儿童过敏性紫癜575例分析

目的探讨儿童过敏性紫癜(HSP)的临床特点.方法对1996年1月1日～2000年12月31日,在本院住院的575例过敏性紫癜患儿的发病特点、临床表现、肾损害相关因素及预后等方面进行回顾性分析.结果 (1)1996～2000年,每年的患病率依次为0.43%、0.60%、0.75%、0.78% 及1.21%.(2)农村患儿386例,占67.13%.(3)诱因感染337例,占58.61%,进食特殊食物128例,占22.26%.(4) 出现肾脏症状272例,发生率为47.30%.118例皮肤紫癜者25例出现肾脏损害,占21.19%;114例皮肤紫癜加关节症状者,34例出现肾脏损害者,占29.82%;192例皮肤紫癜加消化道症状者中119例发生肾损害,占61.98%;151例皮肤紫癜加消化道症状加关节症状者中94例发生肾损害,占62.25% (P＜0.005).(5)预后543例治愈好转,占94.48%.9例血C3减低者中3例分别在1～2年后出现典型的SLE表现.结论 (1)HSP患病率有逐年升高趋势.农村患儿发病较多.发病诱因仍以感染为第一位.(2)HSP早期出现较多肾外症状,特别是消化道症状明显者,易发生肾脏损害.(3)HSP中出现血C3减低者,其早期"紫癜样皮疹"有可能为SLE的皮肤损害之一;紫癜肾伴血C3减低者,应及早做肾活检,以与早期狼疮肾鉴别.     

儿童过敏性紫癜325例回顾性分析

目的 了解儿童过敏性紫癜(HSP)的临床特点,以提高对本病的认识,并指导临床诊治。方法 收集2012 年6 月至2014 年6 月诊断为HSP 的325 例住院患儿的临床资料进行回顾性分析。结果 325 例患儿中,春季和冬季发病人数较多,分别占33.8% 和27.4%;感染为诱发HSP 的主要因素(57.2%);紫癜伴发腹部症状和紫癜伴发关节及腹部症状的患儿肾脏损害发生率分别为60.3% 和48.9%,与单纯紫癜患儿相比差异有统计学意义(P+ 降低。结论 HSP 发病以冬春季节好发,感染为主要诱因,合并消化道症状者更易发生肾脏损害,肾脏损害的病理分级以Ⅲ a 和Ⅲ b 居多。     

儿童过敏性紫癜236例临床分析

目的了解儿童过敏性紫癜(HSP)的肾脏受累因素。方法总结2000年1月～2004年3月236例HSP患儿的临床资料,对HSP发生的流行病学、临床特征及肾脏受累的危险因素进行分析。结果①发病年龄在7～13岁者占78.40%,平均年龄(9.0±2.8)岁,最小年龄1岁8个月;9月～次年3月发病率占全年发病的84.32%;感染仍是主要诱因(35.59%);胃肠症状首发时36.54%发生误诊。②紫癜遍及全身,以双下肢最多见(99.15%),58.47%反复出现,72.03%紫癜于1个月内消退;胃肠道及关节症状发生率分别是60.59%和47.88%。③55.51%出现肾脏损害,95.42%发生在起病3个月以内,临床上以肾病综合征最常见(41.22%),病理改变Ⅲ级以上者占70.26%,4.05%表现为毛细血管内增生型,急性期无肾功能不全。④发病年龄≥10岁,反复性紫癜超过1个月在肾脏易感因素Logistic回归分析中有显著性意义(P<0.05)。结论儿童HSPN急性期肾衰竭少见,但肾脏病理改变相对严重;起病年龄>10岁、紫癜反复超过1个月是HSP易发生肾脏损害的危险因素,肾外症状的严重性并非危险因素。     

过敏性紫癜患儿血清高迁移率族蛋白B1表达及意义

目的 测定过敏性紫癜(HSP)患儿血清高迁移率族蛋白B1(HMGB1)水平,分析血清HMGB1与实验室指标的相关性,初步探讨HMGB1与HSP发病及肾损害的关系.方法 纳入2017年12月至2019年12月在重庆医科大学附属儿童医院初诊为HSP患儿83例,其中45例无肾脏受累(A组)、38例合并肾脏损害(B组),恢复期...     

