紫癜性肾炎患儿肠道菌群变化及临床意义研究

背景目前国内外关于过敏性紫癜（HSP）患儿肠道菌群变化的研究数量有限,且尚未见关于紫癜性肾炎（HSPN）患儿疾病早期肠道菌群变化的相关报道。目的 探讨HSPN患儿肠道菌群的变化及其在疾病发生、发展中的作用。方法 于2019年7—9月选取郑州大学第一附属医院儿科收治的37例HSP初治患儿作为试验组,另外同时选取12例健康志愿儿童作为对照组;并对HSPN患儿随访6个月,根据有无肾损伤进一步分为无肾损伤试验亚组13例和肾损伤试验亚组24例。收集HSP患儿与健康儿童的一般资料及粪便标本,应用高通量测序技术对所有研究对象的肠道菌群进行测序及分析,采用Alpha多样性（Shannon指数、Chao1指数、ACE指数）分析探讨样本内的微生物群落的丰度和多样性,通过主坐标分析（PCoA）来探究不同组别间群落结构的差异,利用线性判别分析及影响因子（LEfSe）分析找到组间差异显著的物种。结果 Alpha多样性分析显示,三组研究对象Shannon指数、Chao1指数、ACE指数比较,差异均无统计学意义（P>0.05）。PCoA显示,三组研究对象肠道菌群群落结构均有差异（P<0.05）;Adon...     

原发性血小板减少性紫癜患者外周血T淋巴细胞及其亚群分析

用单克隆抗体OKT_3,OKT_4及OKT_8对40例原发性血小板减少性紫癜患者外周血T淋巴细胞及其亚群进行了分析,结果表明,该病患者T抑制/杀伤细胞亚群增高。     

Study of platelet-associated immunoglobulins of IgG,IgM, IgA,and IgE classes and platelet kinetics in 33 patients with idiopathic thrombocytopenic purpura

Summary The role of platelet-associated immunoglobulins (PAIg) of four different immunoglobulin classes -IgM, IgG, IgA, and IgE- and their relation to platelet count and platelet kinetics was studied in 33 patients with idiopathic thrombocytopenic purpura (ITP). During the course of 1 year, repeated determinations of PAIg were made. The results indicate that PAIgG, PAIgM, and PAIgA are present in all ITP patients, and that autoantibodies of all three Ig classes show highly significant correlations to the platelet counts (p< 0.0001). Double logarithmic negative correlations have been found between PAIgG and platelet count (r=–0.71), PAIgM and platelet count (r=–0.84), and PAIgA and platelet count (r=–0.79). Statistical analyses using partial correlation and multiple regression methods showed that PAIgM is predominantly related to the platelet count, whereas PAIgG and PAIgA are only of secondary importance. Accordingly, a relation of PAIgM (and PAIgA) to increased liver destruction of platelets was found in kinetic studies using¹¹¹indium-labeled platelets. Taken together, these results suggest a predominant role of PAIgM in the pathogenesis of ITP.     

Management of thrombotic thrombocytopenic purpura

Daily therapeutic plasma exchange (TPE) transformed the historically fatal prognosis of acquired, anti-ADAMTS13 antibody-mediated thrombotic thrombocytopenic purpura (TTP), leading to the current overall survival rates of >80%. However, relapses occur in up to 40% of patients and refractory disease with fatal outcomes still occurs, typically within the first days of management. In this context, the introduction of rituximab has been the second major breakthrough in TTP management. Rituximab is now routinely recommended during the acute phase, typically in patients with a suboptimal response to treatment, and increasingly as frontline therapy, with high response rates in the following weeks. In more severe patients, salvage strategies typically include twice daily TPE, pulses of cyclophosphamide, as well as splenectomy in the more desperate cases. In this life-threatening and debilitating disease, relapses can be efficiently prevented in patients with a severe acquired ADAMTS13 deficiency and otherwise in remission with the use of rituximab. In the coming years, the TTP therapeutic landscape should be enriched by original strategies stemming from clinical experience and new agents that are currently being evaluated in large, international, clinical trials. Promising agents under evaluation include caplacizumab (an inhibitor of the glycoprotein-Ib/IX-Von-Willebrand factor axis), N-acetylcysteine, recombinant ADAMTS13, and anti-plasmocyte compounds.     

Successful Treatment of mesenteric vasculitis caused by henoch–Schönlein purpura with methylprednisolone pulse therapy

Although mesenteric vasculitis due to Henoch–Schönlein purpura (HSP) is relatively uncommon, it is the most life-threatening manifestation associated with high mortality. We describe a 15-year-old boy with HSP who had massive gastrointestinal bleeding and ileus but delayed onset of the purpuric rash. Abdominal ultrasonography revealed thickening of both small and large intestinal walls, and CT found prominent mesenteric vessels with comb sign and double wall of the bowel. These findings were consistent with mesenteric vasculitis and bowel ischaemia. The ischaemic intestine recovered after methylprednisolone pulse therapy and surgical intervention was avoided. Our report suggests that corticosteroid pulse therapy may help controlling HSP with massive gastrointestinal haemorrhage and ischaemic bowel due to widespread mesenteric vasculitis.     

