发色三肽底物测定血浆蛋白C活性及临床应用

建立了血浆蛋白Ｃ活性水平测定的发色三肽底物法，并对临床５６例病例进行了检测。以正常人血浆蛋白Ｃ活性平均值为１００％作对照，３０例肝硬化病人血浆蛋白Ｃ活性平均为（６１．４±２７．３）％；７例慢性肾功能不全病人血浆蛋白Ｃ活性平均为（５１．２±２５．７）％，１８例正常晚期妊娠妇女血浆蛋白Ｃ活性平均为（１３８．０±２３．５）％。与正常对照组比较，结果差异均有显著性。在的１例血栓性血小板减少性紫癜（ＴＴＰ）     

Role of β2 integrins in glomerular leucocytes in Henoch-Schoenlein purpura nephritis: an age-matched comparative study with immunoglobulin A nephropathy

SUMMARY: A comparative immunohistological study was performed for the glomerular deposition of complements (C1q and C3c), fibrin/fibrinogen‐related antigen (FRA), the expression of intercellular adhesion molecule‐1 (ICAM‐1), and the infiltration of leucocytes bearing β₂ integrins (leucocyte function associated antigen‐1 (LFA‐1), complement receptor 3 (CR3) and complement receptor 4 (CR4)) on renal biopsy specimens from 49 cases with Henoch‐Schoenlein purpura nephritis (HSPN), and 49 age‐matched cases with immunoglobulin A nephropathy (IgAN). the glomerular expression of ICAM‐1 was signifcantly correlated with the glomerular infiltration of leucocyte function associated antigen (LFA)‐1⁺ leucocytes in both diseases, and with that of CR3⁺ leucocytes in HSPN. the expression of ICAM‐1 was closely localized with the infiltration of LFA‐1⁺ leucocytes in the study with double immunostaining. the incidence and intensity of glomerular deposition of FRA were significantly higher in HSPN than in IgAN (P< 0.001), and those of C3c were significantly lower in HSPN than in IgAN (P< 0.001). the glomerular deposition of FRA was significantly correlated with the glomerular infiltration of CR4⁺ leucocytes in HSPN (P<0.05) but not in IgAN. In contrast, the glomerular deposition of C3c was significantly correlated with the glomerular infiltration of CR4⁺ leucocytes in IgAN (P<0.05), but not in HSPN. Studies with double immunostaining revealed a close association of CR4⁺ leucocytes with FRA deposition in HSPN and with C3c deposition in IgAN, respectively. the number of glomerular leucocytes bearing β₂ integrins was significantly correlated with urinary protein at the time of renal biopsy in both diseases. These results suggested the differential roles of β₂ integrins in the induction of glomerular injury in HSPN and IgAN. the ICAM‐1/LFA‐1 interaction may commonly be involved in the glomerular infiltration of leucocytes in both diseases. the ICAM‐1/CR3 interaction may be involved only in HSPN. Complement receptor 4 may function as a fibrin/fibrinogen receptor in HSPN, while CR4 may function as a complement receptor in IgAN.     

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...     

过敏性紫癜急性期血液流变性改变及其临床意义

为了研究过敏性紫癜（ＨＳＰ）急性期血液流变性改变及其临床意义，采用ＮＸＥ－Ｉ型锥板式粘度计，测定了２１例ＨＳＰ急性期血液流变学各项指标。结果表明，ＨＳＰ组全血粘度、血浆粘度、红细胞聚集性增多显著高于对照组；氧释放系数ＯＤ值较对照组明显降低；ＨＳＰ患儿红细胞压积、血小板计数、血清ＩｇＡ和ＩｇＭ呈明显升高。提示ＨＳＰ时存在明显的血液流变性异常，其发生与血液浓缩、高Ｉｇ血症等因素有关。及时治疗ＨＳＰ高粘滞血症对减轻症状、防止复发是十分有益的     

Three Kinds of Foamy Cells in the Spleen: Comparative Histochemical and Ultrastructural Studies

By light and electron microscopy, we observed foamy cells in the spleens from a patient with hemolytic anemia due to red cell adenosine deaminase (ADA) overproduction, a patient with rheumatoid arthritis (RA) treated with gold, and patients with idiopathic thrombocytopenic purpura (ITP)

The foamy cells associated with red cell ADA overproduction were essentially similar to Gaucher-like cells described in patients with thalassemia, and it was suggested that the accelerated destruction of red cells was one of the factors responsible for the development of foamy cells. Foamy cells in ITP and RA were closely associated with an increased destruction of platelets in the spleen. Morphologic transitions between phagocytosed platelets and myelinlike materials were traced in these disorders. In RA, however, foamy cells were heterogeneous from an ultrastructural standpoint, with different cytoplasmic inclusions. In addition to myelinlike materials, dense bodies, vacuoles with flocculent materials, and gold were noted in most of foamy cells. As gold compounds are known to inhibit lysosomal enzymes, we surmise that an acquired disturbance in lysosomal digestion is partially responsible for the accumulation of intermediate metabolites.

In the pathogenesis of foamy cells associated with blood cell dyscrasia, the accelerated destruction of blood cells and/or acquired disorders in catabolic pathways within the macrophages are suggested to be the underlying mechanism of an intralysosomal accumulation of incompletely degraded cellular debris.     