小儿过敏性紫癜56例临床研究

目的探讨小儿过敏性紫癜的临床特点。方法对在我院住院的56例过敏性紫癜患儿发病特点、临床表现、肾损害等进行分析。结果农村、牧区患儿38例，占67．86％；感染39例，占69．64％；进食特殊食物28例，占50．00％；出现肾脏症状22例，占39．29％；皮肤紫癜加关节症状者16例，占28．57％；皮肤紫癜加消化道症状者36例，占64．29％。53例治愈好转，占94．64％。结论患病率有逐年升高趋势。农村、牧区患儿发病以往增多。发病诱因仍以感染为第一位。消化道症状明显者，易发生肾脏损害。     

儿童再发过敏性紫癜39例临床分析

目的 探讨儿童再发过敏性紫癜(Henoch-Schonlein purpura,HSP)的临床特点.方法 总结广东省中山市博爱医院儿科2007年12月至2011年12月39例再发HSP患儿的临床及随访资料,对再发HSP发病特点、临床表现、复发危险因素、治疗及预后等方面进行回顾性分析.结果 再发情况:研究期间住院的HSP患儿共有275例,其中再发HSP 39例,再发率为14.18％(39/275),再发者平均年龄(7.10±2.48)岁;非再发者236例,平均年龄(6.05±2.63)岁,两者比较差异有统计学意义(P =0.0274).再发发生时间:最短1个月,最长1年.再发的临床表现:皮疹首发多见,其次为腹痛,较少出现关节症状.再发诱因:感染是HSP再发的主要诱因(41.02％),多见于幽门螺旋杆菌及链球菌感染;食物过敏也是HSP再发因素之一.再发者合并肾损害情况:再发HSP的患儿中,51.28％(20/39)合并肾脏损害,非再发HSP合并肾脏损害发生率16.1％ (38/236),两者比较差异有统计学意义(P=0.0002).20例再发HSP肾损害者中,孤立性血尿8例,血尿及蛋白尿12例.12例进行了肾活检,病理分级:Ⅱa级7例(58.33％),Ⅲa级5例(41.67％).非再发HSP合并肾脏损害16例进行了肾活检,病理分级:Ⅰ级2例(12.5％),Ⅱa级8例(50.0％),Ⅲa级6例(37.5％).治疗及预后:19例未合并肾损害者经过治疗均治愈,包括3例血液灌流者均无肾损害发生;20例肾损害者16例治愈,4例在随访中,无终末期肾功能衰竭发生.结论 再发HSP临床表现以皮疹多见,但肾损害发生率高于非再发者.感染是再发HSP的第1位诱因.严重HSP患者血液灌流治疗可能预防肾损害发生.     

男童过敏性紫癜累及生殖器24例报告

目的探讨生殖器受累的过敏性紫癜(HSP)临床特点及诊治方法。方法对2003年3月—2009年4月收治的24例生殖器受累的HSP患儿的临床资料进行分析。结果 612例男性HSP患儿中生殖器受累者为24例(4%),均为混合型紫癜,与消化道症状并存者最多(21/24例)。生殖器皮肤紫癜11例(46%),生殖器肿痛15例(63%),其中4例有睾丸和/或附睾炎;生殖器受累出现在病程的第2～21天。24例HSP中13例患儿给予甲基泼尼松龙静脉冲击治疗,11例症状较轻给予氢化可的松静滴,生殖器肿痛5 d内缓解,生殖器皮疹7 d左右消退,生殖器受累症状在1～2周内全部消失。1例患儿1个月后紫癜复发时,再次出现右侧睾丸炎,再次治疗后好转。随访6个月～4年,全部患儿症状持续缓解,11例合并肾脏受累,均未进展至肾功能不全。结论 HSP生殖器受累多见于病程急性期,常伴有其他系统损害,肾上腺皮质激素治疗效果明显,预后良好。     

