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130例儿童紫癜性肾炎临床病理5年随访研究
王美秋, 王忍, 何旭, 张沛, 夏正坤, 高春林
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130例儿童紫癜性肾炎临床病理5年随访研究
A five-year follow-up study on the clinicopathology of 130 children with Henoch-Schönlein purpura nephritis
目的 分析儿童紫癜性肾炎（Henoch-Schönlein purpura nephritis，HSPN）的临床病理改变特点及其预后。方法 回顾性分析2001年1月至2015年6月在南京大学医学院附属金陵医院接受过肾活检且随访时间≥5年的HSPN患儿临床病理资料。随访终点事件定义为估算肾小球滤过率（eGFR）<90 ml·min-1·(1.73 m2)-1。按照患儿是否发生终点事件将入选者分为达终点事件组和未达终点事件组。采用Cox比例风险模型法分析HSPN患儿肾脏不良预后的影响因素。用Kaplan-Meier生存曲线法进行生存分析，Log-rank检验比较肾小球节段性硬化（S1）组和无肾小球节段性硬化（S0）组患儿肾脏累积生存率的差异；采用受试者工作特征曲线（ROC）及曲线下面积（AUC）评价诊断价值。结果 共130例HSPN患儿纳入本研究，中位发病年龄为11.7（8.6，13.3）岁，男性71例（54.6%），中位随访时间为100.0（75.8，119.0）个月，有12例（9.23%）患儿发生终点事件。与未达终点事件组（n=118）比较，达终点事件组（n=12）患儿基线高血压比例、24 h尿蛋白量、血总胆固醇、血尿酸、血肌酐水平较高，血白蛋白水平较低（均P<0.05）。两组患儿用药种类的差异无统计学意义（均P>0.05）。在肾组织病理改变方面，与未达终点事件组比较，达终点事件组患儿系膜细胞增生（M1）、S1、肾小管萎缩或间质纤维化（T1/T2）和球囊粘连的比例较高（均P<0.05）。多因素Cox回归分析结果显示，S1是肾脏不良预后的独立危险因素（HR=7.739，95%CI 1.422~42.114，P=0.018）。生存分析结果显示，S1组患儿肾脏累积生存率明显低于S0组（Log-rank χ2=17.069，P<0.001）。ROC曲线评估S1预测肾脏不良预后发生的AUC为0.710（95%CI 0.549~0.872），敏感度为0.667（95%CI 0.349~0.901），特异度为0.754（95%CI 0.666~0.829）。结论 S1是HSPN患儿肾脏不良预后的独立危险因素，具有预测诊断价值。
Objective To analyze the clinicopathologic features and prognosis of children with Henoch-Schönlein purpura nephritis (HSPN). Methods The clinicopathological data of children with HSPN who were followed up for more than 5 years and underwent renal biopsy in Jinling Hospital affiliated to Medical School of Nanjing University from January 2001 to June 2015 were retrospectively analyzed. The follow-up endpoint event was defined as estimated glomerular filtration rate (eGFR)<90 ml·min-1·(1.73 m2)-1. Participants were divided into two groups according to whether the children had reached the primary endpoint event or not. Cox proportional hazards model was used to analyze the influencing factors of renal poor prognosis in children with HSPN. Kaplan-Meier survival curve method was used for survival analysis, and log-rank test was used to compare the difference of renal cumulative survival rate between segmental sclerosis/adhesion (S1) group and non-segmental sclerosis/adhesion (S0) group. Receiver operating characteristic curve (ROC curve) and area under the curve (AUC) were used to evaluate the diagnostic value. Results A total of 130 children with HSPN were enrolled in the study. The median onset age was 11.7(8.6, 13.3) years old, of whom 71 cases were males (54.6%). At a median follow-up time of 100.0(75.8, 119.0) months, 12 cases (9.23%) with HSPN reached the primary endpoint event. Compared with the non-endpoint event group, the endpoint event group had higher proportion of hypertension, higher levels of 24-hour urinary protein, serum cholesterol, serum uric acid, and serum creatinine, and lower levels of serum albumin (all P<0.05). There was no statistical difference in treatment between the two groups (all P>0.05). In terms of pathological features, compared with the non-endpoint event group, the endpoint event group had higher proportion of mesangial hyperplasia (M1), S1, tubular atrophy/interstitial fibrosis (T1/T2) and Glomerulus-Bowman's capsule adhesion (all P<0.05). Multivariate Cox regression model showed that S1 was significantly correlated with renal poor prognosis (HR=7.739, 95%CI 1.422-42.114, P=0.018). As was revealed in a Kaplan-Meier plot, renal cumulative survival rate in the S1 group was significantly lower than that in the S0 group (log-rank χ2=17.069, P<0.001). The ROC curve showed S1 accurately predicted the outcome (AUC=0.710, 95%CI 0.549-0.872) with specificity of 0.667(95%CI 0.349-0.901) and specificity of 0.754(95%CI 0.667-0.829). Conclusions S1 is an independent risk factor affecting renal poor prognosis and has a diagnostic value.
