小儿过敏性紫癜的心电图改变

过敏性紫癜(HSP)常累及皮肤、关节、胃肠道及肾脏,近年来偶有心脏受累的文献报道。为探讨HSP的心脏改变,我们对1990年1月～1995年6月间我院收治的100例HSP患儿进行了体表心电图描记,发现异常52例,现将其分析总结如下。 1 资料和方法     

成人过敏性紫癜35例临床分析

目的 分析成人过敏性紫癜(HSP)临床特点,以提高临床诊治水平.方法 对1993年9月至2005年12月天津医科大学总医院血液科收治的35例成人HSP患者的发病特点、临床表现、相关实验室检查等方面资料进行回顾性分析.结果 成人HSP以男性及青壮年患者为主.冬季为主要发病季节.上呼吸道感染及进食鱼、虾、蟹等为主要诱因.多数患者以皮肤损害为首发表现.混合型HSP多见.所有患者在病程中均先后出现皮肤损害,双下肢为皮疹最易侵犯的部位.有关节损害者15例(42.9%).有胃肠道损害者20例(57.1%).有肾脏损害者20例(57.1%).结论 成人过敏性紫癜的临床特点有其特殊性,易发生严重肾损害和多器官受累.     

儿童过敏性紫癜56例临床分析

目的 探讨儿童过敏性紫癜的临床特点,提高该病的诊断和治疗水平.方法 回顾性分析56例过敏性紫癜住院患儿的临床资料,并予以统计分析.结果 5～12岁儿童是过敏紫癜的高发群体,男多于女.秋冬季是其高发季节.以双下肢、臀部皮肤紫癜为首发症状的达45例,占80.4％;以消化道症状为首发者9例,占16.1％;以关节为首发症状的1例,占1.8％;以肾脏表现为首发症状的1例,占1.8％.结论过敏性紫癜临床表现多样,皮肤型紫癜较为多见,腹型、关节型、肾型过敏性紫癜较少见,且首发症状常因缺乏皮肤紫癜表现而易造成误诊,值得临床关注.     

胃镜与胃肠电图联合检查在儿童腹型过敏性紫癜诊断中的应用

目的 探讨胃镜和胃肠电图联合检查在儿童腹型过敏性紫癜(HSP)诊断中的应用价值.方法 选择172例急性腹痛患儿,根据后期皮肤是否出现紫癜症状分为HSP组(112例)和对照组(60例).患儿均进行电子胃镜检查和胃肠电图检查,HSP组在确诊和治疗后6个月恢复期时进行门诊随访.结果 胃镜下HSP组在急性期均表现出不同程度消化...     

凝血酶、奥美拉唑联用治疗过敏性紫癜并消化道出血的体会

过敏性紫癜是最常见的毛细血管变态反应性疾病,以皮肤紫癜、消化道出血、关节肿胀、肾炎等症状为主要临床表现,2/3的患儿有消化道症状,表现为腹痛、呕血、便血.我科采用凝血酶联合奥美拉唑治疗消化道出血12例,效果满意.现总结并报告如下.     

过敏性紫癜在内镜下的胃、肠黏膜改变

过敏性紫癜 (ＡＰ)是临床常见病 ,我院自 1995年 1月～2 0 0 0年 12月间 ,对过敏性紫癜各种类型均做电子胃镜及结肠镜检查 ,观察各型过敏性紫癜有无食管、胃、肠黏膜病变、病变形态及分布特点 ,以提高对过敏性紫癜胃、肠黏膜病变特点的认识 ,为过敏性紫癜胃、肠黏膜病变的诊断提供依据 ,有利于指导临床治疗。一、对象和方法1.对象选择 :均为门诊及住院患者 ,依据ＡＰ的诊断标准 :①四肢皮肤分批出现对称性紫癜 ;②紫癜出现前后伴有腹痛、恶心、呕吐、便血和 /或关节肿痛及血尿等症状 ;③血小板计数、出凝血时间正常 ;④排除与症状相关的其他…     