Study of the arterial vascularisation of the medial tibial condyle in the fetus

Summary Varus deformity of the knee is common in young children who have suffered from fulminating purpura. This study was directed at the anatomic features of the vascularisation of the upper end of the tibia that might account for such deformation. It was based on the dissection of 28 anatomic specimens prepared by injection of Indian ink into the vascular trunk. 16 specimens were diaphanised for better analysis of the intracartilaginous distribution of the vessels. The study showed that the vascularisation of the medial condyle of the tibia is poor and of terminal nature, which may explain the occurrence of ischemic growth disorders following fulminating purpura.

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Etude de la vascularisation artérielle du condyle médial du tibia chez le foetus

Résumé Les déformations en varus du genou chez les jeunes enfants ayant présenté un purpura fulminans sont fréquentes. Ce travail a pour objet de rechercher les caractéristiques anatomiques de la vascularisation de l'extrémité supérieure du tibia qui peuvent expliquer ces déformations. L'étude porte sur la dissection de 28 pièces anatomiques préparées par injection de l'axe vasculaire à l'encre de Chine. Pour mieux analyser la répartition intra-cartilagineuse des vaisseaux, 16 pièces ont été diaphanisées. Cette étude montre que la vascularisation du condyle médial du tibia est pauvre, de type terminal, ce qui peut expliquer la survenue de troubles de croissance ischémiques dans les suites d'un purpura fulminans.

     

IgG inhibits the increase of platelet-associated C3 stimulated by anti-platelet antibodies

We investigated the increase of platelet-associated IgG and complement component 3 (C₃) caused by the in vitro action of anti-platelet MoAbs, and the effect of mouse and human IgG on these events. Anti-glycoprotein IIb/IIIa and anti-glycoprotein Ib MoAbs caused a slight increase of C₃, but not of platelet-associated IgG. In contrast, anti-CD9 and anti-Fcγ II receptor MoAbs caused an increase of both platelet-associated C₃ and IgG. In particular, three MoAbs which activated the complement system caused a marked increase of C₃. When platelet-rich plasma was treated with aspirin and prostaglandin E₁ before incubation with antibodies, the increase of platelet-associated IgG was inhibited in all cases. In contrast, the increase of platelet-associated C₃ was scarcely influenced. These results suggest that the binding to platelets of platelet-activating antibodies caused the increase expression of IgG molecules on the platelet surface and a possible increase of platelet-associated IgG. However, the increase of platelet-associated C₃ appeared to depend on specific characteristics of the antibodies tested, such as a complement-activating effect. In addition, intact mouse or human IgG inhibited the increase of platelet-associated C₃ caused by complement-activating antibodies, while F(ab')₂ mouse or human IgG had no such effect. This suggested that the Fc portion of IgG may block the increase of C₃ mediated by anti-platelet antibodies.     

High dose gammaglobulin therapy in adults with idiopathic thrombocytopenic purpura (ITP) clinical effects

Summary This study of the effect of high-dose intravenous gammaglobulins with one or two courses of therapy in 18 adults with idiopathic thrombocytopenia purpura showed a platelet rise in thirteen patients. The highest response rates were seen in splenectomized adults. In chronic patients the response was transient only. If therapy was effective, increased values of platelet-associated IgG were reduced, while shortened platelet survival times were prolonged. There was no influence of high-dose gammaglobulins on platelet function. Different 7S-preparations such as -propiolactone modified Ig, pH 4 treated Ig and reduced and alkylated Ig have comparable effects.Supported by the Deutsche Forschungsgemeinschaft (Mu 277/9-4)     

单病种护理质量控制在小儿特发性血小板减少性紫癜中的应用

目的 探讨单病种护理质量控制在小儿特发性血小板减少性紫癜中的应用效果。方法 将我院2020年1月—2021年6月收治的60例特发性血小板减少性紫癜患儿按照随机数字表法进行编号和分组,其中单号患儿纳入为对照组(30例),接受传统护理模式;双号为观察组(30例),按照小儿特发性血小板减少性紫癜护理质量控制标准分阶段实施评估,制定护理计划,落实护理措施。比较两组患儿:⑴临床治疗相关指标(治疗有效率、平均住院时间及住院费用);⑵护理水平相关指标(护理质量、护理人员业务能力);⑶护理效果相关指标(护理依从性、健康教育知晓率及护理满意度)。结果 ⑴观察组治疗总有效率为80.00%(24/30)高于对照组53.33%(16/30)(χ2=4.800,P=0.028);观察组住院时间及住院费用少于对照组(P<0.001);⑵观察组护理质量及护理人员业务能力高于对照组(P<0.001);⑶护理后,观察组护理依从性、健康教育知晓率及护理满意度高于对照组(P<0.006～0.010)。结论 实施单病种护理质量控制标准管理,有利于提高患儿临床治疗效果及护理效果,提升护理水平,缩短住院时间及费用。     

抗中性粒细胞胞浆抗体对过敏性紫癜的检测意义

目的：探讨抗中性粒细胞胞浆抗体（ANCA）在过敏性紫癜（HSP）患儿中的检测意义。方法：应用间接免疫荧光法（IIF）和酶联免疫法（ELISA）测定45例过敏性紫癜患儿抗中性粒细胞胞浆抗体的水平和特异性抗原。结果：间接免疫荧光法检测紫癜肾,IgA型ANCA阳性率显著高于过敏性紫癜无肾脏损害者及正常对照组,二者间有显著性差异（P＜0．05）,而过敏性紫癜无肾脏损害者IgA型ANCA阳性率与正常对照组无显著性的差异（P＞0．05）;ELISA法检测过敏性紫癜患儿特异性抗原PR3、MPO,均为阴性。结论：IgA型ANCA定性检测可反映过敏性紫癜肾脏损害的情况,其特异性抗原与其它类型血管炎IgG型ANCA特异性抗原 不同。