诊断存在一定难度。本例患者为中年女性,临床表现为血肌酐缓慢升高伴血压升高,少量尿蛋白,溶血性贫血,失蛋白肠病,辅助检查提示低补体血症,抗核抗体(ANA)阳性,抗心磷脂抗体(anticardiolipin,aCL)、抗β2糖蛋白-1抗体、狼疮抗凝物(lupus anticoagulant,LA)高滴度阳性。肾穿刺活检光镜及电镜均提示缺血性肾损害,且发现相对特征性的甲状腺样肾小管萎缩,未见电子致密物沉积。故综合临床和病理,诊断为系统性红斑狼疮(systemic lupus eythematosus,SLE)、继发性抗磷脂综合征(antiphospholipid syndrome,APS)、APLN可能性大。     

系统性红斑狼疮患者贫血和疾病活动及肾脏损害的关系

目的 探讨系统性红斑狼疮(systemic lupus erythematosus,SLE)患者贫血和疾病活动及肾脏损害的关系.方法 选取2016年9月至2019年10月乐山市人民医院收治的187例SLE患者作为SLE组,另选取187名体检健康者作为健康对照组,比较其贫血发生率.根据血红蛋白(hemoglobin,Hb...     

系统性红斑狼疮并发腹膜后纤维化的临床特征分析

目的分析系统性红斑狼疮(systemic lupus erythematosus,SLE)并发腹膜后纤维化(retroperitoneal fibrosis,RPF)的发病机制、临床表现、实验室检查、影像学检查以及治疗预后,旨在提高临床医师对SLE并发腹膜后纤维化的认识。方法收集整理我科1例SLE并发RPF患者的临床资料并进行分析,同时查阅并复习国内外相关文献,总结SLE并发RPF的临床特征。结果本文报道1例及国外报道7例SLE并发RPF病例,其中6例为女性患者,2例为男性患者,发病年龄17~54岁,中位年龄为26岁,平均年龄(30.9±15.8)岁。8例SLE并发RPF患者中,4例为腰腹痛就诊,3例为水肿,1例为关节痛。实验室检查提示8例患者均出现不同程度的蛋白尿、血尿,除1例未提及,其他7例抗核抗体均阳性。8例患者中,1例因病情恶化而死亡,7例行糖皮质激素或激素联合外科手术,病情好转后出院,随访无复发。结论 SLE并发RPF在临床上属于罕见病,目前发病机制不清,主要认为与自身免疫性疾病有关,临床症状无特异性,主要表现为局部疼痛和下肢水肿,其诊断主要依赖于影像学检查,目前治疗建议使用激素和免疫抑制剂,对于病变累及输尿管引起输尿管梗阻肾积水的患者可行外科输尿管松解术。     

生物制剂在狼疮肾炎治疗中的应用

<正>一、狼疮肾炎治疗的演变和局限系统性红斑狼疮(systemic lupus erythematosus,SLE)是自身免疫介导的,以免疫性炎症为突出表现的弥漫性结缔组织病。血清抗核抗体为代表的多种自身抗体阳性和多系统受累是SLE的主要临床特征。狼疮肾炎(lupus nephritis,LN)是SLE临床最常见的严重并发症。诊断SLE时50%伴有肾脏损害,而在SLE整体病程中超过60%的成人和超     

系统性红斑狼疮及其肾炎患者尿α1微球蛋白和β2微球蛋白测定的意义

系统性红斑狼疮 (systemiclupuserythematosus,SLE)是一种累及多系统、多器官的自身免疫性疾病。肾脏是最常见的受累的器官。SLE的肾损害既有肾小球的损害又有肾小管间质的损害[1] 。近年来研究表明肾小管间质损伤对肾脏 10年存活率的影响甚至较肾小球病变更为重要[2 ] 。尿α1微球蛋白 (α1-MG)和 β2 微球蛋白 (β2 -MG)是反映肾小管间质损伤较敏感而可靠的指标。为了解SLE及狼疮性肾炎 (LN)患者肾小管受损情况 ,我们检测了尿α1-MG、β2-MG的水平 ,以探讨其在SLE和LN中的临床意义。资料与方法1　临床资料　选择住院确诊的SLE…     

狼疮性脑病研究进展

正系统性红斑狼疮(systemic lupus erythematosus,SLE)是一种自身免疫性疾病,可累及全身各组织脏器。狼疮性脑病(neuropsychiatric systemic lupus erythematosus,NPSLE)是系统性红斑狼疮严重的并发症,发生率约30%~40%~([1]),无论早期还是晚期SLE患者都容易并发,因此早期诊治狼疮性脑病极其重要。本文基于NPSLE的发病机制、临床表现、血清学、影像学等方面的分析,现将其诊断和治疗的最新进展作一综述,为临床诊治提供参考。     