复方丹参治疗过敏性紫癜的疗效与血小板聚集功能观察

我们应用复方丹参注射液治疗过敏性紫癜 (HSP) ,并观察其对血小板聚集功能的影响。临床资料 :5 0例 HSP皆为本院住院及门诊患儿 ,均符合HSP的诊断标准。治疗组及对照组各 2 5例 ,两组患儿在年龄、症状和并发症等方面经统计学处理无差异。治疗方法 :两组患儿均应用抗生素、肾上腺皮质激素、抗过敏等常规治疗。治疗组加用复方丹参注射液 ,按 0 .2～ 0 .5 ml/ (kg· d)加入 10 %葡萄糖液中静滴 ,每日 1次 ,连用 1周。治疗前及治疗 1周后分别检测血小板聚集功能 ,并观察皮肤紫癜消退天数、住院 (或治疗 )天数。数据以 x± s表示 ,两组资料差…     

儿童过敏性紫癜与不同类型幽门螺杆菌感染的相关性

目的: 探讨儿童过敏性紫癜( Henoch -Schonlein purpura, HSP)与不同类型幽门螺杆菌( H pylori)感染的关系.方法: 用免疫印迹法对80例HSP患儿及40例正常儿童的血清进行幽门螺杆菌分型检测. 将80例HSP患儿分为有消化系症状HSP组(51例)和无消化系症状HSP组(29例).结果: 有消化系症状HSP组患儿Ⅰ型H pylori感染占25.5%, Ⅱ型H pylori感染占52.9%; 而无消化系症状HSP组和正常对照组均无Ⅰ型H pylori感染, Ⅱ型H pylori感染分别为34.5%、12.5%; 三组的H pylori抗体分型检出率有统计学意义( P<0.001). 有消化系症状HSP组患儿的H pylori抗体阳性检出率明显高于无消化系症状HSP组和正常对照组(78.4% vs 34.5%,结论: HSP的发病可能与H pylori感染有关, 其消化系症状与Ⅰ型H pylori感染密切相关. 免疫印迹技术可应用于对H pylori感染分型诊断.     

系统性硬化症下消化道受累13例

目的分析和总结系统性硬化症(systemic sclerosis,SSc)并发下消化道受累患者的临床特点。方法回顾性分析2000年11月至2015年6月北京协和医院住院SSc并发下消化道受累患者的临床、实验室、影像学和内镜检查结果、治疗和转归。结果 SSc住院患者并下消化道受累发生率约2.3%(13/569)。13例SSc并下消化道受累患者中男∶女=1∶1.25。弥漫性皮肤型SSc 6例,局限性皮肤型SSc 7例,重叠综合征2例。SSc并下消化道受累起病平均年龄(45±18)岁,从SSc起病到出现下消化道受累中位时间为5年,92%(12/13)下消化道症状出现在SSc其他系统首发表现之后,1例SSc患者以下消化道症状为首发表现。SSc并下消化道受累临床表现以肠梗阻最常见(8/13,62%),其次是肠溃疡、失蛋白肠病、小肠细菌过度生长、肛门括约肌受累。13例患者中6例失访,另7例随访中位时间20个月,病情平稳。结论 SSc并下消化道受累并非罕见,严重者将显著影响患者生活质量。对SSc患者及时行影像学和内镜检查,有利于早期发现下消化道病变。     

甲基强的松冲击疗法对过敏性紫癜患儿血清免疫学指标的影响

2016年11月～2017年10月112例HSP患儿随机平分为对照组接受常规药物治疗,观察组加用甲基强的松冲击治疗。结果观察组关节肿痛、腹部压痛、消化道症状、皮肤紫癜消失时间、住院时间少于对照组,总有效率高于对照组(P 0. 05);治疗后观察组C3、IgA、IL-6、TNF-α水平低于对照组(P 0. 05);两组不良反应发生率相比(P 0. 05)结论将甲基强的松冲击疗法用于HSP患儿治疗中效果确切且安全性较高。     

