过敏性紫癜的心肌损害

目的观察儿童过敏性紫癜并心肌损害的发生率及临床特点。方法回顾性分析93例过敏性紫癜患儿并心肌损害的临床资料，对其发生率、临床表现及其他系统损害的关系和预后进行分析。结果497例过敏性紫癜患者中有93例（18．71％）有心肌受损，主要表现为心肌酶学异常和（或）心电图异常。所有病例应用肾上腺皮质激素治疗后均治愈。结论过敏性紫癜合并心肌损害是临床较常见的表现，早期识别并及时应用激素治疗及对症处理可避免严重并发症的发生，提高治疗效果。     

从热、虚、瘀辨证论治小儿过敏性紫癜

从热、虚、瘀三个方面论述了小儿过敏性紫癜的病因病机特点，抓住病因病机的核心，确定了热伤血络、阴虚火旺、气不摄血三个本证证型和风热、热毒、湿热、血瘀四个标证证型。采用本证和标证相结合的方法对小儿过敏性紫癜进行辨证论治，并从理论上探讨了该方法的科学性和实用性。     

免疫性血小板减少性紫癜辨证分型与血小板表面相关抗体及T淋巴细胞亚群的关系

为探讨免疫性血小板减少性紫癜（ITP）中医辨证分型与免疫机理的关系,将ITP患者中医辨证分型结合血小板相关抗体（PAIg）及T淋巴细胞亚群进行分析。发现作为本病主要抗体的PAIgG脾肾阴亏型明显高于脾虚失统型（P<0.01）;OKT₈⁺值亦显著高于脾虚失统型（P<0.05）,OKT₄⁺/OKT₈⁺比值则明显低于脾虚失统型（P<0.05）。提示脾肾阴亏型的免疫损伤程度较脾虚失统型严重。     

Subacute (acute,persistent) thrombotic microangiopathy

Six patients with prolonged acute courses of thrombotic microangiopathy are reviewed. These patients had in common courses of acute disease requiring plasma support for more than 3 months, with subsequent complete remission. Plasma support requirements may be prodigious, and the acute course may require more than 100 plasma exchanges before a stable remission is achieved. These patients appear to represent a subset of thrombotic microangiopathy distinct from the more common acute T.T.P. course, which resolves in 3–6 weeks, and the chronic relapsing pattern, which may have a short or prolonged acute course. © 1992 Wiley-Liss, Inc.     

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.     

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.