肾小球肾炎 / 儿童 / 病理学 / 紫癜性肾炎 / 牛津分型 / IgA血管炎 {{custom_keyword}} /
Glomerulonephritis / Child / Pathology / Henoch-Schönlein purpura nephritis / Oxford classification / IgA vasculitis {{custom_keyword}} /
孙玉玲 , {{custom_editor}}
表1 两组紫癜性肾炎患儿基线临床资料和实验室检查结果的比较 |
| 项目 | 总体(n=130) | 达终点事件组(n=12) | 未达终点事件组(n=118) | 统计量(Z/χ2) | P值 |
|---|---|---|---|---|---|
| 男性[例(%)] | 71(54.6) | 7(58.3) | 64(54.2) | 0.074 | 0.786 |
| 紫癜出现年龄(岁) | 11.7(8.6，13.3) | 12.0(8.1，14.2) | 11.6(8.6，13.3) | -0.273 | 0.784 |
| 紫癜至肾脏受累时间(d) | 13.0(0，49.8) | 9.0(0，30.8) | 13.5(0，58.0) | -0.168 | 0.866 |
| 肾脏受累至肾活检时间(d) | 57.5(24.0，339.8) | 478.5(35.8，1 360.8) | 54.0(24.0，248.5) | -1.882 | 0.060 |
| 随访时间(月) | 100.0(75.8，119.0) | 111.0(89.9，118.8) | 97.5(74.0，119.0) | -0.990 | 0.322 |
| 肾脏受累表现[例(%)] | |||||
| 血尿a | 112(86.2) | 10(83.3) | 102(86.4) | 0.672 | |
| 肉眼血尿 | 36(27.7) | 3(25.0) | 33(28.0) | 1.000 | |
| 蛋白尿 | 109(83.8) | 11(91.7) | 98(83.1) | 0.689 | |
| 肾脏以外表现[例(%)] | |||||
| 皮肤紫癜 | 130(100.0) | 12(100.0) | 118(100.0) | ||
| 胃肠道反应 | 46(35.4) | 5(41.7) | 41(34.7) | 0.753 | |
| 关节症状 | 48(36.9) | 2(16.7) | 46(39.0) | 0.209 | |
| 高血压 | 4(3.1) | 3(25.0) | 1(0.8) | 0.002 | |
| 尿蛋白量(g/24 h) | 1.2(0.5，2.6) | 3.5(2.8，5.5) | 1.0(0.5，2.0) | -3.531 | <0.001 |
| 尿NAG酶(U/g·cr) | 15.4(10.4，31.9) | 25.4(17.4，52.5) | 14.8(10.3，30.3) | -1.858 | 0.063 |
| 尿RB蛋白(g/L) | 0.2(0.1，0.4) | 0.3(0.1，0.8) | 0.2(0.1，0.4) | -1.008 | 0.239 |
| 血红蛋白(g/L) | 12.80(12.08，13.70) | 12.75(11.15，14.33) | 12.80(12.10，13.70) | -0.121 | 0.904 |
| 血白蛋白(g/L) | 39.8(34.5，43.3) | 34.3(23.2，39.5) | 40.1(35.2，43.4) | 2.369 | 0.018 |
| 血胆固醇(mmol/L) | 5.0(4.2，6.4) | 6.1(5.0，8.0) | 4.9(4.1，6.0) | -2.510 | 0.012 |
| 血尿酸(μmol/L) | 300.0(243.8，365.3) | 373.0(273.0，448.0) | 295.0(238.8，353.3) | -2.538 | 0.011 |
| 血肌酐(μmol/L) | 48.0(38.0，61.0) | 63.5(50.5，79.5) | 48.0(38.0，59.0) | -2.836 | 0.005 |
| eGFR[ml·min-1·(1.73 m2)-1] | 120.9(99.9，140.6) | 107.4(77.0，138.3) | 121.5(100.4，143.6) | -1.504 | 0.133 |
| 注：尿NAG酶：尿N-乙酰-β-D-氨基葡萄糖苷酶；尿RB蛋白：尿视黄醇结合蛋白；eGFR：估算肾小球滤过率；a：血尿包括肉眼血尿和镜下血尿；正态分布的计量资料用 |
表2 达终点事件组和未达终点事件组患儿应用药物种类的比较[例(%)] |
| 药物种类 | 总体(n=130) | 达终点事件组(n=12) | 未达终点事件组(n=118) | P值 |
|---|---|---|---|---|
| RAS阻滞剂 | 41(31.5) | 5(41.7) | 36(30.5) | 0.517 |
| 泼尼松 | 109(83.8) | 12(100.0) | 97(82.2) | 0.213 |
| 免疫抑制剂a | 95(73.1) | 10(83.3) | 85(72.0) | 0.512 |
| 甲泼尼龙 | 45(34.6) | 4(33.3) | 41(34.7) | 1.000 |
| 注：RAS：肾素-血管紧张素系统；a：免疫抑制剂包括霉酚酸酯、环磷酰胺、钙调磷酸酶抑制剂和咪唑立宾 |
表3 两组紫癜性肾炎患儿肾组织病理学改变的比较[例（%）] |
| 病理指标 | 总体(n=130) | 达终点事件组(n=12) | 未达终点事件组(n=118) | χ2值 | P值 |
|---|---|---|---|---|---|
| 肾脏病理学改变 | |||||
| M1 | 62(47.7) | 10(83.3) | 52(44.1) | 0.009 | |
| E1 | 43(33.1) | 6(50.0) | 37(31.4) | 0.210 | |
| S1 | 37(28.5) | 8(66.7) | 29(24.6) | 0.005 | |
| T1/T2 | 3(2.3) | 3(25.0) | 0(0) | 0.001 | |
| C | 3.008 | 0.222 | |||
| C0 | 55(42.3) | 5(41.7) | 50(42.4) | ||
| C1 | 61(46.9) | 4(33.3) | 57(48.3) | ||
| C2 | 14(10.8) | 3(25.0) | 11(9.3) | ||
| 球性硬化 | 4(3.1) | 1(8.3) | 3(2.5) | 0.324 | |
| 球囊粘连 | 44(33.8) | 9(75.0) | 35(29.7) | 0.003 | |
| 免疫荧光 | |||||
| IgG | 52(40.0) | 2(16.7) | 50(42.4) | 0.122 | |
| IgM | 63(48.5) | 3(25.0) | 60(50.8) | 0.088 | |
| C3 | 96(73.8) | 10(83.3) | 86(72.9) | 0.731 | |
| C4 | 6(4.6) | 2(16.7) | 4(3.4) | 0.095 | |
| C1q | 9(6.9) | 1(8.3) | 8(6.8) | 0.594 |
| 注：系膜细胞增殖（M）：PAS染色下，≥50%的肾小球系膜区内可见>3个系膜细胞则为M1，否则为M0；毛细血管内增生（E）：肾小球毛细血管袢内细胞增生导致管腔狭窄，若存在则为E1，否则为E0；肾小球节段性硬化（S）：存在肾小球节段性硬化或粘连则为S1，否则为S0；肾小管萎缩/间质纤维化（T）：皮质区细胞外基质增多导致管腔狭窄，根据受累面积百分比分别为T0（≤25%）、T1（>25%~50%）和T2（>50%）；细胞或混合型新月体（C）：根据占肾小球总数的百分比分别为C0（无）、C1（≤25%）和C2（>25%） |
表4 肾脏不良预后的影响因素分析（Cox回归分析，n=130） |
| 影响因素 | 单因素Cox回归 | 多因素Cox回归 | ||
|---|---|---|---|---|
| HR(95%CI) | P值 | HR(95%CI) | P值 | |
| 性别(男/女) | 0.688(0.218~2.172) | 0.524 | ||
| 关节症状(有/无) | 0.306(0.066~1.408) | 0.128 | ||
| 胃肠道症状(有/无) | 1.015(0.320~3.219) | 0.980 | ||
| 高血压(有/无) | 7.697(2.005~29.554) | 0.003 | 1.737(0.362~8.325) | 0.490 |
| 肉眼血尿(有/无) | 0.506(0.132~1.942) | 0.321 | ||
| eGFR[≥90/<90 ml·min-1·(1.73 m2)-1] | 3.077(0.363~26.081) | 0.303 | ||
| 血红蛋白a(贫血/无贫血) | 1.546(0.460~5.190) | 0.481 | ||
| 血白蛋白(≥25/<25 g/L) | 2.887(0.759~10.980) | 0.120 | ||
| 血胆固醇(≥5.7/<5.7 mmol/L) | 2.005(0.645~6.236) | 0.230 | ||
| 血尿酸b(异常/正常) | 6.536(2.090~20.442) | 0.001 | 2.018(0.511~7.976) | 0.317 |
| 24 h尿蛋白量(1.0~3.5/<1.0 g) | 8.670(0.994~75.625) | 0.051 | ||
| 24 h尿蛋白量(>3.5/<1.0 g) | 17.341(2.082~44.433) | 0.008 | 7.826(0.742~32.517) | 0.087 |
| 尿NAG酶(≥9.7/<9.7 U/g·cr) | 0.786(0.212~2.913) | 0.718 | ||
