Abstract Background Previously, several studies have indicated that pediatric IgA nephropathy (IgAN) might be different from adult IgAN, and treatment strategies might be also different between pediatric IgAN and adult IgAN. Methods We analyzed two prospective cohorts established by pediatric and adult nephrologists, respectively. A comprehensive analysis was performed investigating the difference in clinical and pathological characteristics, treatment, and prognosis between children and adults with IgAN. Results A total of 1015 children and 1911 adults with IgAN were eligible for analysis. More frequent gross hematuria (88% vs. 20%, p < 0.0001) and higher proteinuria (1.8 vs. 1.3 g/d, p < 0.0001) were seen in children compared to adults. In comparison, the estimated glomerular filtration rate (eGFR) was lower in adults (80.4 vs. 163 ml/min/1.73 m 2 , p < 0.0001). Hypertension was more prevalent in adult patients. Pathologically, a higher proportion of M1 was revealed (62% vs. 39%, p < 0.0001) in children than in adults. S1 (62% vs. 28%, p < 0.0001) and T1–2 (34% vs. 8%, p < 0.0001) were more frequent in adults. Adjusted by proteinuria, eGFR, and hypertension, children were more likely to be treated with glucocorticoids than adults (87% vs. 45%, p < 0.0001). After propensity score matching, in IgAN with proteinuria > 1 g/d, children treated with steroids were 1.87 (95% CI 1.16–3.02, p = 0.01) times more likely to reach complete remission of proteinuria compared with adults treated with steroids. Conclusions Children present significantly differently from adults with IgAN in clinical and pathological manifestations and disease progression. Steroid response might be better in children. Graphical Abstract
Objective: To investigate the clinical characteristics and related factors of corticosteroid induced adrenal crisis (AC) in children with primary nephrotic syndrome (NS). Methods: Case control study. The case group included 7 children aged 1 to 18 years with NS combined with AC hospitalized in Peking University First Hospital from January 2016 to May 2021 (AC group). According to the ratio of case group: control group 1: 4, 28 children aged 1 to 18 years who were diagnosed with NS without AC during the same period were matched as controls (non-AC group). Clinical data were collected. The clinical characteristics of AC were described. The clinical parameters were compared between the 2 groups by t test, Mann-Whitney U test or Fisher's test. Receiver operating characteristic (ROC) curve was used to analyze the cutoff values of clinical parameters for prediction of AC. Results: The AC group included 4 boys and 3 girls aged 6.9 (4.6, 10.8) years. The non-AC group included 20 boys and 8 girls aged 5.2 (3.3, 8.4) years. All AC events occurred during the relapse of NS with infection. Seven children had gastrointestinal symptoms such as nausea, vomiting and abdominal pain. Six children had poor mental state or impaired consciousness. No significant differences in NS course, corticosteroid treatment course, corticosteroid type, steroid dosage, steroid medication interval, the proportion of gastroenteritis and fever existed between the two groups (all P>0.05). Compared with the non-AC group, the duration from the onset of the relapse of NS until hospitalization in the AC group was significantly