血管紧张素转换酶基因多态性对过敏性紫癜的影响

目的 探讨过敏性紫癜(HSP)与血管紧张素转换酶(ACE)基因多态性间的关系。方法 选择70例HSP患儿,其中无肾脏损害32例,过敏性紫癜性肾炎(HSPN)38例。HSPN中单纯性血尿15例,蛋白尿23例。健康对照儿童100例。采用聚合酶链反应(PCR)检测ACE基因型。结果 1.HSP患儿与健康对照组ACE基因型分布比较无差异(P>0.05);2.非肾脏损害患儿32例与伴单纯性血尿HSPN患儿15例ACE基因型分布比较亦无差异(P>0.05);但与23例伴蛋白尿HSPN患儿ACE基因型分布比较,缺失型(DD),型者发生蛋白尿频率明显升高(P<0.05)。结论 HSPN蛋白尿发生与ACE基因多态性相关,DD型者发生蛋白尿机会明显增多。     

过敏性紫癜肾脏损害的临床因素分析

目的　探讨过敏性紫癜 (HSP)肾脏损害的临床因素。方法　过敏性紫癜初发患儿 10 0例 ,根据尿常规检查 ,分为尿检正常组 (5 1例 )及紫癜肾组 (4 9例 ) ;紫癜肾组分为一过性尿异常组 (2 7例 )及持续性尿异常组(2 2例 )。观测临床指标 ,并行统计学分析。结果　紫癜肾组年龄、皮疹分布范围、腹痛及消化道出血的发生率及严重程度均高于尿检正常组 ,有显著性差异 (P均 <0 .0 5 )。持续性尿异常组皮疹持续时间、血尿并蛋白尿发生率、2 4h尿蛋白定量均高于一过性尿异常组 ,有显著性差异 (P均 <0 .0 5 )。结论　年龄增长、皮疹分布范围广泛、腹痛、消化道出血是HSP肾脏受累的危险因素 ,而皮疹持续、血尿并蛋白尿、尿蛋白大于 1.0 g/d ,与肾脏持续受累有关     

过敏性紫癜患儿肾受累的实验室指标选择与分析

探讨过敏性紫癜(HSP)肾受累情况及相关实验室指标的敏感性。方法检测HSP患儿肾功 能、肾脏B超、尿常规、尿12hAddis计数及尿4项蛋白,结合临床,分析患儿肾脏受累情况及上述指标的临床价 值。结果HSP患儿肾受累占42.44％。肾功能、肾脏B超、尿常规、尿12hAddis计数和尿4项蛋白的敏感性 分别为5.64％、6.89％、25.84％、41.30％和76.19％。结论HSP患儿可有肾脏受累,且随着病程的延长,肾受 累的发生率增加;尿4项蛋白能敏感地反映HSP肾受累情况,可作为HSP早期肾受累的敏感判断指标。     

抗心磷脂抗体与儿童过敏性紫癜关系的探讨

目的探讨抗心磷脂抗体（anticardiolipin,ACA）与儿童过敏性紫癜（Henoch．schoenleinpurpura,HSP）的关系。方法采用酶联免疫吸附试验（ELISA）对91例急性期HSP患儿（HSP组）和30例健康体检儿童（对照组）的血清进行IgG型ACA（IgG—ACA）和IgM型ACA（IgM—ACA）检测,并对两组ACA阳性率进行统计学比较。结果91例HSP患儿中有16例ACA阳性,其中IgM．ACA阳性13例,IgG-ACA阳性3例,总阳性率占17．6％,且均无抗磷脂综合征（APS）表现;而健康体检儿未检出ACA阳性者。经Fisher精确概率检验,差异有显著性（χ2=6．078,P〈0．05）。HSP组中50例伴有腹痛的患儿中其13例ACA阳性,阳性率为26．0％;而41例不伴腹痛的患儿中仅3例ACA阳性,阳性率为7．3％,经χ2检验,差异有显著性（χ2=5．426,P〈0．05）。26例肾脏受损者中ACA阳性4例;65例无肾脏受损者中ACA阳性12例,经∥检验,差异无显著性（χ2=0．002,P〉0．05）。结论ACA可能与儿童HSP发病及腹痛症状有关、与HSP肾损害无关;APS可能与儿童HSP无关。     