| 尿RB蛋白(≥0.7 /<0.7 g/L) | 2.624(0.706~9.753) | 0.150 | ||
| M1(M1/M0) | 6.530(1.419~30.055) | 0.016 | 1.141(0.160~8.141) | 0.896 |
| E1(E1/E0) | 2.431(0.780~7.574) | 0.125 | ||
| S1(S1/S0) | 10.193(2.654~39.146) | 0.001 | 7.739(1.422~42.114) | 0.018 |
| T1/T2(T1~T2/T0) | 12.163(3.123~47.378) | <0.001 | 1.359(0.217~8.512) | 0.743 |
| C1(C1/C0) | 0.797(0.213~2.982) | 0.736 | ||
| C2(C2/C0) | 3.025(0.717~12.758) | 0.132 | ||
| 球性硬化(有/无) | 3.157(0.402~24.819) | 0.275 | ||
| 球囊粘连(有/无) | 4.300(1.153~16.036) | 0.030 | 0.809(0.123~4.971) | 0.819 |
| 注：eGFR：估算肾小球滤过率；尿NAG酶：尿N-乙酰-β-D-氨基葡萄糖苷酶；尿RB蛋白：尿视黄醇结合蛋白；系膜细胞增殖（M）：PAS染色下，≥50%的肾小球系膜区内可见>3个系膜细胞则为M1，否则为M0；毛细血管内增生（E）：肾小球毛细血管袢内细胞增生导致管腔狭窄，若存在则为E1，否则为E0；肾小球节段性硬化（S）：存在肾小球节段性硬化或粘连则为S1，否则为S0；肾小管萎缩/间质纤维化（T）：皮质区细胞外基质增多导致管腔狭窄，根据受累面积百分比分别为T0（≤25%）、T1（>25%~50%）和T2（>50%）；细胞或混合型新月体（C）：根据占肾小球总数的百分比分别为C0（无）、C1（≤25%）和C2（>25%）；a：贫血定义为6个月~6岁<110 g/L，>6岁为<120 g/L；b：高尿酸血症定义为女孩血尿酸>360 μmol/L，男孩血尿酸>420 μmol/L |
表5 两组紫癜性肾炎患儿最后一次随访实验室检查结果的比较 |
| 项目 | 总体(n=130) | 达终点事件组(n=12) | 未达终点事件组(n=118) | 统计量(Z/χ2) | P值 |
|---|---|---|---|---|---|
| 年龄(岁) | 21.2(18.4，24.2) | 23.2(21.9，27.4) | 20.9(18.3，24.0) | -2.252 | 0.024 |
| 尿蛋白量(g/24 h) | 0.3(0.2，0.5) | 0.5(0.4，1.8) | 0.3(0.2，0.5) | -3.117 | 0.002 |
| 尿蛋白量分组 | 9.212 | 0.007 | |||
| 尿蛋白量<0.4 g/24 h[例(%)] | 87(66.9) | 4(33.3) | 83(70.3) | ||
| 尿蛋白量0.4~1.0 g/24 h[例(%)] | 31(23.8) | 4(33.3) | 27(22.9) | ||
| 尿蛋白量>1.0 g/24 h[例(%)] | 12(9.2) | 4(33.3) | 8(6.8) | ||
| 尿红细胞计数(个/HPF) | 0.8(0.3，2.1) | 0.6(0.3，7.8) | 0.8(0.3，1.9) | -0.476 | 0.634 |
| 血清白蛋白(g/L) | 47.3(44.8，49.5) | 43.6(40.5，47.1) | 47.7(45.5，49.7) | 2.554 | 0.011 |
| 血谷丙转氨酶(U/L) | 15.9(11.0，24.0) | 15.4(13.3，19.5) | 16.0(11.0，24.0) | -0.306 | 0.760 |
| 血谷草转氨酶(U/L) | 20.0(16.8，24.0) | 18.0(16.3，20.8) | 20.0(16.8，24.0) | -0.879 | 0.379 |
| 血胆固醇(mmol/L) | 4.2(3.7，4.8) | 4.6(3.7，5.5) | 4.2(3.7，4.7) | -1.536 | 0.124 |
| 血三酰甘油(mmol/L) | 1.0(0.7，1.3) | 1.0(0.8，1.2) | 0.9(0.7，1.3) | -0.575 | 0.565 |
| 血尿酸(μmol/L) | 342.0(290.5，398.0) | 501.0(359.8，536.5) | 333.0(283.0，389.3) | -3.684 | <0.001 |
| 血肌酐(μmol/L) | 61.5(49.9，76.3) | 107.5(100.8，459.8) | 59.1(49.0，71.3) | -5.044 | <0.001 |
| eGFR[ml·min-1·(1.73 m2)-1] | 128.5(109.3，138.1) | 55.1(9.5，79.3) | 129.8(117.8，138.8) | 5.695 | <0.001 |
| 注：HPF：高倍视野；eGFR：估算肾小球滤过率；数据形式除已注明外，余非正态分布的计量资料以M（P25，P75）形式表示 |
| [1] |
To validate the previously proposed classification criteria for Henoch-Schönlein purpura (HSP), childhood polyarteritis nodosa (c-PAN), c-Wegener granulomatosis (c-WG) and c-Takayasu arteritis (c-TA).Step 1: retrospective/prospective web-data collection for children with HSP, c-PAN, c-WG and c-TA with age at diagnosis <or=18 years. Step 2: blinded classification by consensus panel of a representative sample of 280 cases. Step 3: statistical (sensitivity, specificity, area under the curve and kappa-agreement) and nominal group technique consensus evaluations.827 patients with HSP, 150 with c-PAN, 60 with c-WG, 87 with c-TA and 52 with c-other were compared with each other. A patient was classified as HSP in the presence of purpura or petechiae (mandatory) with lower limb predominance plus one of four criteria: (1) abdominal pain; (2) histopathology (IgA); (3) arthritis or arthralgia; (4) renal involvement. Classification of c-PAN required a systemic inflammatory disease with evidence of necrotising vasculitis OR angiographic abnormalities of medium-/small-sized arteries (mandatory criterion) plus one of five criteria: (1) skin involvement; (2) myalgia/muscle tenderness; (3) hypertension; (4) peripheral neuropathy; (5) renal involvement. Classification of c-WG required three of six criteria: (1) histopathological evidence of granulomatous inflammation; (2) upper airway involvement; (3) laryngo-tracheo-bronchial involvement; (4) pulmonary involvement (x-ray/CT); (5) antineutrophilic cytoplasmic antibody positivity; (6) renal involvement. Classification of c-TA required typical angiographic abnormalities of the aorta or its main branches and pulmonary arteries (mandatory criterion) plus one of five criteria: (1) pulse deficit or claudication; (2) blood pressure discrepancy in any limb; (3) bruits; (4) hypertension; (5) elevated acute phase reactant.European League Against Rheumatism/Paediatric Rheumatology International Trials Organisation/Paediatric Rheumatology European Society propose validated classification criteria for HSP, c-PAN, c-WG and c-TA with high sensitivity/specificity.