A case of Henoch-Sch?nlein purpura with psoas muscle abscess and full-blown gastrointestinal complications]

Henoch-Sch?nlein purpura (HSP) is a vasculitis involving small vessels of skin, joints, gastrointestinal (GI) tract, and kidneys. The patients typically show palpable purpura with one or more characteristic manifestations including abdominal pain, hematuria or arthritis. HSP shows gastrointestinal symptoms in 50~85% of patients, and in 14~40% of patients GI symptoms precede purpuric rash which makes the diagnosis of HSP difficult. We present a case of Henoch-Schonlein purpura with GI bleeding, septic shock by ileal microperforation, small bowel obstruction as a result of ileal stricture and psoas muscle abscess.     

Paroxysmal drastic abdominal pain with tardive cutaneous lesions presenting in Henoch-Schönlein purpura

Henoch-Schönlein purpura (HSP) is a small-vessel vasculitis mediated by IgA-immune complex deposition. It is characterized by the clinical tetrad of non-thrombocytopenic palpable purpura, abdominal pain, arthritis and renal involvement. The diagnosis of HSP is difficult, especially when abdominal symptoms precede cutaneous lesions. We report a rare case of paroxysmal drastic abdominal pain with gastrointestinal bleeding presented in HSP. The diagnosis was verified by renal damage and the occurrence of purpura.     

腹型过敏性紫癜的临床及内镜表现

目的 总结腹型过敏性紫癜的临床和内镜特点,以加强对此病的认识。方法 对1994 年1月以来收治的197名过敏性紫癜患者病例资料进行回顾性分析,其中81名为腹型紫癜。19例次 接受了胃镜或肠镜检查。结果 腹型紫癜占紫癜病例的41.1％,其中51例(63.0％)在发病前1-3 周有明确诱因。21例以消化道症状首发,其消化道症状可早于皮损1-40 d出现。74例(91.4％)出 现腹痛,37例(45.7％)表现为消化道出血。内镜表现为胃肠道黏膜弥漫性充血水肿,广泛多发的出 血点、红斑、糜烂、溃疡,小肠病变较重。胃肠道黏膜组织病理学表现为黏膜及黏膜下层中到大量中性 粒细胞浸润、小血管壁纤维素性坏死、灶性出血、糜烂和溃疡。患者的内镜和临床病理表现的范围和 严重程度与消化道症状的程度一致。结论 41.1％的紫癜患者表现为腹型过敏性紫癜,腹型紫癜中 25.9％以消化道症状为首发。小肠病变程度重于胃或结肠。其典型的临床和内镜表现对早期诊断有 一定帮助。     

Primary biliary cirrhosis and Henoch-Schonlein purpura: report of two cases and review of the literature.

We describe, for the first time, two adult patients with primary biliary cirrhosis (PBC) who presented palpable purpuric lesions on the lower extremities and buttocks. Skin biopsy was consistent with Henoch-Schonlein purpura (HSP). One of the patients exhibited renal and gastrointestinal involvement, as well. A favourable outcome was achieved in both the patients (after treatment with prednisolone only in the patient with renal and gastrointestinal manifestations). The possible pathophysiological relationships between PBC and HSP--particularly on the significance of the IgA antimitochondrial autoantibodies--are discussed. To the best of our knowledge, these are the first two cases of HSP, which developed in PBC patients. Therefore, this case study suggests that HSP may be rarely involved in the extensive list of immune-mediated diseases associated with PBC.     

