159例儿童过敏性紫癜性肾炎的临床病理及预后

过敏性紫癜性肾炎（HSPN）是儿童时期最常见的继发性肾小球肾炎之一。我们对159例儿童HSPN的资料进行分析，研究其临床、病理、预后特点及相互关系。     

儿童紫癜性肾炎的肾脏病理分型及个人建议

紫癜性肾炎(henoch schonlein purpura nephritis, HSPN)是儿童临床肾脏专业最常见的继发性肾小球疾病之一。儿童HSPN肾脏病理分型标准较多,其中国际儿童肾脏疾病研究组分型最为经典和常用,儿童IgA肾病的牛津分型次之,但更为全面、可能更有应用前景。本文综述了儿童HSPN的不同病理分型,并提出笔者个人观点。     

儿童IgA肾病伴过敏性紫癜性肾炎二例

儿童原发性IgA肾病与过敏性紫癜性肾炎临床表现不同，但肾脏病理改变十分相似，本院收治了2例患儿，兼有2种疾病的表现，报告如下。     

紫癜性肾炎患儿肾脏病理程度评分与临床关系的探讨

紫癜性肾炎（ａｎａｐｈｙｌａｃｔｏｉｄ ｐｕｒｐｕｒａｎｅｐｈｒｉｔｉｓ, ＡＰＮ）是儿童继发性肾脏疾病中最常见疾病之一,以全身坏死性小血管炎为主要病变,决定 ＡＰＮ 临床发生发展重要指标为肾脏病理改变程度及病理进展情况。以往研究均为 ＡＰＮ肾脏病理定性分析,缺乏定量标准,我们制定肾脏病理改变评分标准,对３０例 ＡＰＮ肾小球、肾小管病理改变进行定量分析,探讨其与临床表现预后之间的关系,为ＡＰＮ治疗提供依据。 一、对象与方法 １．病例选择与诊断标准：本组３０例,均为我科 １９９３年 ５月～ １９９９年 ２月住…     

34例儿童肾病综合征型紫癜性肾炎肾脏病理与临床分析

目的：探讨儿童肾病综合征型紫癜性肾炎肾脏病理及临床之间的关系。方法：对1997年～2010年我科34例临床以肾病综合征表现及肾活检病理诊断为紫癜性肾炎的患儿进行回顾性分析,肾小球病理分级根据ISKDC分类标准,肾小管间质病理分级参照Bohle方法。结果：（1）肾脏病理分级如下：Ⅰ级2例（5.88%）,Ⅱ级8例（23.53%）,Ⅲ级17例（50.00%）,Ⅳ级6例（17.65%）,Ⅴ级1例（2.94%）,其中Ⅲ级最常见;肉眼血尿组Ⅳ级和Ⅴ级比例较镜下血尿组高,差异有统计学意义（P〈0.05）。（2）肾小管间质病理分级：（-）级12例（35.30%）,（＋）级15例（44.12%）,（＋＋）级5例（14.71%）,（＋＋＋）级0例,（＋＋＋＋）级2例（5.88%）。（3）肾小管间质病变程度与病程呈正相关（r=0.643,P〈0.01）,与血肌酐呈正相关（r=0.577,P〈0.01）。结论：儿童肾病综合征型紫癜性肾炎肾小球病理损害较重,但肾小管间质损害轻,有肉眼血尿表现的临床及病理均较镜下血尿的重,需结合临床表现及肾脏病理进行综合评估,拟定治疗方案。     