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Henoch-Schönlein Purpura nephritis (HSPN) has been extensively studied in children but, its natural history in adults is much less known. A cohort of 250 adults suffering HSP was retrospectively analyzed for a median follow-up period of 14.8 yr. All patients had biopsies consistent with HSP (predominant IgA mesangial deposits) associated with purpura, bowel angina, and/or abdominal pain. At presentation, palpable purpura was present in 96% of patients, and arthritis was reported in 61%, and gastrointestinal involvement in 48%. Thirty-two percent of the patients showed renal insufficiency (Creatinine clearance [CrCl] <50 ml/min), usually associated with proteinuria (99%) and/or hematuria (93%). Endocapillary glomerulonephritis was the most frequent lesion on renal biopsy (61%). At the end of follow-up, patient survival was only 74%. The first cause of death was carcinoma (most of them of respiratory or digestive tract). Regarding renal function, 11% of patients reached end-stage renal failure, 13% exhibited severe renal failure (CrCl <30 ml/min), and 14% moderate renal insufficiency (CrCl <50 ml/min). Clinical remission defined as the absence of proteinuria, hematuria, and a normal renal function was achieved in only 20%. This is a retrospective study; therefore, it is not possible to demonstrate any steroid and/or cyclophosphamide efficacy in diminishing the incidence of renal insufficiency. Multivariate analysis demonstrated that renal function impairment and proteinuria level at presentation and, on renal biopsy, the degree of interstitial fibrosis, percentage of sclerotic glomeruli, and presence of glomeruli with fibrinoid necrosis were associated with a poor renal prognosis. The data indicate that clinical presentation of HSPN in adults is severe and its outcome relatively poor, worse than in children. Identification of clinical and histologic prognostic factors may permit the design of appropriate therapeutic prospective studies.
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| [5] |
All children with Henoch-Schoenlein glomerulonephritis (HSP-GN) and nephrotic-range proteinuria (> 40 mg/h/m2), treated at 5 university hospitals and in 1 central hospital in Finland during in 1990-1997, were analyzed retrospectively. The mean age of these 19 patients (8 girls, 11 boys) at the time of diagnosis was 9.9 years (range 4.6-15.1 years). A renal biopsy had been performed in all cases, giving findings according to the classification used in the International Study of Kidney Diseases in Children (ISKDC) of grade II (4 patients), grade III (10), grade IV (4) and grade V (1). Six patients underwent a second biopsy.The yearly incidence of nephrotic-range HSP-GN in Finland was 2 per 1 million children under 15 years of age. After a mean follow-up of 4.6 years (range 9 months-9.1 years), 3 patients (15.7%) had no signs of nephritis, 11 (57.9%) had proteinuria < 1 g/day or microscopic hematuria, 2 (10.5%) had proteinuria > 1 g/day, and 3 (15.7%) had developed ESRD or uremia. 47% of the patients needed medication for proteinuria at the time of the latest follow-up. The first kidney biopsy did not predict the outcome of HSP-GN, since all the patients with the poorest outcome had only ISKDC II-III findings in their first biopsy.According to our series, the morbidity in cases of HSP-GN with nephrotic-range proteinuria is high and a close clinical follow-up is needed. The treatment of HSP-GN patients should be based on the clinical presentation rather than on the biopsy findings.
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| [6] |
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| [7] |
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| [8] |
Equations to estimate glomerular filtration rate (GFR) are routinely used to assess kidney function. Current equations have limited precision and systematically underestimate measured GFR at higher values.To develop a new estimating equation for GFR: the Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI) equation.Cross-sectional analysis with separate pooled data sets for equation development and validation and a representative sample of the U.S. population for prevalence estimates.Research studies and clinical populations ("studies") with measured GFR and NHANES (National Health and Nutrition Examination Survey), 1999 to 2006.8254 participants in 10 studies (equation development data set) and 3896 participants in 16 studies (validation data set). Prevalence estimates were based on 16,032 participants in NHANES.GFR, measured as the clearance of exogenous filtration markers (iothalamate in the development data set; iothalamate and other markers in the validation data set), and linear regression to estimate the logarithm of measured GFR from standardized creatinine levels, sex, race, and age.In the validation data set, the CKD-EPI equation performed better than the Modification of Diet in Renal Disease Study equation, especially at higher GFR (P < 0.001 for all subsequent comparisons), with less bias (median difference between measured and estimated GFR, 2.5 vs. 5.5 mL/min per 1.73 m(2)), improved precision (interquartile range [IQR] of the differences, 16.6 vs. 18.3 mL/min per 1.73 m(2)), and greater accuracy (percentage of estimated GFR within 30% of measured GFR, 84.1% vs. 80.6%). In NHANES, the median estimated GFR was 94.5 mL/min per 1.73 m(2) (IQR, 79.7 to 108.1) vs. 85.0 (IQR, 72.9 to 98.5) mL/min per 1.73 m(2), and the prevalence of chronic kidney disease was 11.5% (95% CI, 10.6% to 12.4%) versus 13.1% (CI, 12.1% to 14.0%).The sample contained a limited number of elderly people and racial and ethnic minorities with measured GFR.The CKD-EPI creatinine equation is more accurate than the Modification of Diet in Renal Disease Study equation and could replace it for routine clinical use.National Institute of Diabetes and Digestive and Kidney Diseases.
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National High Blood Pressure Education Program Working Group on High Blood Pressure in Children and Adolescents. The fourth report on the diagnosis, evaluation, and treatment of high blood pressure in children and adolescents[J]. Pediatrics, 2004, 114(2 Suppl 4th Report): 555-576.
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余松林. 医学统计学[M]. 北京: 人民卫生出版社, 2002: 164-176.
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高春林, 章丹, 夏正坤, 等. 2004-2017年单中心9925例儿童肾活检病理分析[J]. 中华肾脏病杂志, 2019, 35(3): 177-183. DOI: 10.3760/cma.j.issn.1001-7097.2019.03.003.
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| [12] |
Henoch-Schönlein purpura arising in childhood could cause renal impairment or even an end-stage renal disease later in life. We aimed to assess long-term outcome of childhood Henoch-Schönlein purpura after 24 years.We studied a cohort of 26 boys and 26 girls who were treated for Henoch-Schönlein purpura at Helsinki University Hospital during 1964-83. Mean follow-up was 24.1 years (SD 6.0; 16.4-36.5). All participants were asked about their state of health in a questionnaire, and 47 (90%) were examined by a doctor. Patients' medical history data were obtained from health-care centres and regional hospitals.Seven (35%) of 20 adults who had severe Henoch-Schönlein purpura and glomerulonephritis at onset had renal impairment as adults, compared with two (7%) of 27 with mild or no renal symptoms at onset (relative risk 4.7, 95% CI 1.3-18.7). Relative risk for a poor outcome was 5.0 in women (1.1-32.5) and 2.0 in men (0.2-17.5). All patients with no renal symptoms at onset had a good outcome after 24 years of follow-up. Severity of first kidney biopsy finding did not correlate with risk of a poor outcome. 16 (70%) of 23 pregnancies had been complicated by hypertension, proteinuria, or both. Five (56%) of the nine women with complicated pregnancies had a poor renal outcome.Long-term follow-up of all patients who had Henoch-Schönlein purpura with severe renal symptoms at onset is needed during adulthood. All women who had even mild renal symptoms at onset of Henoch-Schönlein purpura should be carefully observed during and after pregnancy.
