Related factors analysis of poor prognosis in children with Henoch-Sch?nlein purpura nephritis

Objective To explore the related factors of poor prognostis in children with Henoch-Sch?nlein purpura nephritis (HSPN), and provide reference for predicting and improving the prognosis of children with HSPN. Methods The clinical and pathological data of children with HSPN hospitalized in the Department of Nephrology, Children's Hospital Affiliated to Capital Institute of Pediatrics from May 2007 to June 2019 were retrospectively reviewed. According to the prognosis, the patients were divided into complete remission group and persistent abnormal group. Results (1) Among 108 cases, there were 73 males and 35 females, with the onset age ranging from 5 to 16 years and average age of (9.5±2.8) years. The interval time from the first clinic in our hospital to the last follow-up was 2-131 months, with average of 24.8 months. Renal involvement occurred in the course of Henoch-Sch?nlein purpura from 1 day to 51 months, and the renal biopsy time was 5 days to 60 months after renal involvement. (2) Hematuria with proteinuria type and nephrotic syndrome type were predominant, and there was no significant difference between the two groups. The proportion of gross hematuria in the persistent abnormal group were significantly higher than that in the complete remission group (52.6% vs 31.4%, χ2=4.659, P=0.031). There were significant differences in serum creatinine and urea between the two groups (both P＜0.05). The proportion of hyperuricemia in the persistent abnormal group was higher than that in the complete remission group (39.5% vs 21.4%, χ2=3.998, P=0.046). After clinical treatment, though there was no significant difference in proteinuria between the two groups at the beginning of the disease, the negative transformation rate of proteinuria in the complete remission group was higher than that in the persistent abnormal group after 3 months(55.7% vs 34.2%, χ2=4.562, P=0.033). (3) According to International study of Kidney Disease in Children (ISKDC) pathology classification, 14 cases (36.8%), 21 cases (55.3%), 3 cases (7.9%) withⅡ, Ⅲ, Ⅳ level in the persistent abnormal group and 21 cases (30.0%), 49 cases (70.0%), 0 case with Ⅱ,Ⅲ, Ⅳ level (70.0%) in the complete remission group after (20.16±24.86) months of follow-up, and the difference between the two groups was not statisticcally significant (Z=-0.135, P=0.892). According to the Oxford Classification of IgA nephropathy, 36(33.3%) children had tubule-interstitial lesions (T1, 26%-50% tubular atrophy or interstitial fibrosis), and the proportion in the persistent abnormal group was significantly higher than that in the complete remission group (50.0% vs 24.3%, Z=-2.695, P=0.007). (4) Compared with T0 (0-25% tubular atrophy or interstitial fibrosis), the incidence of gross hematuria and hyperuricemia in the T1 tubule-interstitial lesion were both higher than that (respectively 63.9% vs 27.8%, χ2=13.061,P＜0.001; 38.9% vs 22.2%, χ2=3.983, P=0.046). (5) Multivariate logistic regression analysis showed that renal tubule-interstitial lesion was a risk factor for poor prognosis of HSPN (OR=2.580, 95%CI 1.055-6.310, P=0.038). Conclusions Renal tubule-interstitial lesion is a risk factor for the persistent abnormal of HSPN. Gross hematuria and hyperuricemia are related to tubule-interstitial lesions.     

腹型过敏性紫癜45例临床分析

背景与目的:过敏性紫癜(HSP)是一种小血管受累的多系统血管炎性疾病,可累及皮肤、关节、胃肠道、肾脏等多个部位,临床表现缺乏特异性,在胃肠道症状先于紫癜样皮疹出现的患者中,诊断更加困难。本研究总结分析腹型HSP的临床特点和诊治经验,旨在提高对该病的早期认识。方法:回顾性分析2012年1月-2018年1月在中南大学湘雅医院收治的以腹痛为主要表现的45例HSP患者的临床资料。结果:45例腹型HSP患者中男34例,女11例,男女比3.1:1;平均发病年龄(31.1±18.6)岁,以18岁以下青少年居多;最常见的发病季节为秋冬两季。24例(53.3%)以腹痛为首发表现,部位以中上腹部多见,以阵发性绞痛为主,14例(31.1%)以紫癜样皮疹为首发表现,5例(11.1%)腹痛和皮疹同时出现,其他伴随症状有恶心、呕吐、便血、腹胀以及双膝关节疼痛等。实验室检查中,白细胞计数升高32例(71.1%),其中以中性粒细胞升高为主;尿蛋白阳性13例(28.9%)、尿红细胞阳性13例(28.9%)、白蛋白降低35例(77.8%)、C反应蛋白升高(CRP)27例(60.0%)、大便隐血试验阳性33例(73.3%)、免疫球蛋白A(IgA)升高4例(16.0%);32例行食物不耐受检查,其中含有食物过敏原19例(59.4%),以异种蛋白为主。33例(73.3%)行胃镜检查,病变主要累及十二指肠球降部以及胃窦,26例(57.8%)行肠镜检查,病变主要累及回肠末端,3例(6.7%)行小肠镜检查,病变主要累及回肠中下段;内镜下主要表现为胃肠黏膜的充血水肿及片状糜烂,少数伴有溃疡形成。7例(15.6%)行腹部CT,均表现为肠壁增厚,少数伴有腹膜后及肠系膜淋巴结肿大。12例(26.7%)行病理活检,提示为黏膜非特异性炎症,部分可见嗜酸性粒细胞和浆细胞浸润。所有患者诊断明确后给予综合治疗,40例(88.9%)予以糖皮质激素治疗,消化道症状和皮疹均可得到明显缓解。6例(13.3%)复发,复发时间1~24个月不等。结论:腹型HSP皮疹往往晚于胃肠道症状出现,缺乏特异性症状和体征,容易误诊,临床上可以早期行胃肠镜检查,以提高对该疾病的诊断及认识。     