Henoch-Schönlein purpura in north-eastern Turkey

AIM: To evaluate the epidemiological and clinical findings in children with Henoch-Sch?nlein purpura (HSP) admitted during a 10-year period, 1995 to 2004, and to compare them with series from other parts of the world. METHODS: The medical records of all children aged 17 years or less admitted with a diagnosis of HSP to the Department of Pediatrics of Karadeniz Technical University were evaluated retrospectively for epidemiological and clinical features. RESULTS: Of 116 children, 73 (63%) were boys. The mean (SD) age at presentation was 8.9 (3.7) years and one-third of them were older than 10 years of age. Over half the cases presented between September and January. All patients had the typical skin rash. Gastro-intestinal manifestations were seen in 64 (55.1%) and joint manifestations, common during the early course of the disease, in 73 (62.9%). Two patients required laparatomy, one for acute abdomen and the other for bowel resection owing to intussusception. Renal manifestations were observed in 36 (31%), all within 3 months of initial symptoms, and one patient (0.8%) with nephritic syndrome progressed to end-stage renal disease. Five patients had hypertension without urinary findings. Symptoms recurred in eight patients (6.9%) over a period ranging from 2 to 5 months after complete resolution of symptoms. There was a history of a preceding upper respiratory tract infection in 16 (13.7%) and a streptococcal infection was confirmed by throat culture in 12 of the 42 (28.5%) children at presentation. CONCLUSION: HSP is generally benign and self-limiting. Hypertension may be seen during the course of the disease without urinary findings. In this area, it seems to affect older children and there is a relatively lower incidence of renal manifestations.     

Steroids for prophylaxis of nephropathy in Schnlein Henoch purpura? Follow-up of 171 patients

The necessity of the controversially discussed general steroid prophylaxis in Henoch-Sch?nlein Purpura (HSP) was analysed based on frequency, risk factors and prognosis of renal involvement. Case histories and follow up of at least 1.5 years were evaluated in all 171 patients (median age 6 years) in our institution suffering from HSP between 1.1.1987 and 30.6.1997. HSP was frequently manifest with an involvement of joints (64%) and gastrointestinal tract (58%). Renal involvement occurred in only 29% of the children. Excepted one girl (age 12 year), all children with renal disease completely recovered. Renal involvement was significantly rarer in young children (16%) and after one week therapy with prednisone (7%). In young children, renal involvement always followed abdominal pain. CONCLUSION: In our collective, a general preventing of renal disease in HSP was unnecessary. Especially in young children, the most common manifestation age, renal involvement rarely occurred and had always a good prognosis.     

初发过敏性紫癜患儿肾脏受累危险因素分析

目的 对初发过敏性紫癜(HSP)患儿进行随访,探讨HSP患儿肾脏受累的危险因素.方法 前瞻性纳入2009年12月至2010年11月在南京医科大学附属南京儿童医院肾脏科住院的初发HSP患儿,随访6个月,根据肾脏受累定义,将HSP患儿分为肾脏受累组和无肾脏受累组.复习文献提取与肾脏受累可能相关的3项人口学特征、8项临床症状...     

Damage index in childhood-onset systemic lupus erythematosus in Egypt

Background

To investigate the prevalence of cumulative organ damage among Egyptian children with juvenile-onset systemic lupus erythematosus (jSLE) and the relationships between the organ damage and the demographic data, clinical variables, and disease activity.

Methods

A total of 148 patients with jSLE have been followed in the pediatric rheumatology clinic and section at Cairo University. These patients were evaluated by retrospective chart review. The organ system damage due to SLE was measured using the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI). Risk factors for damage were also studied including demographic criteria as well as clinical and laboratory manifestations.

Results

Overall, 43.9% of the patients had damage within a mean of 6.57 ± 3.59 years of disease diagnosis. Neuropsychiatric (NPS-21%) and renal (16.9%) system involvement were observed most frequently, followed by cardiovascular (11.5%), skin (9.5%), pulmonary (6.1%), and ocular (4.8%), with a mean SDI score of 0.93 ± 1.37. In our study, the presence of neuropsychiatric manifestations at diagnosis showed the strongest association with the presence of later disease damage.The number of SLE diagnostic criteria at presentation was strongly associated with the total SDI score, and the renal damage was significantly more prevalent in patients with age at disease diagnosis below 10 years of age. A higher mean disease duration was found in patients with musculoskeletal damage.

Conclusion

We found that cumulative organ damage, as measured by the SDI, was present in 43.9% of Egyptian patients with juvenile-onset SLE. The damage was significantly more likely in patients who had more SLE diagnostic criteria at time of disease presentation and NPS manifestations at the time of diagnosis.

