Successful treatment of hemorrhagic bullous Henoch‐Schonlein purpura with intravenous immunoglobulins

Henoch‐Schonlein purpura (HSP) is the most common systemic vasculitis in childhood. There is no consensus about the management for isolated cutaneous manifestations in HSP. We describe a case of HSP presenting with severe skin lesions that did not respond to standard therapy with corticosteroids. The 11‐year‐old child was treated with intravenous immunoglobulins, which induced rapid and persistent resolution of symptomatology.     

儿童过敏性紫癜及紫癜性肾炎的凝血象及血尿D-二聚体改变

目的　探讨过敏性紫癜及紫癜性肾炎患儿体内否存在高凝状态及其严重程度。方法　使用血液凝固时间自动分析仪和胶体金免疫渗透法对 4 2例过敏性紫癜和紫癜性肾炎以及 2 0例正常对照儿童进行凝血象 (PT、TT、APTT、FIB)和血、尿D -二聚体 (D -D)测定。结果　过敏性紫癜及紫癜性肾炎患儿体内存在不同程度的高凝状态 ,4 2例患儿中TT缩短 2 0例 (4 7 6 % ) ,PT缩短 2 2例 (5 2 4 % )、APTT缩短 18例(4 2 85 % )、血FIB增高 18例 (4 2 8% )、血D -D增高 18例 (4 2 8% )、尿D -D增高 2 0例 (4 7 6 % )。而对照组无1例异常。在对照组、过敏紫癜组、紫癜性肾炎组相互比较中 χ2 >7 79,均有显著性差异。结论　患儿体内外凝血系统被激活 ,为临床上指导用药 ,判断病情提供了理论依据。     

104例紫癜性肾炎临床病理及免疫病理的研究

通过对１０４例紫癜性肾炎患者临床、病理及免疫病理的分析，探讨三者之间关系。方法对１０４例１２－４０岁ＨＳＰＮ患者进行临床分型，病理分级和免疫产现理分型，分析三者间内在联系，同时对间质面积及间质内ＣＤ４＾＋，ＣＤ＾＋８细胞进行了测定。结果：１０４例ＨＳＰＮ患者中蛋白尿、镜下血尿和肉眼血尿的发生率分别为９９％、９４．２％、４３．３％；消化道症状及关节痛的发生率为４３．３％和２８     

Chylothorax in Henoch-Schonlein purpura: a case report and review of the literature

Henoch-Schonlein purpura (HSP) is the most common acute vasculitis in the pediatric population, with an incidence of 10-14 per 100,000. The classic presentation of this disorder includes erythematous papules followed by palpable purpura in the lower extremities, trunk, and face, arthralgia or arthritis, abdominal pain, gastrointestinal bleeding, and nephritis. While renal abnormalities in HSP are common, the classic pulmonary manifestations, such as hemorrhage and pneumonitis, are thought to be infrequent. Subclinical pulmonary manifestations, including diffusion defects and radiographic anomalies, seem to be quite frequent in patients with HSP but are not commonly reported. Other respiratory manifestations include pleural effusion and chylothorax, but these are rarely mentioned in the literature. Chylothorax was only reported once in an adult patient with HSP in whom the mechanism of formation was demonstrated to be secondary to transdiaphragmatic passage of chylous fluid from the peritoneal cavity. Here we describe an 8-year-old girl with HSP, nephrotic syndrome, and chylothorax, and we report the results of a review of the literature regarding respiratory complications in HSP. The present case is the first pediatric patient reported with HSP and chylothorax. The therapeutic measures utilized were effective in resolving her edema, ascites, and chylothorax, and we advocate the use of these measures as first-line therapy in future patients with similar complications from HSP.     

HLA class 1 associations in Henoch Schonlein purpura: increased and decreased frequencies

Henoch Schonlein purpura (HSP) is the most common vasculitis of childhood. Susceptibility to HSP and associated clinical heterogeneity in HSP may be conferred by a number of genetic loci, including the major histocompatibility complex. We aimed to investigate the implications of the human leukocyte antigen (HLA) class 1 alleles in susceptibility to HSP and determine the possible associations with renal, gastrointestinal (GI), and joint manifestations of the disease. 110 children with HSP (66 boys, 44 girls) and 250 unrelated healthy controls were enrolled in the study. The mean age was 8.65 ± 3.59 years. HSP was diagnosed on the basis of clinical and laboratory data according to the American College of Rheumatology classification. The diagnosis was supported with skin and/or kidney in most of the patients. Clinical and laboratory findings revealed: skin involvement in 110 (100%), joint manifestations in 82 (74.5%), GI symptoms in 58 (52.7%), and hematuria and/or proteinuria in 36 (32.7%) patients. HLA class 1 alleles were identified by DNA amplification, hybridized with specific primer sequences. Comparison of frequencies between patients and controls were made by using the Fisher’s exact test. Odds ratio (OR) was used as the measure of association. HLA A2, A11, and B35 antigens showed an increased risk for predisposition to HSP (OR = 1.714, 95%CI = 1.088–2.700, p = 0.020; OR = 2.185, 95%CI = 1.289–3.703, p = 0.003; and OR = 2.292, 95%CI = 1.451–3.619, p = 0.000, respectively), while HLA A1, B49, and B50 antigens revealed decreased risk for predisposition to HSP (OR = 4.739, 95%CI = 1.828–12.345, p = 0.001; OR = 3.268, 95%CI = 0.955–11.236, p = 0.047; and OR = 7.462, 95%CI = 0.975–55.555, p = 0.024, respectively). Considering the renal involvement and severity of proteinuria, there was no association with HLA class 1 alleles. Our results suggest that the increased frequency of HLA A2, A11, and B35 alleles in unselected pediatric HSP patient population and miscarrying of HLA A1, B49, and B50 could be considered as a risk factor for susceptibility to HSP.     