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| [13] |
Henoch-Schönlein purpura nephritis is a rare kidney disease leading to chronic kidney disease in a non-negligible percentage of patients. Although retrospective studies suggest beneficial effects of some therapies, prospective randomized clinical trials proving treatment efficacy are still lacking. The dilemma of spontaneous recovery even in patients with severe clinical and histologic presentation and of late evolution to chronic kidney disease in patients with mild initial symptoms renders it difficult for clinicians to expose patients to treatment protocols that are not evidence-based. A better understanding of the pathophysiology of progression to chronic kidney disease in Henoch-Schönlein purpura patients could be achieved by designing prospective international multicenter studies looking at determinants of clinical and histopathological evolution as well as possible circulating and urinary markers of progression. Such studies should be supported by a database available on the web and a new histologic classification of kidney lesions. This paper reports clinical, pathologic, and experimental data to be used for this strategy and to assist clinicians and clinical trial designers to reach therapeutic decisions.
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| [14] |
Factors predictive of renal outcome were investigated in 219 cases of biopsy-proven Henoch-Schönlein purpura nephritis (HSPN); 83 children and 136 adults enrolled in a national study were followed up for up to 27 years (median, 4.5 years).The criterion for defining disease progression was time elapsed until doubling of baseline creatinine level and until dialysis therapy. Age, sex, data at onset (renal function, proteinuria, hematuria, hypertension, and crescents), and data during follow-up (proteinuria and therapy) were tested as covariates.Multivariate Cox regression analysis indicated the following parameters as independent prognostic predictors: age (adults versus children, relative risk, 3.57; 95% confidence interval, 1.18 to 10.79; P = 0.024 for creatinine level doubling; relative risk, 14.89; 95% confidence interval, 1.72 to 129.07; P = 0.014 for dialysis therapy), sex (females versus males, relative risk, 5.71; 95% confidence interval, 1.67 to 19.55; P = 0.006 for creatinine level doubling; relative risk, 26.03; 95% confidence interval, 2.64 to 256.73; P = 0.005 for dialysis therapy), and mean proteinuria during follow-up (for each 1 g/d of protein increase, relative risk, 1.77; 95% confidence interval, 1.35 to 2.32; P < 0.001 for creatinine level doubling; relative risk, 1.73; 95% confidence interval, 1.18 to 2.52; P = 0.005 for dialysis therapy). Information for mean proteinuria levels during follow-up increased the sensitivity at logistic regression to 62.5%, with dialysis therapy as the end point. No data detected at diagnosis, including renal function impairment, proteinuria, hypertension, and crescentic nephritis (involving > 50% of glomeruli in only 2.6%), were significantly related to functional decline at multivariate Cox.This analysis indicates that, even more than when decreased renal function, severe proteinuria, hypertension, or crescents are present at onset, the risk for progression of HSPN (greater in adults and females) was associated with increasing mean proteinuria levels during follow-up.
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| [15] |
<i>Background/Aim:</i> The aim of this retrospective study was to evaluate the presentation, clinical and pathological manifestations and outcome of the Henoch-Schönlein purpura (HSP) nephritis in children. <i>Methods:</i> Clinical and laboratory data of 443 children with HSP nephritis aged between 3 and 16 years from 16 pediatric nephrology reference centers were analyzed retrospectively. The biopsy findings were graded according to the classification developed by the International Study of Kidney Disease in Children (ISKDC). <i>Results:</i> Renal biopsy was performed in 179 of the patients with HSP nephritis. The most common presenting clinical finding in patients who were biopsied was nephrotic range proteinuria (25%) which was followed by nephritic-nephrotic syndrome (23.5%). The biopsy findings according to the ISKDC were as follows: class I: 8.3%; II: 44.1%; III: 36.3%; IV: 6.7%; V: 3.3%; VI: 1.1%. All of the patients who developed end-stage renal disease had nephritic-nephrotic syndrome at presentation. Of 443 patients, 87.2% had a favorable outcome and 12.8% had an unfavorable outcome. The overall percentage of children who developed end-stage renal disease at follow-up was 1.1%. Logistic regression analysis did not show any association of initial symptoms and histology with outcome. <i>Conclusion:</i> In the presented cohort, the presence of crescents in the first biopsy or presenting clinical findings did not seem to predict the outcome of HSP nephritis in children. We conclude that children with HSP nephritis even with isolated microscopic hematuria and/or mild proteinuria should be followed closely.
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| [16] |
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| [17] |
Factors predictive of renal outcome were studied in 78 children with Henoch-Schönlein nephritis followed up for as long as 17 (mean 5.2) years. Patients with a good outcome (74%) were healthy or had microalbuminuria or mild proteinuria at the final follow-up (FU), and those with poor outcome (26%) had active renal disease or chronic kidney disease at stages IV-V. Patients with mild symptoms at onset (hematuria + or - mild proteinuria) had a poor outcome in 15% of cases versus 41% of those with severe symptoms (nephritic or nephrotic syndrome or nephritic-nephrotic picture) (p = 0.011). However, among patients with mild proteinuria at onset, 18% showed a poor prognosis; non-nephrotic proteinuria with a urine albumin/creatinine ratio at a cut-off value of >144 mg/mmol at the 1-year FU was predictive of a poor outcome. Among 59 biopsied patients, 37% of those with advanced histological findings [International Study of Kidney Disease in Children (ISKDC) stages III-V] had a poor outcome compared to none of those with mild findings (ISKDC stages I-II) (p = 0.0015). Patients with a poor outcome were older at onset, had more proteinuria, and lower glomerular filtration rate at the 1-year FU compared with patients with a good outcome. Multivariate analysis showed that proteinuria at the 1-year FU and the ISKDC grading score of the renal biopsy were the two most discriminant factors of a poor prognosis.
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| [18] |
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| [19] |
Henoch-Schönlein purpura nephritis (HSPN), a small-vessel vasculitis, shares renal pathological features with immunoglobulin A nephropathy. Oxford classification of immunoglobulin A nephropathy pathology has been updated to the MEST-C score, but its application in HSPN remains unresolved.Two hundred and thirteen patients with biopsy-proven HSPN were retrieved from the Seoul National University Hospital between 2000 and 2017. Renal outcome risks (i.e., end-stage renal disease or doubling of serum creatinine) were evaluated according to MEST-C scores after stratification by age: 113 children aged < 18 years (9.2 ± 3.6 years) and 100 adults aged ≥18 years (38.6 ± 18.3 years). We pooled our data with four previous cohort studies in which MEST or MEST-C scores were described in detail.Twenty-one child (19%) and 16 adult (16%) patients reached the renal outcome during the median follow-up periods of 12 years and 13 years, respectively (maximum 19 years). In children, M1 and T1/T2 scores revealed worse renal outcomes than did M0 and T0 scores, respectively, whereas the T score was the only factor related to worse outcomes in adult patients after adjusting for multiple clinical and laboratory variables. The pooled data showed that M1, S1, and T1/T2 in children and E1 and T1/T2 in adults were correlated with poorer renal outcomes than those of their counterpart scores.The Oxford classification MEST-C scores can predict long-term renal outcomes in patients with HSPN.