参麦注射液对特发性血小板减少性紫癜患者外周血调节性T细胞的影响

目的：探讨参麦注射液对特发性血小板减少性紫癜患者外周血CD4＋CD2＋5 Treg细胞计数和分泌因子水平的影响。方法选取特发性血小板减少性紫癜患者78例,以随机数字表法分为两组;对照组常规泼尼松治疗,观察组在对照组的基础上加用参麦注射液。对比临床疗效,并分别于治疗前后抽取患者空腹静脉血,以流式细胞法测定CD4＋CD＋25FoxP3＋Treg细胞计数,以ELISA试剂盒测定IL-2、IL-4以及IFN-γ血清含量。结果观察组显效率56．41％、总有效率92．31％,均显著高于对照组的30．77％和71．79％（χ^2＝4．222、3.933,均P ＜0．05）。治疗后观察组和对照组患者外周血 CD4＋CD2＋5 FoxP3＋Treg 细胞计数比30．66％比24.10％、7．49％比5．15％,血清IL-2、IL-4以及IFN-γ血清含量（15．6±4．3） ng／mL比（10．3±3．1） ng／mL、（9．5±2．7）ng／mL比（13．9±3．4）ng／mL、（37．1±4．3）ng／mL比（31．2±4．9）ng／mL,较治疗前均有不等程度的升高,且观察组显著高于对照组（χ^2＝4．329、4．012、4．568、4．105,均P＜0．05）。结论 CD4＋CD2＋5调节性T细胞数量减少或功能缺陷可能参与了特发性血小板减少性紫癜的发病进程,参麦注射液可能能够通过该环节发挥治疗作用。     

Purpura rhumatoïde de l’adulte

Henoch-Schönlein purpura is a systemic vasculitis of the small vessels characterized by perivascular leucocyte infiltrates. It is an immunoglobulin A-related immune complex-mediated disease involving the skin, the joints and the gastrointestinal system. Renal disease may sometimes be associated to these clinical manifestations. Prevalence of the nephritis is highly variable, depending on the series. More rarely, other organs such as the lungs, the heart or the nervous system may be involved. The clinical diagnosis is confirmed by histopathology of the skin (leukocytoclastic vasculitis) and kidney (endo-capillary proliferative glomerulonephritis), showing IgA deposits in these tissues. Short-term prognosis depends on the severity of digestive involvement, but long-term prognosis depends on the renal disease. Recent publications of pediatric and adult series show that the chronic renal failure may progress, sometimes more than ten years after the initial flare. Treatment is usually supportive. The benefit of more specific treatments (corticosteroids or immunosuppressive drugs) in severe visceral forms (usually abdominal or kidney) has not yet been established.     

槐杞黄颗粒治疗儿童紫癜性肾炎临床疗效及对免疫功能的影响

目的 观察槐杞黄颗粒治疗儿童紫癜性肾炎的临床疗效及其对患儿免疫功能的影响.方法 选取山西省儿童医院肾内科收治的紫癜性肾炎患儿120例为研究对象,按照随机数字表法分为常规综合治疗的对照组和联合槐杞黄颗粒治疗的观察组,每组各60例.2组均连续治疗随访6个月,并于治疗后1年进行回访.观察2组患儿临床治疗效果,治疗前后血白蛋白...     

肾紫癜误诊11例报告

目的探讨肾紫癜误诊原因,提高肾紫癜诊治水平。方法回顾性分析1973年6月～2005年3月间肾紫癜误诊11例临床资料。结果11例患者术前全部误诊,其中6例行肾切除术,3例经肾穿刺活检,2例经后腹腔镜探查并活检。所有病例均经病理检查证实为肾紫癜。结论肾紫癜较为少见,其临床表现不典型,极易误诊,临床医师应予高度重视。对疑似病例可行肾穿刺活检或后腹腔腹腔镜探查并活检可减少肾的切除。     

Update on vasculitis: an overview and dermatological clues for clinical and histopathological diagnosis – part I

The term vasculitis refers to the inflammation of vessel walls. It may range in severity from a self-limited disorder in one single organ to a life-threatening disease due to multiple organ failure. It has many causes, although they result in only a few histological patterns of vascular inflammation. Vessels of any type and in any organ can be affected, a fact that results in a broad variety of signs and symptoms. Different vasculitides with indistinguishable clinical presentations have quite different prognosis and treatments. This condition presents many challenges to physicians in terms of classification, diagnosis, appropriate laboratory workup, and treatment. Moreover, it compels a careful follow-up. This article reviews the Chapel-Hill 2012 classification, etiology, recent insights in pathophysiology, some important dermatological clues for the diagnosis and summarizes treatment of some of these complex vasculitis syndromes.     