系统性红斑狼疮并发无菌性股骨头坏死的相关因素分析

目的 对系统性红斑狼疮(SLE)并发无菌性股骨头坏死(ONF)的相关因素进行分析,提高SLE合并无菌性股骨头坏死的认识.方法 对32例SLE合并无菌性股骨头坏死的患者进行回顾性分析,并抽取同期年龄、性别匹配的SLE患者作对照,分为股骨头坏死组和非股骨头坏死组,收集整理其临床资料及有关实验室检查,分析其发病情况、临床脏器累及、实验室检查及其治疗等特点.结果 合并有ONF的SLE患者比无ONF组在临床上以网状青斑与雷诺现象更常见,而抗心磷脂抗体阳性率明显高于对照组;激素的起始剂量、每日最高剂量、累积用量与ONF发病无明显相关,而大剂量激素冲击治疗与ONF的发病密切相关;此外,血脂水平、SLEDAI积分、有无使用免疫抑制剂等均与ONF发生无相关性.结论 SLE患者以血管炎为主要临床表现者或凝血功能障碍者,其脉管系统对激素敏感性增加,大剂量激素冲击治疗容易并发ONF.     

男性和女性系统性红斑狼疮临床对比研究

目的 探讨不同性别系统性红斑狼疮（SLE）的临床特点。方法 对30例男性和34例女性SLE患者的临床和实验室检查结果进行了对比研究。结果 平均发病年龄、首次症状到确诊时间、临床表现（关节痛、蝶形红斑、发热、浆膜炎、口腔溃疡等）和实验室检查（dsDNA、IFANA、C3等）男女两组相比无显著差异。男性组反复皮肤溃疡为首发症状明显高于女性组（P＜0．01），肝肿大发生率也明显增高（0．01＜P＜0．05）。女性组ssA抗体阳性率高于男性组（0．01＜P＜0．05）。结论 男性SLE患者常以皮肤溃疡为首发症状，男性SLE患儿常以网状内皮系统增生与血液系统异常为首发症状，老年SLE患者常以浆膜炎为首发症状，女性SLE患者更易继发干燥综合征。     

Coombs-positive autoimmune hemolytic anemia in ulcerative colitis--a report of two cases.

Two cases of ulcerative colitis with a positive Coombs test are reported herein. In a 30 year old woman, hemolytic anemia was well controlled by excision of a rectal stump left over from a former operation. A 53 year old woman had a positive Coombs test without clinical hemolytic anemia 10 years after undergoing a total procto-colectomy with ileo-anal anastomosis. Thus we concluded that total procto-colectomy may be preventive, as well as therapeutic, for Coombs positive hemolytic anemia associated with ulcerative colitis.     

Coombs-positive autoimmune hemolytic anemia in ulcerative colitis —A report of two cases—

Two cases of ulcerative colitis with a positive Coombs test are reported herein. In a 30 year old woman, hemolytic anemia was well controlled by excision of a rectal stump left over from a former operation. A 53 year old woman had a positive Coombs test without clinical hemolytic anemia 10 years after undergoing a total procto-colectomy with ileo-anal anastomosis. Thus we concluded that total procto-colectomy may be preventive, as well as therapeutic, for Coombs positive hemolytic anemia associated with ulcerative colitis.     

Autologous blood donation with recombinant human erythropoietin in anemic patients

BACKGROUND: Various blood management strategies can be used to reduce the need for allogeneic blood in cardiac surgery. In anemic patients, however, avoidance of allogeneic blood transfusion is difficult to achieve. This study was performed to assess the safety and effectiveness of preoperative blood collection using recombinant human erythropoietin (rHuEPO) for reducing the exposure to allogeneic blood in anemic patients. METHODS: Thirty-two anemic patients undergoing cardiac surgery at our hospital between January 1994 and October 1997 were divided into two groups according to preoperative strategies: 3-week treatment with rHuEPO and blood donation (group 1, n = 16) or iron supplementation alone (group 2, n = 16). RESULTS: There were no statistically significant differences between the two groups in patients' characteristics and surgical data. The number of reticulocytes was increased at just before surgery in group 1, whereas group 2 showed no significant increase. The estimated hemoglobin increases in group 1 were higher at 7 days and just before surgery. The mean number of required allogeneic blood for patients during surgery was 0.59 +/- 1.12 U in group 1 and 5.01 +/- 2.63 U in group 2. In 75% of group 1 patients, allogeneic blood transfusion was successfully avoided, whereas all patients in group 2 received allogeneic blood. CONCLUSIONS: This study suggests that the combination of rHuEPO administration and autologous blood donation can reduce the need for allogeneic blood in anemic patients.     

Are hemoglobin levels really lower in faecal immunochemical test positive patients with colorectal cancer?