     

糖皮质激素对过敏性紫癜患儿肾损害预防作用的Meta分析

目的评价糖皮质激素对过敏性紫癜患儿肾损害的预防作用。方法检索Coehrane图书馆、MEDUNE、EMBASE、CBM等资料库,收集有关糖皮质激素对过敏性紫癜患儿肾损害预防作用的随机对照研究后进行Meta分析。结果3个随机对照研究符合纳入标准。显示治疗组肾损害的发生率为7．0％（11／158）,对照组肾损害的发生率为19．9％（30／151）（RR=0．35,95％CI：0．19～0．65）;治疗组未出现明显的不良反应。结论过敏性紫癜患儿早期接受泼尼松治疗可显著减少肾脏损害的发生,且无明显不良反应。但本项分析的试验数较少,且存在研究结果方向的不一致,故仍需要进行大样本、前瞻性、随机对照研究来进一步明确。目前建议糖皮质激素应有选择地早期应用于存在肾脏受累危险因素的高危患儿,以预防肾脏损害。     

Risk factors of renal involvement and significant proteinuria in Henoch-Schönlein purpura

Risk factors of renal involvement and significant proteinuria in patients with Henoch-Sch?nlein purpura (HSP) were retrospectively evaluated by univariate and multivariate analyses. The analysis was performed in 134 patients with HSP. Renal involvement was found in 65 patients (49%) and 97% of the renal involvement was found within 3 months of disease onset. Moderate or severe proteinuria was recognised in 25 patients. A univariate analysis revealed that an age of more than 4 years at the onset, severe abdominal pain with gastrointestinal bleeding, persistent purpura over a month, coagulation factor XIII activity < 80%, and treatment with factor XIII concentrate were associated with developing renal involvement. A multivariate analysis showed that severe abdominal symptoms, an age of more than 4 years, and persistent purpura increased the risk of renal involvement. Risk factors of moderate or severe proteinuria were also examined. The risk factors in a univariate analysis were severe abdominal symptoms, persistent purpura, decreased factor XIII activity, treatment with steroids, and treatment with factor XIII concentrate. Of those, persistent purpura, treatment with factor XIII concentrate, and factor XIII activity < 80% were associated with significant proteinuria in a multivariate analysis. Among the patients with severe abdominal symptoms, factor XIII activity was significantly decreased in patients with significant proteinuria compared to other patients without significant proteinuria. CONCLUSION: Long-term prognosis of Henoch-Sch?nlein purpura is dependent on the severity of renal involvement. In those patients who have the risk factors of renal involvement, especially significant proteinuria, close attention should be paid to a urinalysis for at least 3 months from the onset of the disease.     

Serum complement components in Henoch-Sch?nlein purpura.

Serum levels of C1q, C4, C3, C5, factor B, and properdin were measured in patients with Henoch-Schönlein purpura (HSP). In the cases of acute HSP, 9 of 23 (39%) had a low CH50, and 5 of 17 (30%) a low properdin; C1q, C4, and C3 levels were not depressed. In 10 cases with chronic nephritis following HSP, complement components were normal except for 2 with reduced C4 and one with low properdin. These findings confirm that complement activation occurs in HSP; the low serum levels of properdin in the acute group indicate that there is activation of the alternative pathway in these patients.     

Clinical profile of acute glomerulonephritis in children

One hundred patients with acute glomerulonephritis (AGN), which comprised 0.5 percent of medical admissions to Institute of Child Health, kabul were reviewed and analysed. Antecedant history of sore throat, 1–3 wk before the onset of disease, was obtained in 57 percent patients. ASLO titre was elevated above 250 Todd units in 52 percent cases. Majority (72%) of patients were between 3–10 yr with only two patients below 3 yr of age. Males were affected twice as frequently as females. More than one sibling in the family was affected by AGN in three families and one patient had an established underlying rheumatic heart disease. Disease was ushered as acute onset of edema (93%), tea-colored urine (56%), hypertension (49%) and fever (41%). Urine abnormalities were detected in 96 percent of patients and azotemia in 85 percent cases. The main complications were congestive heart failure (8%), hypertensive encephalopathy (5%) and acute renal shut down (5%). Out of three deaths in our series, one each was due to pulmonary edema, renal shut down and nosocomial infection.