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.     

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.     

复方丹参注射液联合孟鲁司特钠治疗儿童过敏性紫癜疗效观察

目的：观察复方丹参注射液联合孟鲁司特钠治疗儿童过敏性紫癜的疗效。方法：选取2010年1月至2013年1月我院收治的过敏性紫癜患儿82例,随机分为对照组40例和观察组42例。两组均给予卧床休息、控制饮食及马来酸氯苯那敏、维生素c、10％葡萄糖酸钙注射液、山莨菪碱、西咪替丁、抗生素抗感染等常规治疗,对明显腹痛、关节痛者予以激素治疗;观察组在常规治疗基础上加用复方丹参注射液0．5mL／（kg·d）（最大剂量20mL／d）加入5％GS静脉滴注,同时给予孟鲁司特钠咀嚼片（顺尔宁）每天1次睡前口服（2～6岁4mg／d,〉6岁～14岁5mg／d）,两组均治疗1周,观察两组患儿皮疹、腹痛、消化道症状及关节症状消失时间,并比较疗效。结果：观察组皮疹、腹痛、消化道症状及关节症状消失时间均比对照组短,观察组和对照组总有效率分别为95．2％和47．5％,两组比较差异均有统计学意义（P〈0．05）。结论：在常规治疗基础上加用复方丹参注射液联合孟鲁司特钠治疗儿童过敏性紫癜,可提高疗效,值得临床推广应用。     

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。     

Unusual manifestations of henoch-schonlein purpura

Henoch schonlein syndrome (HSP) represents a diagnostic challenge when gastrointestinal symptoms or other atypical symptoms precede the cutaneous manifestations. We describe two patients whose abdominal symptoms antedated cutaneous manifestations. Six-year-old, black male patient, presented with 1 wk history of vomiting (without diarrhea or urinary symptoms), hypertension (140/90 mmHg), diffuse abdominal pain and hypoactive bowel sounds:. WBC’s 40,000/Cu mm³ with 80 percent hypoproteinemia, (albumin 1.6 G%), high plasma renin and stools positive for occult blood. Renal function and urinalysis was normal. Two weeks later patient developed urticarial purpuric rash over the ankles, gross hematuria and gross edema. Biopsy of the skin lesion was consistent with HSP vasculitis. Second patient 4 yr Brazilian male patient presented with abdominalpain, vomiting, blood in the stools, and pain in the right testis. Three days later, developed purpuric rash over his limbs and trunk. He also had hypoproteinemia, hypoalbuminemia, leukocytosis and normal urine analysis. Abdominal pain, bloody stools, painful testicular swelling and hypoproteinemia abated with steroid therapy. In HSP patients, atypical presentation of abdominal pain, hypertension, hypoproteinemia without proteinuria testicular swelling and leucocytosis in the absence of cutaneous and joint manifestations poses as diagnostic problem. Protein-losing enteropathy should be considered in HSP patients with hypoproteinemia.     

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。     

儿童过敏性紫癜临床特征分析(附184例报道)

目的　了解过敏性紫癜儿童的临床特征。方法　回顾性分析过敏性紫癜患儿 184例 ,对其临床表现及特征进行分析。结果　①发病年龄均 >2岁 ,平均发病年龄 (7.8± 2 .6 )岁 ;6 7.4 %患儿于秋冬季节发病 ;6 2 .5 %患儿有明确诱因 ,以上呼吸道感染为主。②所有病人均有典型皮肤紫癜 ,分布于下肢 (10 0 % )、臀部(47.3% )、上肢 (17.4 % )等。 89.1%患儿以皮肤紫癜首发 ,少数以胃肠道及关节症状为首发症状后 2周内出疹 ,先出现肾脏损害而后出疹者罕见。胃肠道及关节症状发生率分别为 6 0 .9%和 4 4 .6 %。紫癜性肾炎发生率为 5 2 .2 % ,临床上主要表现为血尿和 (或 )蛋白尿 (6 8.8% ) ,病理改变主要为Ⅰ～Ⅲ级 (85 .2 % )。③ 4 4 .0 %病人血沉增快 ,2 4 .1%ASO增高 ;6 3.7%病人IgA增高 ,IgG ,IgM及补体绝大多数正常。 结论　过敏性紫癜为儿童常见病 ,且有其本身临床特征及发病规律