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| [20] |
Children with Henoch-Schönlein purpura nephritis (HSPN) have an increased risk of chronic kidney disease (CKD). Renal biopsy diagnostic of HSPN is graded using the International Study of Kidney Disease in Children criteria, which do not predict outcomes. The 2016 Oxford Classification's MEST-C scoring system predicts outcomes in adults with histologically identical IgA nephropathy, but evidence of its utility in pediatric HSPN is lacking. Our hypothesis was that MEST-C scores predict outcomes in children with HSPN.A retrospective cohort analysis of data from 32 children with HSPN who underwent renal biopsy was performed. We used logistic regression and receiver operator characteristic curves to analyze the ability of MEST-C to predict the composite outcome of hypertension (blood pressure ≥ 95% for age/sex/height), CKD (estimated glomerular filtration rate < 90 mL/min/1.73 m), or proteinuria (urine protein-to-creatinine ratio > 0.2 mg/mg).The median age at diagnosis was 7.9 years (IQR 5.8, 11.7); 56% were male, 19% were Hispanic, and 9% were Black. After a median follow-up of 2.7 years, 38% of patients (n = 12) reached the outcome. S1 score was significantly associated with the outcome (OR 7.9, 95% CI 1.5-42.6). S1 accurately predicted the outcome (area under the curve 0.72, 95% CI 0.55-0.88) with 58.3% sensitivity and 85.0% specificity, indicating a positive predictive value of 70.0% and a negative predictive value of 77.3%.S1 accurately predicted our composite outcome of hypertension, CKD, and proteinuria in a diverse cohort of U.S. children with HSPN. Further investigation is warranted to validate these findings.
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| [21] |
The widely used International Study of Kidney Disease in Children (ISKDC) classification for Henoch-Schönlein purpura nephritis (HSPN) does not completely correlate with the clinical presentation and long-term prognosis of this disease. Primary IgA nephropathy (IgAN) and HSPN share common features; thus, the Oxford classification of IgAN might be useful in predicting the long-term outcomes of HSPN. However, its value has not been confirmed in children with HSPN.We selected children with HSPN diagnosed between 2003 and 2015, and reclassified their renal biopsies according to the Oxford classification scoring system. The primary outcome was impaired renal function, and remission of proteinuria and clinical remission were secondary outcomes.We included 104 patients (58 males, 46 females) with a median age of 10 (4-17) years. Mesangial hypercellularity (M1) was strongly associated with proteinuria, and tubular atrophy/interstitial fibrosis (T1&2) and C2 (with crescents in > 25% of glomeruli) were associated with reduced estimated glomerular filtration rate (eGFR) at the time of biopsy. Patients with M1, endocapillary proliferation (E1), segmental glomerulosclerosis (S1), and crescents (C1&2) were more likely to have been treated with high-dose methylprednisolone. At univariate time-dependent analyses, S1 was strongly associated with the primary outcome (p = 0.025), whereas T1&2 was significantly negatively associated with proteinuria remission (p = 0.035) and clinical remission (p = 0.038).Our findings suggest that the Oxford classification is valid for children with HSPN. S and T lesions, which are ignored in the ISKDC classification, can be used to assess renal outcomes of HSPN, and such assessments are not affected by currently available treatments. The value of M, E and C lesions in predicting response to therapy and renal outcome warrants further study.
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| [22] |
In Henoch-Schönlein nephritis (HSN), a risk factor for unfavorable outcome is prolonged proteinuria, but the value of renal biopsies in prognosis assessment is debatable.We evaluated serial renal biopsies from 26 HSN patients. Follow-up biopsy occurred at median 2.1 years after diagnostic biopsy. Patients formed two groups at the follow-up biopsy: patients without proteinuria (group I; n = 11) and with proteinuria (group II; n = 15). Biopsies underwent evaluation according to three classifications: International Study of Kidney Disease in Children (ISKDC), Oxford (MEST-C), and semiquantitative classification (SQC) including an activity and chronicity score. Analysis also included expression of pro-fibrotic (alpha-smooth muscle actin and vimentin) and inflammatory (P-selectin glycoprotein ligand-1) molecules in the diagnostic biopsy specimens. Definition of unfavorable outcome was active renal disease or reduced renal function at last follow-up.Between the biopsies, SQC chronicity score increased in 22 (85%) patients, whereas activity score and ISKDC grade decreased in 21 (81%) and 17 (65%), respectively. Of the MEST-C parameters, endocapillary proliferation (from 83 to 13%; p < 0.001) and crescents (from 63 to 25%; p = 0.022) showed significant reduction, and segmental glomerulosclerosis (from 38 to 79%; p = 0.006) significant increment. These changes occurred similarly in groups I and II. Expression of the pro-fibrotic and inflammatory molecules showed no clinically significant differences between groups I and II. None in group I and five (33%) patients in group II had unfavorable outcome (p = 0.053).Our results suggest that follow-up biopsies provide limited additional information to clinical symptoms in HSN outcome prediction.
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| [23] |
Henoch-Schönlein purpura (HSP) is the most common vasculitis in childhood. The long-term prognosis is variable and depends on renal involvement. The aims of this study were to investigate the clinical and laboratory characteristics of our HSP patients, to identify the risk factors for the development of Henoch-Schönlein purpura nephritis (HSPN) and to assess the efficacy of the Oxford Classification system for predicting renal outcomes.We performed a retrospective review of HSP patients who admitted to our center between 2001 and 2016, and were < 18 years on admission.A total of 1120 children with HSP were analyzed. Their mean age was 7.4 ± 3.4 years. At onset, purpura was present in all cases, arthritis/arthralgia in 42.4%, abdominal involvement in 39% and renal involvement in 37%. Risk factors for the development of nephritis were age ≥ 8 years, atypical distribution of purpura, ESR > 20 mm/h and abdominal pain. Renal biopsy was performed on 75 patients before immunosuppressive treatment. The mesangial score was strongly associated with proteinuria. Segmental glomerulosclerosis, tubular atrophy/interstitial fibrosis, and crescent formation of ≥ 50% were associated with reduced eGFR at the time of biopsy. A Kaplan-Meier plot showed that segmental glomerulosclerosis and tubular atrophy/interstitial fibrosis significantly predict poor renal outcome.The long-term morbidity of HSP is predominantly attributed to renal involvement. Patients with HSP, who have a high risk to develop nephritis, could be followed for longer periods of time. The Oxford classification is useful in predicting long-term outcomes of HSPN.