Introduction

Faecal immunochemical test (FIT) is advocated in many colorectal cancer-screening programs. A positive FIT translates to the need for a colonoscopy. However, waiting times for diagnostic colonoscopy is long. The aim of our study is to determine the correlation of hemoglobin levels in patients with a positive FIT who were subsequently diagnosed with colorectal cancer, and to compare them with patients with only colonic adenomas and those with normal colonoscopy with the intention of determining if hemoglobin levels could be used to stratify the urgency of colonoscopy.

维持性血透患者乙、丙型肝炎病毒和人免疫缺陷病毒感染随访研究

目的:调查维持性血透患者在长程血透治疗过程中乙型肝炎病毒(HBV)、丙型肝炎病毒(HCV)和人免疫缺陷病毒(HIV)感染情况,通过总结进一步降低血透患者上述病毒感染率。方法:收集2004年12月～2009年12月底在我院行规则维持性血液透析半年以上者共381例,每半年检查其血清HBV标志物(HBV-M)、HCV抗体(抗HCV)和抗HIV等情况;2008年1月起严格把抗HCV阳性的血液透析患者与HBsAg阳性患者单独分区和分血透机进行血液透析。比较各患者HBV-M、抗HCV和抗HIV变化情况,同时对比分区分机前后HBV和HCV感染情况。结果:(1)HBV-M检查及HBsAg抗原阳转率:2004年底～2009年底,HBsAg阳性患者分别为3,4,4,7,13,16例,增加的阳性患者均为新进入血透患者,维持性血透患者HBsAg阳转率均为0;(2)抗HCV检查及阳转率:2004年底～2009年底,抗HCV阳性患者总数分别为52例(43.3%),50例(32.3%),40例(25.8%),46例(29.9%),37例(18.8%),27例(11.3%);2005年,2006年和2007年阳转数分别为5例,2例,6例;2008年和2009年没有抗HCV阳转患者;分区分机血透后的两年和前面3年比较,抗HCV阳转率差异有统计学意义(P<0.001)。(3)抗HIV检查及阳转率:所有381例患者在随访期内没有发生抗HIV阳性。结论:在广泛应用促红素减少输血后明显降低了血透患者HBV和HCV感染率,进一步对HBV和HCV感染者采取分区分机的原则和隔离血透的治疗措施,降低了血透患者感染HBV和HCV的风险。     

CAPD的内分泌激素与rHuEPO疗效的相关性

目的：探讨在连续性非卧床腹膜透析(CAPD)干预治疗下慢性肾衰竭尿毒症内分泌激素与人类重组促红细胞生成素(rHuEPO)疗效的关系。方法：对经CAPD治疗的慢性肾衰竭尿毒症患内分泌激素等多重因素及血红蛋白进行多元回归分析。结果：非CAPD组贫血改善程度显低于CAPD组，内生肌酐清除率、甲状旁腺素、甲状腺激素、血皮质酵及透析治疗均与Hb显相关。结论：单用rHuEPO对改善肾衰竭尿毒症的贫血状态存在较大的局限，主要和包括PTH等在内的尿毒症红细胞生长抑制因子(inhibitors of erythropoiesis，IE)有关。rHuEPO配合CAPD是清除IE、改善贫血状态、提高生活质量的很好的组合治疗方法。     

Safety and efficacy of blood donation prior to elective cardiac surgery in anemic patients.

OBJECTIVE: To determine the safety and effectiveness of blood donation in anemic patients, we harvested blood from cardiac patients with baseline hemoglobin levels below 11.0 g/dl. METHODS: Subjects were 118 patients who underwent elective cardiac surgery between January 1994 and October 1997. We assigned patients to 1 of 2 groups based on their entry hemoglobin level: an anemic group (hemoglobin < 11.0 g/dl, n = 20) and a nonanemic group (hemoglobin > or = 11.0 g/dl, n = 98). All patients received subcutaneous administration of recombinant human erythropoietin, and autologous blood was collected once a week for at least 3 weeks before the scheduled surgery date if the hemoglobin level exceeded 10.5 g/dl. RESULTS: No statistically significant differences were seen between the 2 groups in patient profiles or surgical data. The estimated hemoglobin increase did not differ significantly between groups at any time point. The total collected blood volume per patient was greater in the nonanemic group than in the anemic group (1098 +/- 224 ml vs. 712 +/- 334 ml), but the difference in volume was not statistically significant. The percentage of patients who received allogeneic blood did not differ significantly between groups. No side effects were associated with hemoglobin level prior to donation because the incidence of side effects was similar across hemoglobin levels. CONCLUSIONS: This study suggests that autologous blood donation reduces the need for allogeneic blood in patients with baseline hemoglobin levels below 11.0 g/dl.     