The American College of Rheumatology 1990 criteria for the classification of Henoch-Sch?nlein purpura

Criteria for identifying Henoch-Sch?nlein Purpura (HSP) and distinguishing HSP from other forms of systemic arteritis were developed by comparing the manifestations in 85 patients who had HSP with those of 722 control patients with other forms of vasculitis. By the traditional format of choosing different combinations of candidate criteria and comparing the combinations for their ability to separate HSP cases from controls, 4 criteria were identified: age less than or equal to 20 years at disease onset, palpable purpura, acute abdominal pain, and biopsy showing granulocytes in the walls of small arterioles or venules. The presence of any 2 or more of these criteria distinguish HSP from other forms of vasculitis with a sensitivity of 87.1% and a specificity of 87.7%. The criteria selected by a classification tree method were similar: palpable purpura, age less than or equal to 20 years at disease onset, biopsy showing granulocytes around arterioles or venules, and gastrointestinal bleeding. These were able to distinguish HSP from other forms of vasculitis with a sensitivity of 89.4% and a specificity of 88.1%.     

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.     

Clinical features of Henoch-Sch?nlein purpura. Italian Group of Renal Immunopathology.

Henoch-Sch?nlein purpura (HSP) is a vasculitis that often involves the kidneys with a IgA nephropathy indistinguishable from Berger's disease. It can affect patients of any age, even though an uneven distribution of prevalence and severity of renal disease is commonly observed as the renal involvement with transient hematuria without renal functional impairment is much more frequent in children than in adults. The Italian Group of Renal Immunopathology analyzed a cohort of HSP patients with the homogeneous criterium of a renal disease severe enough to indicate renal biopsy both in adults and in children. We report the clinical features of 219 HSP patients (136 adults and 83 children) enrolled from 43 Italian Centers of Nephrology. The diagnosis was based on the detection of IgA nephropathy in a clinical setting of palpable purpura and/or bowel angina. The peak age was in second decade (28% of the patients). In 37% of the cases a potential eliciting factor was identified, mostly infectious (41% in children vs 23% in adults, p < 0.05). A seasoned incidence was found in June (20% of the cases). Most patients had palpable purpura (96.3%), while bowel angina was reported in 54.1% and arthritis in 50.2% at onset. The full extrarenal syndrome was more frequent in children (59%) than in adults (47%, p < 0.05). The extrarenal signs were often not coincident with urinary manifestations. The clinical onset with nephrotic syndrome was more common in children than in adults (35% vs 24.3% respectively, p < 0.02) who more often presented with minimal or moderate proteinuria. The frequency of impaired renal function at onset was similar in both adults and children (24.2% and 31.2% respectively).     

Henoch-Sch?nlein Purpura in Northern Spain: Clinical Spectrum of the Disease in 417 Patients From a Single Center

The severity of clinical features and the outcomes in previous series of patients reported with Henoch-Schönlein purpura (HSP) vary greatly, probably due to selection bias. To establish the actual clinical spectrum of HSP in all age groups using an unselected and wide series of patients diagnosed at a single center, we performed a retrospective review of 417 patients classified as having HSP according to the criteria proposed by Michel et al. Of 417 patients, 240 were male and 177 female, with a median age at the time of disease diagnosis of 7.5 years (interquartile range [IQR], 5.3–20.1 yr). Three-quarters of the patients were children or young people aged 20 years or younger (n = 315), and one-quarter were adults (n = 102). The most frequent precipitating events were a previous infection (38%), usually an upper respiratory tract infection, and/or drug intake (18.5%) shortly before the onset of the vasculitis. At disease onset the most common manifestations were skin lesions (55.9%), nephropathy (24%), gastrointestinal involvement (13.7%), joint symptoms (9.1%), and fever (6.2%). Cutaneous involvement occurring in all patients, mainly purpuric skin lesion, was the most common manifestation when the vasculitis was fully established, followed by gastrointestinal (64.5%), joint (63.1%), and renal involvement (41.2%). The main laboratory findings were leukocytosis (36.7%), anemia (8.9%), and increased serum IgA levels (31.7%). The most frequent therapies used were corticosteroids (35%), nonsteroidal antiinflammatory drugs (14%), and cytotoxic agents (5%). After a median follow-up of 12 months (IQR, 2–38 mo), complete recovery was observed in most cases (n = 346; 83.2%), while persistent, usually mild, nephropathy was observed in only 32 (7.7%) cases. Relapses were observed in almost a third of patients (n = 133; 31.9%).In conclusion, although HSP is a typical vasculitis affecting children and young people, it is not uncommon in adults. The prognosis is favorable in most cases, depending largely on renal involvement.Abbreviations: ACR = American College of Rheumatology, ANA = antinuclear antibodies, ANCAs = antineutrophil cytoplasmic antibodies, ESR = erythrocyte sedimentation rate, HIV = human immunodeficiency virus, HSP = Henoch-Schönlein purpura, IQR = interquartile range, RF = rheumatoid factor, SD = standard deviation, URTI = upper respiratory tract infection     