shorter (0.2 (0.1, 0.6) vs. 1.0 (0.4, 5.0) month,U=25.50, P=0.005). The 24 h urinary total protein (UTP) level was significantly higher in the AC group (193 (135, 429) vs. 81 (17, 200) mg/kg, U=27.00,P=0.036) than the non-AC group. The serum albumin level in the AC group was significantly lower((13.1±2.1) vs. (24.5±8.7) g/L,t=-6.22,P<0.001) than the non-AC group. There were significantly higher total white blood cell counts ((26±9)×109vs. (11±5)×109/L,t=4.26,P=0.004), percentage of neutrophils (0.71±0.08 vs. 0.60±0.19,t=2.56,P=0.017) and the proportion of children with C reactive protein level≥8 mg/L (3/7 vs. 0,P=0.005) in the AC group than in the non-AC group. ROC curve analysis showed that the cutoff value of 24 h UTP was 122 mg/(kg·d) with a sensitivity of 100.0% and specificity of 70.4%. The cutoff value of serum albumin was 17.0 g/L with a sensitivity of 100.0% and specificity of 82.1%. Conclusions: Gastrointestinal symptoms and poor mental state were prominent manifestations of AC in children with NS. High 24 h UTP level, low serum albumin level, high peripheral white blood cell counts, high neutrophils percentage, and high C-reactive protein level during the early stage of NS relapse may be related to the occurrence of AC in children with NS.目的： 探讨儿童原发性肾病综合征（NS）合并糖皮质激素相关肾上腺危象（AC）的临床特点及相关因素。 方法： 病例对照研究。选取2016年1月至2021年5月在北京大学第一医院儿科确诊的1~18岁全部原发性NS合并AC患儿7例为病例组（AC组）。按病例组∶对照组1∶4比例匹配同期住院同年龄原发性NS不合并AC 28例患儿为对照组（非AC组）。收集并分析两组患儿临床资料，组间比较采用t检验、Mann-Whitney U检验或Fisher确切概率法。采用受试者工作特征（ROC）曲线分析AC的预测指标截断值。 结果： AC组男4例、女3例，年龄6.9（4.6，10.8）岁；非AC组男20例、女8例，年龄5.2（3.3，8.4）岁。AC均发生在NS复发后未缓解期，存在感染诱因。7例均有胃肠道症状如恶心、呕吐、腹痛，6例有精神差或意识障碍。两组间NS病程、激素疗程、剂型、剂量、服药间隔方式、胃肠炎和发热的比例差异均无统计学意义（均P>0.05）。与非AC组比较，AC组患儿从NS复发距入院时间短［0.2（0.1，0.6）比1.0（0.4，5.0）个月，U=25.50，P=0.005］，24 h尿蛋白定量（UTP）高［193（135，429）比 81（17，200）mg/kg，U=27.00，P=0.036］，血白蛋白水平低［（13.1±2.1）比（24.5±8.7）g/L，t=-6.22，P<0.001］，外周血白细胞计数高［（26±9）×109 比（11±5）×109/L，t=4.26，P=0.004］，中性粒细胞比值高（0.71±0.08 比 0.60±0.19，t=2.56，P=0.017），C反应蛋白≥8 mg/L患儿比例高（3/7 比 0，P=0.005）。ROC曲线分析示24 h UTP预测AC的截断值为122 mg/（kg·d），灵敏度100.0%，特异度70.4%；血白蛋白截断值为17.0 g/L，灵敏度100.0%，特异度82.1%。 结论： 胃肠道症状和精神差是NS患儿合并AC突出表现。NS复发早期24 h UTP高、血白蛋白水平低、外周血白细胞计数高、中性粒细胞比值高及C反应蛋白高可能与AC发生有关。.
Abstract Primary coenzyme Q10 deficiency‐1, caused by COQ2 disease‐causing variants, is an autosomal recessive disorder, and genetic testing is the gold standard for diagnosing this condition. A Chinese boy with steroid‐resistant nephrotic syndrome, focal segmental glomerulosclerosis, and progressive kidney insufficiency was included in the study. Electron microscopy revealed the glomerular basement membrane with irregular thickness and lamellation with diffuse effacement of foot processes in the podocytes, and swollen mitochondria with abnormal cristae in the podocytes. Coenzyme Q10 supplementation started about 3 weeks after the onset of mild kidney dysfunction did not improve the proband's kidney outcome. Proband‐only whole‐exome sequencing and Sanger sequencing revealed two heteroallelic COQ2 variants: a maternally inherited novel variant c.1013G > A[p.(Gly338Glu)] in exon 6 and a variant of unknown origin c.1159C > T[p.(Arg387*)] in exon 7. Subsequent long‐read sequencing demonstrated these two variants were located on different alleles. Our report extends the phenotypic and genotypic spectrum of COQ2 glomerulopathy.