Anemia in pediatric hemodialysis patients: results from the 2001 ESRD Clinical Performance Measures Project

BACKGROUND: Despite improvements in dialysis care, anemia remains a problem in pediatric hemodialysis patients. METHODS: To assess possible explanations for the anemia, clinical data were obtained from the Centers for Medicare and Medicaid Services on all hemodialysis patients ages 12 to <18 years between October and December 2000. Complete data were available for 435 of the 516 patients (84%). RESULTS: A total of 160 (37%) patients had a mean hemoglobin of <11 g/dL (anemic). The mean (+/- SD) age for these patients was 15.5 +/- 1.8 years compared to 15.9 +/- 1.5 years for the target hemoglobin patients (P < 0.05). Mean time on chronic dialysis was similar for both the anemic and target hemoglobin patients (>/=100 g/dL) ( approximately 3 years) but patients on dialysis <6 months were more likely to be anemic (67%). While nearly all patients were treated with erythropoietin, anemic patients received greater weekly erythropoietin doses (intravenous, anemia 374 +/- 232 units/kg/week vs. target hemoglobin 246 +/- 196 units/kg/week, P < 0.001; and subcutaneous, 304 +/- 238 units/kg/week vs. 167 +/- 99 units/kg/week, P < 0.05). A total of 59% of anemic patients had a mean transferrin saturation (TSAT) >/=20% compared to 71% of patients with a target hemoglobin (P < 0.01). A mean serum ferritin >/=100 ng/mL was present in approximately two thirds of the anemic and target hemoglobin patients. Approximately 60% of all children were treated with intravenous iron. The mean Kt/V values were lower for anemic patients (1.46 +/- 0.4 vs. 1.53 +/- 0.3, P < 0.05). Anemic patients were less likely to have a normal serum albumin (29% anemic vs. 52% target hemoglobin patients, P < 0.001). CONCLUSION: In the final multivariable regression model, dialyzing <6 months, a low albumin, and a mean TSAT <20% remained significant predictors of anemia in children.     

烧伤后多器官功能障碍综合征患者新喋呤的变化及预警价值

目的 探讨严重烧伤后多器官功能障碍综合征（multiple organ dysfumction syndrome,MODS）患血清新喋呤的变化规律及预警价值。方法 烧伤面积大于70％（70％－100％）的患29例,按是否并发MODS分组,并参照Goris等提出的计分法对MODS进行评分。采用放射免疫分析法检测患血清新喋呤含量的动态变化,并计算新喋呤诊断MODS的有效性和实用性。结果 严重烧伤后第3、7、14和21天,MODS组血清新喋呤水平明显高于非MODS组（P＜0.05－0.01）。相关分析显示,伤后第3、7、21天MODS患新喋呤含量与MODS积分值呈显正相关（r=0.470-0.811）。根据血清新喋呤的预警阈值（35.0nmol/L）,其诊断MODS的敏感性、特异性、阳性预测值和阴性预测值分别为81.1%、89.9%、81.1%和89.9%。结论 循环新喋呤的持续和显升高,与严重烧伤后MODS的发生与发展密切相关,动态观察其改变可能有助于MODS病理过程的监测与早期诊断。     

Prospective study of the immune effects of normalizing the hemoglobin concentration in hemodialysis patients who receive recombinant human erythropoietin

Partial correction of anemia by erythropoietin improves hemodialysis (HD)-associated immunosuppression. It is not known whether hemoglobin normalization improves immune status further. The authors prospectively compared the immune function of HD patients with congestive heart failure or ischemic heart disease on erythropoietin therapy randomized to normal versus anemic blood hemoglobin concentration. HD patients were randomized into a normal hemoglobin group (n = 17, target hemoglobin of 14 +/- 1 g/dl) or an anemic hemoglobin group (n = 18, target hemoglobin 10 +/- 1 g/dl). Delayed-type hypersensitivity, CD4 and CD8 counts, anti-tetanus toxoid antibody levels, erythrocyte complement receptor 1 expression, and lymphocyte proliferative responsiveness were measured. The observation period was 1 yr, and the trial was open label. Target hemoglobin was achieved and maintained in both groups. Significantly improved cutaneous reactivity was seen in the normal hemoglobin group (P = 0.003). The prevalence of anergy decreased in the normal hemoglobin group (from 60 to 20%) but increased in the anemic hemoglobin group (from 57 to 86%). The anemic hemoglobin group had higher CD8 counts compared with baseline (P = 0.0001) and compared with the normal hemoglobin group (P = 0.038). Both groups had significant increases in tetanus toxoid antibody levels after vaccination but without significant differences between groups. The anemic hemoglobin group had a progressive increase in erythrocyte complement receptor 1 levels compared with baseline (P = 0.002) and relative to the normal hemoglobin group (P = 0.023). There was no consistent pattern of altered proliferative responsiveness of lymphocytes. The data suggest that certain aspects of immune function, particularly delayed-type hypersensitivity, may be improved in HD patients by normalization of hemoglobin through the administration of increased doses of erythropoietin.