儿童毛细血管内皮细胞增生性紫癜性肾炎的临床病理及预后分析

目的 探讨以弥漫性毛细血管内皮细胞增生为主要病理表现的紫癜性肾炎（DEP-HSPN）的临床、病理及预后。 方法 回顾性分析本院近10年来经肾活检确诊的8例DEP-HSPN患儿临床、病理和预后资料,并分别与同病理级别或具有同等蛋白尿水平（肾病水平蛋白尿）的非DEP-HSPN患儿进行比较。 结果 （1）DEP-HSPN起病急,临床表现重,8例患儿中,4例临床表现为肾炎性肾病,3例表现为肾病水平蛋白尿伴血尿,1例呈急性肾炎综合征,4例患儿合并有肉眼血尿。病理分级均为Ⅲ-b级,光镜主要表现为弥漫性毛细血管内皮细胞和系膜细胞增生,常合并毛细血管袢坏死及肾小球内炎性细胞浸润,4例患儿合并细胞性新月体。（2）与病理为Ⅲ-b级的非DEP-HSPN患儿比较,DEP-HSPN患儿病程较短,临床多见肉眼血尿,24 h尿蛋白量高,更多呈肾炎性肾病表现。病理上,DEP-HSPN肾小球毛细血管袢坏死更常见。与具有肾病水平蛋白尿的非DEP-HSPN患儿相比,DEP-HSPN合并新月体的比例较低。（3）8例患儿均采用口服泼尼松联合静脉滴注环磷酰胺（CTX）冲击,病程早期给予2个疗程甲泼尼龙冲击治疗方案。平均随防（7.00±2.20）月,1例临床痊愈,5例持续镜下血尿,2例微量蛋白尿及持续镜下血尿。两组患儿预后差异无统计学意义。 结论 DEP-HSPN起病较急,临床以大量蛋白尿或肾炎性肾病为主要表现,并且常合并肉眼血尿。病程早期给予积极的免疫抑制剂治疗常能取得较满意的近期疗效。     

63例大量蛋白尿表现的儿童紫癜性肾炎肾脏病理与临床分析

目的：探讨临床以大量蛋白尿表现的儿童紫癜性肾炎肾脏病理及临床之间的关系。方法：回顾性分析1997年～2009年我科临床以大量蛋白尿表现及肾活检病理诊断为紫癜性肾炎的63例患儿,根据ISKDC分类标准进行肾小球病理分级,并对肾小管间质改变进行计量评分。结果：（1）肾脏病理分级：Ⅲ级最常见37例（58.73%）,其次为Ⅱ级13例（20.63%）,肾病综合征组Ⅳ级和Ⅴ级比例高于肾病水平蛋白尿组（χ2=4.103,P〈0.05）;（2）肾小管间质病理评分：0级最常见49例（77.78%）,其次1级12例（19.05%）,3级仅2例（3.17%）,肾小管间质评分与肾小球病理分级存在正相关（r=0.507,P〈0.01）,但并不完全平行。（3）肾小管间质病变程度与病程呈正相关（r=0.8,P〈0.05）。结论：大量蛋白尿表现的紫癜性肾炎肾小球病理损害较重,肾小管间质损害轻,肾小管间质病变程度与病程呈正相关,临床需结合病理（包括肾小球及肾小管）全面评估肾组织损害程度,拟定治疗方案。     

成人与儿童紫癜性肾炎的临床病理特点及预后分析

目的：探讨成人与儿童紫癜性肾炎的临床病理特点及疾病转归的差异。方法：收集温州医学院附属第一医院肾内科住院确诊为紫癜性肾炎（HSPN）的成人患者（≥18岁）36例,38例紫癜性肾炎患儿为温州医学院附属儿童医院住院病人,分析比较二者的临床病埋改变及疾病的转归。结果：（1）所有患者均有血尿。成人紫癜性肾炎临床分型为单纯性血尿2例．血尿＋蛋白尿23例,肾病综合征11例;儿童患者单纯性血尿6例,血尿＋蛋白尿22例,肾病综合征10例;两组比较无统计学差异（P〉0．05）。成人紫癜性肾炎合并高血压14例,合并肾功能损害9例;而所有儿童患者均无高血压或肾功能损害。（2）成人紫癜性肾炎病理分级：Ⅰ级3例、Ⅱ级2例、Ⅲ级28例、Ⅴ级2例、Ⅵ级1例,而儿童患者Ⅱ级31例、Ⅲ级7例,两组比较具有统计学差异（P〈0．001）。（3）27例成人紫癜性肾炎患者随访结果A组10例、B组14例、C组3例,30例儿童紫癜性肾炎的随访结果A组15例、B组13例、C组2例,两组预后无统计学差异（P〉0．05）。结论：成人与儿童紫癜性肾炎临床分型以血尿＋蛋白尿最多见,与儿童患者相比,成人临床表现偏重,合并高血压及肾功能损害较多见。成人紫癜性肾炎病理分级以Ⅲ级多见,而儿童病理分级以Ⅱ级多见,提示成人紫癜性肾炎病理较儿童严重。在本研究观察期内,我们发现成人与儿童紫癜性肾炎的预后均较好,二者预后无统计学差异。     