HLA-DRB1*01 association with Henoch-Sch?nlein purpura in patients from northwest Spain.

OBJECTIVE: To examine the HLA-DRB1 phenotypes of patients with Henoch-Sch?nlein purpura (HSP) and determine if associations exist with disease susceptibility, clinical heterogeneity, or severe systemic complications. METHODS: A retrospective study was performed on an unselected population of patients from Northwest Spain with HSP classified according to proposed criteria. Patients were included in this study if they had at least one year of followup. Fifty Caucasian patients (25 women), 11 of them older than 20 years, were studied. Patients and ethnically matched controls were HLA-DRB1 genotyped from DNA using molecular based methods. RESULTS: During the course of the disease, renal manifestations, especially hematuria, and severe gastrointestinal (GI) manifestations (bowel angina or GI bleeding) were observed in more than 60% of the patients. Twenty percent of patients had persistent renal involvement (renal sequelae). Patients with HSP had a significantly higher frequency of the HLA-DRB1*01 phenotype compared to matched controls. The HLA-DRB1*07 phenotype was also significantly reduced compared with controls. Patients with severe GI manifestations or with persistent renal involvement did not exhibit any specific HLA-DRB1 association other than the underlying association with HLA-DRB1*01. CONCLUSION: HSP in a population from Northwest Spain is significantly associated with HLA-DRB1*01.     

Incidence of vasculitis in children in the Czech Republic: 2-year prospective epidemiology survey

OBJECTIVE: To determine the incidence and presenting features of primary and secondary vasculitides in children across the Czech Republic. METHODS: The population of 2.02 million children under 17 years of age was surveyed over 2 years. Cases were identified through monthly questionnaires posted to consultant pediatricians in all hospital pediatric departments in the country. Patients were included in the analysis if they met established inclusion criteria for each diagnosis and had disease onset between 1997 and 1999. Incidence rates were calculated from population rates derived from the 1991 Census. RESULTS: We identified 452 new cases of vasculitis and connective tissue disease. The estimated annual incidence of Henoch-Schonlein purpura (HSP) was 10.2/100,000 children, with a mean age at onset of 7 years. At disease onset palpable purpura was present in all cases; arthritis/arthralgia in 52%; abdominal pain and/or gastrointestinal bleeding in 40%; hematuria/proteinuria in 15%; and genital involvement in 2.8%. Forty-nine percent of all patients with HSP received short term corticosteroids. The estimated annual incidence of Kawasaki disease (KD) was 1.6/100,000 children under 5 years. Thirteen percent of patients with KD had transient dilatation of coronary arteries; 75% received high dose intravenous immunoglobulin. Other primary systemic vasculitides were extremely rare in this population. Secondary vasculitides of connective tissue diseases had an estimated annual incidence of 0.22/100,000 for systemic lupus erythematosus and 0.19/100,000 for dermatomyositis. CONCLUSION: We determined the incidence of different childhood vasculitides within a hospital based population throughout the Czech Republic. HSP was the most common, with a relatively high proportion of the patients treated with a short course of corticosteroids. A lower incidence than expected of KD raised the suspicion that some cases were not identified. Other childhood vasculitides were rare.