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| [24] |
There is controversy over whether IgA nephropathy (IgAN) and Henoch-Schönlein purpura nephritis (HSPN) are the same diseases. This study focuses on the clinicopathological comparison between HSPN and IgAN in children.Children with IgAN and HSPN who had a diagnostic renal biopsy were enrolled. This study collected the clinical data of patients at biopsy, re-evaluated the pathological lesions of patients according to the Oxford Classification (MEST-C), and made a retrospective comparison between IgAN and HSPN on different stratifications of the course (Tc) and proteinuria.A total of 142 children with IgAN and 57 children with HSPN were enrolled. Various stratification showed the same result, which suggested that IgAN showed more mesangial proliferation (M). HSPN showed more segmental glomerulosclerosis in the Tc > 12 m group than IgAN (S 60.0% vs. 9.10%, P = 0.008). In the non-nephrotic-range and nephrotic-range proteinuria group, there were no significant differences in MEST-C scores between IgAN and HSPN.M is more common in IgAN. HSPN had more S than IgAN over the course of more than 12 months. These results indicate the differences in the pathogenesis in IgAN and HSPN. We propose early biopsy and active treatment of HSPN within 12 months to delay the development of chronic lesions.
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| [25] |
Background: Henoch-Schonlein purpura nephritis (HSPN) is a form of small vessel vasculitis associated with purpura and IgA deposition in the glomeruli. The International Study of Kidney Disease in Children (ISKDC) classification predicts renal prognosis in children with HSPN, but not in adults. Additionally, it is not well known whether the Oxford classification 2016 and/or the Japanese Histologic classification (JHC) are associated with renal outcome. Herein, we investigated the relationship between pathological characteristics and renal outcome among adult patients with HSPN.Methods: A multicenter retrospective cohort study was conducted in adult patients with HSPN who underwent renal biopsy between 2004 and 2014. Two nephrologists classified each patient according to the Oxford classification 2016, JHC, and the ISKDC classification. Renal outcome was defined by a 30% decline in the eGFR and/or end-stage kidney disease.Results: We enrolled 74 adult patients with HSPN (mean age, 47.8 +/- 17.4 years; mean eGFR, 76.4 +/- 25.8 ml/min/1. 73 m(2) ; median proteinuria, 1.40 [IQR: 0.70-2.38] g/day). During a mean follow-up period of 68.0 +/- 33.0 months, fourteen patients (18.9%) reached the renal outcome, and all 14 had received immunosuppressive therapy. The log-rank test revealed that event-free renal survival was significantly shorter in patients with endocapillary proliferation (El) according to the Oxford classification than in those with E0 (p = 0.0072). However, the JHC, ISKDC classification and other Oxford lesions could not demonstrate a significant difference in event-free renal survival. In a multivariate Cox model adjusted for clinical and pathological factors, age (HR, 1.57; 95% CI, 1.12-2.21) and E lesion (HR, 6.71; 95% CI, 1.06-42.7) were independent risk factors for renal outcome.Conclusions: Endocapillary proliferation is significantly associated with renal outcome in adult patients with HSPN, including those receiving immunosuppressive therapy. Other Oxford classification lesions, JHC, and ISKDC classification were not associated with renal outcome.
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| [26] |
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| [27] |
Henoch-Schönlein purpura nephritis (HSPN) shares many similarities with IgA nephropathy. We aimed to analyze the predictive value of the International Study of Kidney Disease in Children (ISKDC) classification and the updated Oxford classification for IgA nephropathy in HSPN patients.Data of 275 HSPN patients (aged≥14 years) were retrieved, and all of them underwent a renal biopsy. We re-classified the biopsies according to the ISKDC classification and the updated Oxford classification to analyze their correlations with clinical features and renal outcomes. The renal endpoints were defined as ≥30% reduction in baseline estimated glomerular filtration rate (eGFR) in 2 years, doubling of serum creatinine (Scr) or end stage renal disease.During follow-up period of 56(30,86) months, 30(10.9%) patients reached renal endpoints. Segmental sclerosis was the only pathological feature independently associated with renal endpoints (HR 4.086, 95%CI 1.111-15.026, P = 0.034). Tubular atrophy/ interstitial fibrosis was associated with eGFR and Scr levels, and its correlation with renal endpoints was found by univariate analysis. Endocapillary hypercellularity was associated with 24 h urine protein and is of prognostic value in univariate analysis. Mesangial hypercellularity was not associated with clinical features or renal endpoints. Crescents were associated with 24 h urine protein, Scr and eGFR levels, but both ISKDC and updated Oxford classifications of crescents were not associated with renal endpoints by multivariate analysis.The updated Oxford classification can help in disease management and renal outcome prediction of HSPN.
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| [28] |
The Oxford Classification of IgA nephropathy does not account for glomerular crescents. However, studies that reported no independent predictive role of crescents on renal outcomes excluded individuals with severe renal insufficiency. In a large IgA nephropathy cohort pooled from four retrospective studies, we addressed crescents as a predictor of renal outcomes and determined whether the fraction of crescent-containing glomeruli associates with survival from either a ≥50% decline in eGFR or ESRD (combined event) adjusting for covariates used in the original Oxford study. The 3096 subjects studied had an initial mean±SD eGFR of 78±29 ml/min per 1.73 m and median (interquartile range) proteinuria of 1.2 (0.7-2.3) g/d, and 36% of subjects had cellular or fibrocellular crescents. Overall, crescents predicted a higher risk of a combined event, although this remained significant only in patients not receiving immunosuppression. Having crescents in at least one sixth or one fourth of glomeruli associated with a hazard ratio (95% confidence interval) for a combined event of 1.63 (1.10 to 2.43) or 2.29 (1.35 to 3.91), respectively, in all individuals. Furthermore, having crescents in at least one fourth of glomeruli independently associated with a combined event in patients receiving and not receiving immunosuppression. We propose adding the following crescent scores to the Oxford Classification: C0 (no crescents); C1 (crescents in less than one fourth of glomeruli), identifying patients at increased risk of poor outcome without immunosuppression; and C2 (crescents in one fourth or more of glomeruli), identifying patients at even greater risk of progression, even with immunosuppression.Copyright © 2017 by the American Society of Nephrology.
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| [29] |
Henoch-Schönlein purpura is the most common vasculitis in children. Its long-term prognosis depends on renal involvement. The management of Henoch-Schönlein purpura nephritis (HSPN) remains controversial. This study reports the prognosis of children with HSPN presenting with class 2 International Study of Kidney Disease in Children (ISKDC) nephritis.All children with HSPN class 2 diagnosed between 1995 and 2015 in four pediatric nephrology centers were included, and clinical and biological data were collected from the medical files. The primary endpoint was proteinuria remission defined as a proteinuria <200 mg/L.Ninety-two children were included in the study with a median follow-up of 36 (6-120) months; 28% had nephrotic syndrome, 31% proteinuria >3 g/L, 52% proteinuria between 1 and 3 g/L, and 18% proteinuria <1 g/L. Forty-seven percent of patients received orally treatment with steroids alone, 37% received methylprednisolone pulses followed by steroids orally, 18% received no steroids. Although 85% reached remission during follow-up, 12% did not maintain complete remission over time so that only 75% remained in complete remission by the end of the follow-up. Univariate analysis found a higher likelihood of remission in patients with higher proteinuria at disease onset (p = 0.009). This trend was not found in the multivariate analysis after adjusting for treatments, as patients with higher proteinuria were most often treated with steroids.Our study shows that one fourth of patients with HSPN class 2 remain proteinuric and thus carry the risk of developing chronic kidney disease over the long term. This finding, together with the better outcome of patients treated with steroids, is in favor of using high-dose steroids orally or IV in these patients.