儿童紫癜性肾炎的临床和病理研究

目的：探讨儿童紫癜性肾炎（HSPN）的临床和肾组织病理改变特点及其关系。方法：回顾性分析1995年12月～2007年12月进行过肾活检的105例HSPN患儿的临床和肾组织病理改变情况。结果：105例HSPN患儿中,单纯血尿组18例（17.14%）,血尿及蛋白尿组61例（58.10%）,肾病综合征组26例（24.76%）;三组间的Scr、BUN、IgA、IgE、CRP和C3水平均无统计学差异（P均〉0.05）,血浆白蛋白在各组间存在统计学差异（P均〈0.01）。病理上ISKDC分级结果为：Ⅱa级8例（7.62%）,Ⅱb级21例（20.00%）,Ⅲa级33例（31.43%）,Ⅲb级38例（36.19%）,Ⅳ级5例（4.76%）;三组间比较有统计学差异（χ^2=44.51,P〈0.01）。肾小管间质病变的组织学分级结果为：（-）级34例（32.38%）,（＋）级64例（60.95%）,（＋＋）级6例（5.71%）,（＋＋＋）级1例（0.95%）,（＋＋＋＋）级0例（0%）;三组间比较有统计学差异（χ^2=40.59,P〈0.01）。结论：儿童HSPN临床表现多样,肾病理改变相对较轻,且两者之间存在密切关系。     

Severe hypertension without urinary abnormalities in a patient with Henoch-Schönlein purpura

Hypertension as a complication of Henoch-Sch?nlein purpura (HSP) is almost uniformly accompanied by evidence of renal involvement, either decreased renal function or urinary abnormalities. We report a 4.5-year-old male with HSP who developed severe hypertension without other manifestations of glomerulonephritis, including no decline in renal function and no development of urinary abnormalities. Extensive evaluation for other identifiable causes for his hypertension was not productive. His hypertension resolved with the resolution of his HSP. This case demonstrates that patients with HSP may on occasion develop severe hypertension without other evidence of nephritis. An extensive evaluation for other causes of severe hypertension may be deferred in this setting until well after all other manifestations of HSP have resolved. Received February 6, 1997; received in revised form and accepted May 30, 1997     

Complement activation in Henoch-Schönlein purpura

The pathogenetic mechanism underlying Henoch-Sch?nlein purpura (HSP) is poorly understood. Complement activation has been thought to have a role, but despite the demonstration of complement components in skin and renal biopsy material, serological evidence of complement activation is not convincing. We have assessed complement activation in 64 children with acute HSP. We used an enzyme-linked immunosorbent assay to measure plasma levels of three multimolecular complement activation protein (CAP) complexes: C1r: C1s: C1-inhibitor, C3bP and C5b-9. We found no significant difference between the levels of CAPs in children with acute HSP and a control group of children. This study does not support a role for complement activation in the pathogenesis of HSP. Received February 12, 1996; received in revised form August 27, 1996; accepted October 29, 1996     

孟鲁司特钠联合双嘧达莫对儿童过敏性紫癜肾损害的保护作用

目的探讨孟鲁司特钠联合双嘧达莫对过敏性紫癜（henoch-schonlein purpura,HSP）患儿肾损害的。肾保护作用。方法将146例尿常规检查正常的HSP患儿分为3组,对照组（A组）46例给予维生素C、芦丁等常规治疗;B组57例在A组常规治疗基础上加用孟鲁司特钠治疗;C组43例在常规治疗的基础上加用孟鲁司特钠和双嘧达莫治疗。3组分别于入院时,治疗后第1个月检测尿免疫球蛋白G（immunoglobulin,IgG）、微量白蛋白（micro-albumin,mAlb）、转铁蛋白（transferrin,TRF）、Ct1微球蛋白（a1-microglobulin,a1-MG）、p2微球蛋白（p2-microglobulin,β2-MG）和N-乙酰-β-氨基葡萄糖苷酶（N-acetyl-β-D-glucosaminidase,NAG）的含量。随访6个月;比较3组血尿和（或）蛋白尿的发生率、发病时间及临床分型。结果A组治疗前后TRF、mAlb、82-MG、NAG含量比较,差异有统计学意义（P〈0．01,P〈0．05）;B、C组治疗后第1个月IgG、mAlb、TRF、α1-MG、B2-MG和NAG均较治疗前降低,除α1-MG外,差异有统计学意义（P％0．01）;B、C组治疗后第1个月IgG、mAlb、TRF、α1-MG、B2-MG和NAG均较A组降低,除Ⅸ1-MG外,差异有统计学意义（P〈0．01）;治疗后第1个月各组间IgG、mAb、TRF、α1-MG、82-MG和NAG比较,除a1-MG外,差异有统计学意义（P〈0．01）;B、C组血尿和（或）蛋白尿的发病率、发病时间及临床分型与A组比较,差异有统计学意义（P〈0．05,P〈0．01）。结论应用孟鲁司特钠或孟鲁司特钠联合双嘧达莫治疗HSP,对HSP早期肾损害均有保护作用,可能对HSP肾损害有预防作用。孟鲁司特钠联合双嘧达莫对过敏性紫癜肾损害进行早期干预疗效优于单用孟鲁司特钠。     