免疫性血小板减少症老年患者个体化治疗的研究进展

免疫性血小板减少症(ITP)是一种由体液免疫及细胞免疫异常共同介导的自身免疫性疾病,血小板破坏增多及生成减少共同参与其发病.既往普遍认为儿童及育龄期妇女为ITP的高发人群,但近年来相关文献报道,随着年龄的增长,ITP的发病率不断增高,年龄≥60岁的老年人已经成为新的ITP高发人群.由于ITP老年患者合并症多,对治疗反应及耐受性较年轻患者差,更易发生严重的出血症状,病死率增高.目前,针对ITP老年患者的经典治疗方法包括:糖皮质激素、静注人免疫球蛋白(IVIG)、脾切除术、免疫抑制剂等,但是约1/3的ITP老年患者接受治疗后,疾病仍迁延不愈.随着相关研究的深入,利妥昔单抗、血小板生成素受体激动剂(TPORA)罗米司亭与艾曲波帕、新型免疫抑制剂霉酚酸酯(MMF)等新药在治疗ITP老年患者中均显示出较好的疗效,然而根据ITP老年患者的具体情况对其实施个体化治疗变得越来越重要.笔者拟对ITP的个体化治疗方法及其最新研究进展,结合ITP老年患者这一特殊群体的具体情况进行综述.     

血清sHLA—G在慢性特发性血小板减少性紫癜患者中的表达及意义

【目的】探讨血清可溶性的人类白细胞抗原G（sHLA-G）在慢性特发性血小板减少性紫癜（ITP）患者中的表达及临床意义。【方法】以2010~2015年本院收治的80例慢性ITP患者为观察对象（ITP组）,同时选取50例健康成年人作为对照组。比较两组血清sHLA—G、炎症细胞因子水平,分析sHLA—G与血小板计数（PLT）、骨髓增生程度和炎症细胞因子水平的相关性。【结果】治疗前IPT组的血清siILA—G、白细胞介素-6（IL-6）、白细胞介素-17（IL-17）和肿瘤坏死因子-α（TNF-α）水平高于对照组（P〈0．05）,治疗后IPT组患者的上述指标水平较治疗前降低（P〈0．05）,但仍高于对照组（P〈0．05）;不同PLT的患者sHLA—G水平存在显著差异：PLT越低,sHLA-G水平越高（P〈0．05）;不同骨髓增生程度下的sHLA—G水平差异具有显著性：骨髓增殖越活跃,sHLA—G水平越高（P〈0．05）;sHLA—G水平与IL-6、IL-10和TNF-α水平显著正相关（P〈0．05）。【结论】血清sHLA-G在慢性ITP中水平较高,且与PLT、炎症细胞因子水平和骨髓增殖程度密切相关。     

特发性血小板减少性紫癜患者脾脏CD5+和CD5-B细胞共同参与血小板自身抗体的产生

目的：研究慢性特发性血小板减少性紫癜（ITP）患者脾脏CD5^ B细胞水平的变化及CD5^ 和CD5^-B细胞与血小板膜糖蛋白（GP）特异性自身抗体产生的关系，以识别致病B细胞亚群。方法：应用双色流式细胞仪检测8例慢性ITP患者脾脏CD5^ B细胞水平。选择4例血浆抗GPⅡb/Ⅲa和抗GP Ⅰb/Ⅸ抗体双阳性ITP切脾患者，应用Ficoll密度梯度离心及花环形成分离法分离脾脏B淋巴细胞，继而采用镝产珠分选法分选、纯化CD5^ B细胞和CD5^-B细胞，并分别进行体外培养，应用改良MAIPA法检测血浆和细胞培养上清液的血小板特异性抗体。结果：ITP患者脾脏CD5^ B细胞水平圈晨自身免疫性疾病患者略有增高，二者之间差异无统计学意义。CD5^ B细胞水平与患者血小板计数无相关性。4例血浆抗GPⅡb/Ⅲa抗体和抗GPⅠb/Ⅸ抗体双阳性。另外1例CD5^ B细胞培养液抗GPⅡb/Ⅲa抗体阴性，抗GPⅠb/Ⅸ抗体阳性;CD5^-B细胞培养液抗GPⅡb/Ⅲa抗体和抗GPⅠb/Ⅸ抗体双阳性。结论：脾脏CD5^ 和CD5^-B细胞 均可产生血小板GP特异性自身抗体，抗体产生种类和滴度无明显差异。提示二者共同参与了ITP的发病过程。