糖皮质激素治疗对过敏性紫癜反复及远期肾损害的影响

目的 研究糖皮质激素治疗对过敏性紫癜复发及远期肾损害的作用.方法 2008年1月～2010年6月期间收集某院儿科过敏性紫癜患者128例,采用随机数字法进行随机分组,随机均分为观察组与对照组2组,观察组在常规治疗基础上予糖皮质激素治疗,对照组予常规治疗,比较2组的紫癜复发情况、随访肾损害情况.对各指标率的变化采用组间t检验,计数资料采用组间x2检验.结果 随＝期间观察组过敏性紫癜复发率45.2％ (28/62),对照组复发率80.0％(48/60),二者相比较差异有统计学意义,x2=7.878,P=0.005.观察组过敏性紫癜肾损害发生率32.3％ (20/62),对照组肾损害发生率73.3％ (44/60),二者相比较差异有统计学意义,x2=10.314,P=0.001.结论 糖皮质激素治疗能够减少HSP复发次数,并能够减少远期肾损害,需临床进一步扩大样本验证.     

不同剂量低分子肝素钙治疗儿童紫癜性肾炎的疗效分析

目的 观察不同剂量低分子肝素钙治疗儿童过敏性紫癜性肾炎的临床疗效.方法 随机将65例过敏性紫癜性肾炎患儿在常规治疗基础上加用低分子肝素钙皮下注射,并按照不同剂量分为A组21例、B组22例及C组22例3组,疗程均为10天,观察3组临床疗效和治疗前及治疗10天后的24小时尿蛋白定量、凝血指标,同时观察出血倾向、肝功能及血小板变化.结果 3个不同剂量组治疗患儿临床疗效（短期1个疗程）相近,组间比较差异无统计学意义（均P＞0.05）;在治疗10天（约1个疗程）后24小时蛋白尿、凝血功能差异亦无显著性（P＞0.05）.结论 不同剂量低分子肝素钙治疗过敏性紫癜性肾炎患儿的临床疗效无明显差异,但小剂量更加安全可靠.     

巨噬细胞移动抑制因子在紫癜性肾炎中的研究进展

近年来,人们对巨噬细胞移动抑制因子(MIF)的研究倍加关注,其作用涉及到炎症、免疫、内分泌、肿瘤及血管等多个方面。该文着重阐述了MIF的分子结构、来源及MIF在分子水平上的作用机制、生物学机制,研究发现MIF参与机体多种疾病的发生和发展,尤其以MIF-173(G/C)位点单核苷酸的多态性与疾病的关系最密切,MIF的C等位基因可能是紫癜性肾炎(HSPN)的易患基因;MIF在HSPN的发生、发展中有重要作用及研究价值,可作为治疗免疫炎性疾病的治疗靶点。     

疏肝清热法治疗糖皮质激素依赖性成人慢性特发性血小板减少性紫癜的临床观察

【目的】观察疏肝清热法治疗糖皮质激素依赖性成人慢性特发性血小板减少性紫癜(idiopathic thrombocytopenic purpura,ITP)的临床疗效。【方法】将产生激素依赖的慢性特发性血小板减少性紫癜患者中属于中医"肝郁内热,肝不藏血"证型标准的32例患者纳入临床观察,在维持原治疗方案下,加用疏肝清热方(由淮山药、柴胡、白芍、生地黄、牡丹皮、茵陈蒿、茯苓、山茱萸、枸杞子、仙鹤草、茜草、甘草等组成)治疗12周(3个月),每日2剂,水煎内服。观察治疗前后中医症候评分及实验室指标的变化情况。【结果】(1)临床疗效方面:治疗3个月后,显效10例,良效16例,进步5例,无效1例,总有效率达81.25%。(2)中医症候评分方面:治疗1个月时,中医临床症候总评分改善不明显,与治疗前比较,差异无统计学意义(P0.05);治疗2个月和完成3个月疗程时,中医临床症候总评分均有明显改善,与治疗前比较,差异均有统计学意义(P0.05或P0.01)。(3)实验室指标方面:血小板计数从治疗第4周开始升高,与治疗前比较,差异有统计学意义(P0.01)。【结论】疏肝清热方治疗糖皮质激素依赖性成人慢性特发性血小板减少性紫癜具有一定疗效,值得临床推广应用。