Abstract Background This study investigated the association between urinary epidermal growth factor (EGF) and complete remission (CR) of proteinuria in children with IgA nephropathy (IgAN). Methods We included 108 patients from the Registry of IgA Nephropathy in Chinese Children. The urinary EGF at the baseline and follow-up were measured and normalized by urine creatinine (expressed as uEGF/Cr). The person-specific uEGF/Cr slopes were estimated using linear mixed-effects models for the subset of patients with longitudinal data of uEGF/Cr. Cox models were used to analyze the associations of baseline uEGF/Cr and uEGF/Cr slope with CR of proteinuria. Results Patients with high baseline uEGF/Cr were more likely to achieve CR of proteinuria (adjusted HR 2.24, 95% CI: 1.05–4.79). The addition of high baseline uEGF/Cr on the traditional parameters significantly improved the model fit for predicting CR of proteinuria. In the subset of patients with longitudinal data of uEGF/Cr, high uEGF/Cr slope was associated with a higher likelihood of CR of proteinuria (adjusted HR 4.03, 95% CI: 1.02–15.88). Conclusions Urinary EGF may be a useful noninvasive biomarker for predicting and monitoring CR of proteinuria in children with IgAN. Impact High levels of baseline uEGF/Cr (>21.45 ng/mg) could serve as an independent predictor for CR of proteinuria. The addition of baseline uEGF/Cr on the traditional clinical pathological parameters significantly improved the fitting ability for the prediction of CR of proteinuria. Longitudinal data of uEGF/Cr were also independently associated with CR of proteinuria. Our study provides evidence that urinary EGF may be a useful noninvasive biomarker in the prediction of CR of proteinuria as well as monitoring therapeutic response, thus guiding treatment strategies in clinical practice for children with IgAN.
Abstract Background Thromboembolism is a life-threatening, limb-threatening or organ-threatening complication that occurs in patients with primary nephrotic syndrome (NS). There are few studies on the spectrum, complications and outcomes of thrombosis in children with NS. This study aimed to determine the spectrum of thrombosis and its relationship with the nephrotic state, treatment and outcomes in children and adolescents with primary NS. Methods The medical records of subjects aged 1–18 years with NS complicated with thromboembolism treated at our centre within the last 26 years were retrieved. Data on the status of NS, site, symptoms and signs, laboratory investigations, diagnosis, treatment, complications and outcomes of thrombosis were collected and reviewed retrospectively. A severe complication was defined as a condition associated with thrombosis requiring a special diagnostic modality to confirm or a specific treatment such as surgical intervention. The outcome of thrombosis was defined as the status of thrombosis, as determined by imaging methods and the functional status with respect to the anatomic sites of thrombosis at the last follow-up. The permanent dysfunction of an organ or limb related to thrombosis was defined as a sequela. Results We observed thrombosis in 1.4% (27/1995) of subjects with NS during the study period. There were 27 subjects with thrombosis, including 21 males and 6 females. Thrombosis was observed in 51.9% (14/27) of the study participants with steroid resistant NS. Most episodes of thrombosis occurred during the active stage of NS; however, 7.4% of thrombosis cases occurred during the remission of proteinuria. Renal vein thrombosis (33.3%) and pulmonary embolism (25.9%) were the most common types of thrombosis. Among the 17 subjects biopsied, minimal change disease and membranous nephropathy were the two most common findings. Six (22.2%) subjects experienced severe complications or sequelae; 1 had persistent intracranial hypertension, 1 had intestinal perforation, 1 had hypoxemia and pulmonary hypertension, 1 had lameness, 1 had epilepsy, and 1 had an askew mouth due to facial paralysis. In 19 (70.4%) subjects, the symptoms resolved completely or improved without severe complications or sequelae. Conclusions Thrombosis mostly occurred in males of school age during the active stage of NS. Renal vein thrombosis and pulmonary embolism were the most common types of thrombosis. In most patients with thrombosis, the symptoms improved completely without complications with standard anticoagulation therapy. However, 22.2% had severe complications or sequelae requiring an advanced diagnostic modality and aggressive treatment.