Multiple combined therapy for severe Henoch-Schönlein nephritis in children

From 1980 through 1992, 14 children with Henoch-Sch?nlein nephritis (HSN) showing severe glomerular changes (grade IV or V) were given a multiple combined therapy with prednisolone, cyclophosphamide, heparin/warfarin, and dipyridamole, and were followed for 7.5±0.9 years. The period between the onset of nephritis and the start of therapy was 0.8±0.4 years. Ten patients underwent follow-up biopsy after therapy. The percentage of glomeruli having crescents/segmental lesions was significantly reduced after therapy (70%±5% vs. 42%±7%, P <0.01), due mainly to the resolution of crescents (51%±8% vs. 13%±5%, P <0.01). Thus, histological grade was significantly improved (5 grade IV and 5 grade V vs. 7 grade III and 3 grade IV, P <0.01). After an average follow-up period of 7.5 years, 9 patients showed normal urine and renal function, 4 showed minor urinary abnormalities, and 1 heavy proteinuria. No patient developed chronic renal insufficiency. These findings suggest that the multiple combined therapy could be effective for histologically severe HSN, although a prospective controlled study should be performed. Received July 2, 1997; received in revised form September 26, 1997; accepted October 2, 1997     

An adult patient with Kabuki syndrome presenting with Henoch-Schönlein purpura complicated with pulmonary hemorrhage

We present a case of a 33-year-old woman with Kabuki syndrome (KS) presenting with Henoch-Schönlein purpura (HSP). She was admitted to our hospital with a brain abscess in the lateral ventricle and meningitis. She had been diagnosed with KS. Skin eruptions had appeared on her lower extremities, with arthralgia, cough, and hemoptysis. She suddenly developed pulmonary hemorrhage and respiratory failure. We intubated her trachea and started mechanical ventilation in the intensive care unit (ICU). Skin biopsy revealed leukocytoclastic vasculitis with granular depositions of immunoglobulin A (IgA) in dermal vessel walls, and she was diagnosed as having HSP. Supportive management and prednisolone at 20 mg·day^?1 cured the pulmonary hemorrhage and respiratory failure. On ICU day 27, she was weaned from mechanical ventilation. Pulmonary hemorrhage as a complication of HSP is rare and sometimes fatal. KS is often associated with an increased incidence of infection and congenital heart disease. Susceptibility to infection and pulmonary hypertension due to congenital heart disease in this patient may have led to the development of the pulmonary hemorrhage. Supportive care and steroid therapy appeared to be beneficial in the treatment of this patient with HSP with pulmonary hemorrhage.     

Henoch-Schönlein purpura in Wiskott-Aldrich syndrome

Wiskott-Aldrich syndrome (WAS) is a rare immune deficiency disease. Sialophorin glycosylation is defective in WAS. Although it is not very common, renal involvement including IgA nephropathy (IgAN) was reported. Abnormal glycosylation plays a key role in the pathogenesis of IgAN. We present an 8-year-old boy with WAS who had recurrent episodes of Henoch-Schönlein purpura with renal involvement following upper respiratory tract infections. His renal function did not deteriorate. Both IgAN and WAS have glycosylation defects, but there must be some other factors (genetic and environmental) to explain their rare association.     

Cerebral vasculitis in Henoch-Schönlein purpura: a case report with sequential magnetic resonance imaging

Neurological complications are rare during the course of Henoch-Schönlein purpura (HSP). We report a 5-year-old girl with HSP who presented with seizures. Sequential magnetic resonance imaging and electroencephalography showed bilateral multifocal cerebral lesions initially, which gradually and completely resolved with clinical improvement. These lesions were compatible with the radiological pattern of the non-hemorrhagic vasculitic involvement of cerebral parenchyma.