中西医结合治疗小儿紫癜性肾炎28例观察

目的：观察中西医结合治疗小儿紫癜性肾炎的临床疗效。方法：将58例紫癜性肾炎随机分为西医组30例和中西医结合组28例。西医组常规应用肾上腺皮质激素、维生素C、潘生丁等治疗,中西医结合组在西医组用药的基础上加用益肾健脾活血汤治疗。治疗4周观察前后24h尿蛋白定量、尿红细胞计数变化。结果：中西医结合组在减少尿蛋白量及红细胞计数和疗效方面均优于西医组,差异有统计学意义（P〈0．01,P〈0．05）。结论：采用中药益肾健脾活血汤结合西药治疗小儿紫癜性肾炎具有良好疗效。     

过敏性紫癜患儿可溶性血管细胞黏附分子-1、免疫球蛋白A抗内皮细胞抗体检测的意义

目的探讨可溶性血管细胞黏附分子-1(SVCAM-1)、IgA抗内皮细胞抗体(IgAAECA)在过敏性紫癜(HSP)诊断中的价值及两者的关系。方法采用ELISA检测55例HSP患儿(其中急性期40例,恢复期15例),20例健康儿血清SVCAM-1水平,应用荧光免疫印片法检测IgAAECA阳性率。结果HSP肾炎组SVCAM-1较非肾炎组明显升高,且急性期高于恢复期,差异均有显著意义(P均<0.01)。HSP肾炎组IgAAECA阳性率明显高于无肾炎组,IgAAECA阳性组SVCAM-1高于阴性组,差异均有显著意义(P均<0.01)。结论SVCAM-1、IgAAECA参与HSP的发病过程,可反映疾病进展程度。     

过敏性紫癜发病机制的研究进展

过敏性紫癜在临床上发病率仍然很高，合并肾损害将严重影响患者的生存质量，而其发病机制目前尚未完全阐明，因此其发病机制仍然是目前研究的热点。近年众多学者对该病发病机制的研究取得较大进展，发现一部分因素T细胞功能改变、细胞间粘附因子-、P选择素、内皮素、一氧化氮、血管性假血友病因子、D-二聚体、基因在过敏性紫癜的发病中起着重要的作用。本文就目前过敏性紫癜发病机制的研究进展作一综述。     

儿童紫癜性肾炎血清、尿液VCAM-1水平及临床意义

目的：探讨紫癜性肾炎（HSPN）患儿的血清、尿液可溶性血管细胞黏附分子-1（sVCAM-1）水平变化及临床意义。方法：过敏性紫癜（HSP）患儿50例,按有无肾脏累及分为HSPN组（30例）和HSP无肾受累（NO—HSPN）组（20例）;正常对照组20例。应用ELISA法检测各组血清、尿液sVCAM-1水平,进行比较,并分析其与主要临床指标（包括24h尿蛋白、尿红细胞）的关系。结果：HSPN组和NO-HSPN组的血清sVCAM-1水平[分别为（809．79±173．32）ng／ml、（623．44±67．27）ng／ml]均高于对照组（494．79±59．84）ng／ml,P〈0．01,HSPN组的血清sVCAM-1水平高于NO—HSPN组（P〈0．01）。HSPN组的尿液sVCAM-1水平（121．24±110．83）ng／ml高于对照纽（20．61±16．76）ng／ml和NO—HSPN组（19．37±12．93）ng／ml,P均〈0．01,NO—HSPN组的尿液sVCAM-1水平与对照组比较,无统计学差异（P〉0．05）。HSPN患儿中,蛋白尿组高于无蛋白尿组（P均〈0．01）,肾病蛋白尿组高于蛋白尿组（P均〈0．05）和无蛋白尿组（P均〈0．01）;肉眼血尿组的血清sVCAM-1水平高于无血尿组,与镜下血尿组比较无统计学差异（P〉0．05）;镜下血尿组的血清sVCAM-1浓度高于无血尿组（P〈0．01）;镜下血尿组和肉眼血尿组的尿液sVCAM-1水平明显高于无血尿组（P均〈0．05）,镜下血尿组和肉眼血尿组之间无统计学差异（P〉0．05）。HSPN患儿的血清和尿液sVCAM-1水平均与24h尿蛋白量和尿红细胞量呈显著性正相关（P均〈0．01）。结论：VCAM-1可能参与了HSPN的发生、发展过程;尿液sVCAM-1的检测在监测肾脏损害方面具有较好的临床实用价值。     

Henoch-Schonlein purpura with ischaemic bowel

Henoch-Schonlein purpura is a vasculitis affecting small arterial vessels. Occasionally, cases are referred for a general surgical opinion due to bowel involvement in the form of abdominal pain with or without rectal bleeding. However, surgical intervention is rarely required. We describe a case of Henoch-Schonlein purpura in a young man who went on to develop ischaemic bowel requiring resection.