Abstract Background Both Pierson syndrome (PS) and isolated nephrotic syndrome can be caused by LAMB2 biallelic pathogenic variants. Only 15 causative splicing variants in the LAMB2 gene have been reported. However, the pathogenicity of most of these variants has not been verified, which may lead to incorrect interpretation of the functional consequence of these variants. Methods Using high‐throughput DNA sequencing and Sanger sequencing, we detected variants in a female with clinically suspected PS. A minigene splicing assay was performed to assess the effect of LAMB2 intron 20 c.2885‐9C>A on RNA splicing. We also performed the immunohistochemical analysis of laminin beta‐2 in kidney tissues. Results Two novel LAMB2 heteroallelic variants were found: a paternally inherited variant c.2885‐9C>A in intron 20 and a maternally inherited variant c. 3658C>T (p. (Gln1220Ter)). In vitro minigene assay showed that the variant c.2885‐9C>A caused erroneous integration of a 7 bp sequence into intron 20. Immunohistochemical analysis revealed the absence of glomerular expression of laminin beta‐2, the protein encoded by LAMB2 . Conclusion We demonstrated the impact of a novel LAMB2 intronic variant on RNA splicing using the minigene assay firstly. Our results extend the mutational spectrum of LAMB2 .
Objective: To assess the reliability of estimated urine protein to predict 24 h urine protein excretion in children with glomerular diseases. Methods: Four hundred and forty-three children with glomerular diseases, who were admitted to pediatric department of Peking University First Hospital from January 2001 to December 2021, were enrolled in the cross-sectional study. The 24 h estimated urine creatinine which calculated by 6 previously described equations, 24 h measured urine creatinine, measured urine protein-to-creatinine ratio(UPCR), 24 h urine protein (24 hUP) and urinary sediment analysis with microscopy were collected, estimated urine protein was computed as the product of measured UPCR and estimated or measured 24 h urine creatinine. Spearman correlation analysis, Bland-Altman analysis and linear regression analysis were used to compare the correlation, agreement and accuracy between estimated urine protein and 24 hUP, and the effect of urinary protein level and erythrocyte numbers on their relationship was analyzed. Results: Of 443 children with glomerular diseases (aged (11±4) years, 221 male, 222 female), there were 216 participants with nephrotic syndrome, 78 participants with IgA nephropathy, 47 participants with Alport syndrome, 42 participants with lupus nephritis, 58 participants with purpura nephropathy, and 2 participants with isolated proteinuria. Spearman correlation analysis showed a strong correlation between estimated urine protein and 24 hUP (r=0.90, P<0.05), and the correlation improved after multiplying the measured UPCR by 24 h measured urine creatinine (r=0.94, P<0.05). Improved correlation was also observed using the estimated