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| [30] |
We studied the long-term outcome of 64 children with biopsy-proven Schönlein-Henoch purpura (SHP) nephritis over 1-23 years of follow-up. Overall renal survival 10 years after onset was 73%. Multivariate logistic regression analysis identified initial renal insufficiency (P=0.004), nephrotic syndrome (P=0.037), and the severity of histological alterations, as defined by the proportion of glomerular crescents (P=0.051), as significant independent predictors of progressive renal failure. Four patients followed for more than 19 years showed glomerular damage after transient recovery. Eight children with crescentic nephritis associated with a rapidly progressive course and/or persistent nephrotic syndrome were treated by at least seven sessions of plasma exchange (PE) within 16 weeks of onset of purpura. During treatment serum creatinine levels dropped in each patient from a mean of 2.3 to 1.1 mg/dl, followed by a rebound increase. Repeated courses of PE in 5 patients produced comparable responses. Four patients undergoing PE reached end-stage renal disease at 1.2.-3.7 years after onset, whilst 3 finally were in preterminal renal failure (creatinine 3.2-6.1 mg/dl after 7-13.5 years), and 1 patient reached a normal glomerular filtration rate. Our experience suggests that initial renal insufficiency is the best single predictor of the further clinical course in children with SHP nephritis. Early PE appears to delay the progression in some patients with severe, rapidly progressive forms of the disease.
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| [31] |
Henoch-Schönlein purpura (HSP) is the most common vasculitis in childhood. The long-term prognosis is variable and depends on renal involvement. The aim of this study was to evaluate both clinical features of the children with HSP and the prognoses of short- and long-term outcome of patients diagnosed as HSP nephritis (HSN). This is a retrospective data study of all children with HSP hospitalized from January 1991 to December 2005. The patients with HSN were classified according to their initial presentation, histologic findings, type of treatment and clinical outcome. All patients have been evaluated once every 2 months. Fifty-three of the patients had kidney biopsies. The patient population consisted of 141 children included 78 boys (55.3%) and 63 girls (44.7%) ranging in age at disease onset from 2 to 17 (8.9+/-3.29) years. Renal involvement was determined in 58.1%. Nephrotic and/or nephritic syndrome were found to be an unfavorable predictor both for short and long-term outcome (P<0.05). However, 35% of these patients and 62% of them showed complete remission after 6 months and long-term course. Overall prognosis of HSN is relatively good and long-term morbidity is predominantly associated with initial presentation and renal involvement.
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| [32] |
This study investigated predictors of renal survival in children with Henoch-Schönlein purpura glomerulonephritis. Records of patients with Henoch-Schönlein purpura glomerulonephritis evaluated at our center, from 1953-1990, were reviewed. Data were abstracted from records of patients seen within 5 years. Others were mailed a questionnaire or contacted by telephone. Primary outcome measures were renal survival and presence of urinary abnormalities or hypertension. Of the 65 eligible patients with Henoch Schönlein purpura glomerulonephritis, follow-up data was obtainable for 81.5%. The median follow-up was 20 years. At last follow-up, 66% of patients had normal renal function and urinalyses, and 21% had progressed to end-stage renal disease. The only factor associated with the development of end-stage renal disease was the use of cytotoxic agents. There are no features at initial presentation that identify children at risk of disease progression. Close follow-up of all children with Henoch Schönlein purpura glomerulonephritis is warranted.
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| [33] |
We evaluated the renal hemodynamics and the urine protein excretion rates of 73 children with Henoch-Schonlein nephritis (HSN). In 40 children we also performed a renal biopsy. The glomerular filtration rate (GFR) and effective renal plasma flow were determined by the clearances of inulin and para-aminohippurate during water diuresis. Urine albumin and IgG excretion were assessed in short-term timed samples. The mean GFR at the first examination was reduced in the HSN patients and most reduced in those with nephrotic proteinuria. There was an inverse correlation between the GFR at the first examination and the amount of albuminuria and urinary IgG excretion. Among the 40 patients with some degree of proteinuria who underwent a renal biopsy, 9 of 13 patients with mild to moderate proteinuria had severe morphological changes. GFR correlated inversely and fractional albumin and IgG excretion directly with the severity of the pathological findings on the biopsy, and with segmental and global sclerosis, the grade of mesangial proliferation, and interstitial inflammation. In conclusion, GFR is moderately reduced early in HSN and more reduced in patients with more proteinuria and in those with more advanced morphological changes. Moreover, even mild to moderate proteinuria may indicate severe morphological changes, which increase the indications for early renal biopsy in these patients.
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| [34] |
Immunoglobulin-A vasculitis (IgAV) is classically a childhood small-sized blood vessel vasculitis with predominant involvement of the skin. Gastrointestinal and joint manifestations are common in patients diagnosed with this condition. Nephritis, which is more severe in adults, constitutes the most feared complication of this vasculitis. The molecular bases underlying the origin of IgAV have not been completely elucidated. Nevertheless, several pieces of evidence support the claim that genes play a crucial role in the pathogenesis of this disease. The human leukocyte antigen (HLA) region is, until now, the main genetic factor associated with IgAV pathogenesis. Besides a strong association with HLA class II alleles, specifically HLA-DRB1 alleles, HLA class I alleles also seem to influence on the predisposition of this disease. Other gene polymorphisms located outside the HLA region, including those coding cytokines, chemokines, adhesion molecules as well as those related to T-cells, aberrant glycosylation of IgA1, nitric oxide production, neoangiogenesis, renin-angiotensin system and lipid, Pyrin and homocysteine metabolism, may be implicated not only in the predisposition to IgAV but also in its severity. An update of the current knowledge of the genetic component associated with the pathogenesis of IgAV is detailed in this review.Copyright © 2018 The Author(s). Published by Elsevier B.V. All rights reserved.
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所有作者声明无利益冲突
王美秋：数据收集、数据整理、统计学分析、论文撰写；王忍：数据收集；何旭、张沛：研究指导；夏正坤：论文修改、经费支持；高春林：研究指导、论文修改、经费支持
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表1 两组紫癜性肾炎患儿基线临床资料和实验室检查结果的比较表2 达终点事件组和未达终点事件组患儿应用药物种类的比较[例(%)]表3 两组紫癜性肾炎患儿肾组织病理学改变的比较[例（%）]表4 肾脏不良预后的影响因素分析（Cox回归分析，n=130）
图1 肾小球节段性硬化对紫癜性肾炎患儿肾脏累积生存率的影响（Kaplan Meier生存曲线）图2 肾小球节段性硬化预测紫癜性肾炎患儿肾脏不良预后的诊断价值（受试者工作特征曲线）表5 两组紫癜性肾炎患儿最后一次随访实验室检查结果的比较/
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