urine creatinine which calculated by Hellerstein formula, Ghazali-Barratt formula, Ellam formula, Walser formula, Cockcroft-Gault formula, Ix formula (r=0.93, 0.94, 0.90, 0.90, 0.94, 0.93, all P<0.05).Bland-altman analysis showed that the difference between measured UPCR and 24 hUP was (-0.30±2.22) g, consistency limit was -4.65-4.04, and the consistency improved after 24 h measured urine creatinine correction (difference was (0.27±1.31) g, consistency limit -2.30-2.84). The consistency of estimated urine protein was further improved after correction by different formulas, and the Cockcroft-Gault formula showed the best consistency between estimated urine protein and 24 hUP (difference was (0.11±1.18)g, consistency limit was -2.20-2.42). Linear regression analysis showed that measured UPCR had poor accuracy in predicting 24 hUP (R2=0.55, α=0.48, β=0.60, P<0.05), and the accuracy improved after 24 h measured urine creatinine correction, the accuracy of estimated urine protein for predicting 24 hUP was further improved by using different formulas, and Cockcroft-Gault formula was the best (R2=0.81, α=0.18, β=0.96, P<0.05). With the increase of urinary protein level and the decrease of urinary erythrocyte numbers, the correlation, agreement and accuracy between estimated urine protein and measured UPCR and 24 hUP were improved(all P<0.05). Except Ellam and Ix formulas, estimated urine protein using the rest four formulas outperformed measured UPCR(all P<0.05). Conclusion: The 24 h urine creatinine excretion rate (obtained by the Cockcroft-Gault equation)-weighted urine protein-to-creatinine ratio more reliably predicts 24 hUP than measured UPCR alone in children with glomerular diseases.目的： 评价不同公式估算尿蛋白反映儿童肾小球疾病24 h尿蛋白定量的可靠性。 方法： 横断面研究，收集2001年1月至2021年12月就诊于北京大学第一医院儿科的443例肾小球疾病患儿实测24 h尿肌酐和通过6个相关公式计算得出的估算24 h尿肌酐、实测24 h尿蛋白定量、实测尿蛋白肌酐比（UPCR）、尿沉渣镜检结果，用实测24 h尿肌酐或估算24 h尿肌酐乘实测UPCR计算得到估算尿蛋白。采用Spearman相关分析、Bland-Altman分析法及线性回归分析比较估算尿蛋白和实测UPCR与24 h尿蛋白定量间的相关性、一致性和准确性，并分析尿蛋白水平和尿红细胞数目对于二者关系的影响。 结果： 443例患儿中男221例、女222例，年龄（11±4）岁，其中肾病综合征216例、IgA肾病78例、Alport综合征47例、系统性红斑狼疮42例、紫癜性肾炎58例以及孤立性蛋白尿2例。Spearman相关分析提示实测UPCR与24 h尿蛋白定量具有相关性（r=0.90，P<0.05），应用实测24 h 尿肌酐得到的估算尿蛋白和24 h尿蛋白定量相关性得到改善（r=0.94，P<0.05），应用Hellerstein、Ghazali-Barratt、Ellam、Walser、Cockcroft-Gault、Ix公式估算的尿蛋白与24 h尿蛋白定量的相关性均加强（r值分别为0.93、0.94、0.90、0.90、0.94、0.93，均P<0.05）；Bland-Altman分析法示实测UPCR与24 h尿蛋白定量的差值为-0.30±2.22，一致性界限为-4.65~4.04，应用实测24 h尿肌酐校正后一致性好转［差值为（0.27±1.31）g，一致性界限为-2.30~2.84 g］，不同公式估算尿蛋白一致性进一步好转，其中Cockcroft-Gault公式估算尿蛋白与24 h尿蛋白定量一致性最好［差值为（0.11±1.18）g，一致性界限为-2.20~2.42 g］。线性回归分析示实测UPCR预测24 h尿蛋白定量准确性欠佳（R2=0.55、α=0.48、β=0.60，P<0.05），应用实测24 h尿肌酐校正后准确性好转，应用不同公式估算尿蛋白预测24 h尿蛋白定量准确性进一步加强，其中Cockcroft-Gault公式准确性最佳（R2=0.81、α=0.18、β=0.96，P<0.05）。随尿蛋白定量增加和尿红细胞数目减少，估算尿蛋白和实测UPCR与24 h尿蛋白定量间相关性、一致性和准确性均增加（均P<0.05）；除Ellam和Ix公式外，其余4个公式计算的及实测24 h尿肌酐得到的估算尿蛋白与24 h尿蛋白定量间相关性、一致性和准确性均高于实测UPCR（均P<0.05）。 结论： 基于Cockcroft-Gault公式计算的24 h尿肌酐得到的估算尿蛋白能更准确地评估儿童肾小球疾病24 h尿蛋白水平。.
Abstract Background To characterize the phenotypic spectrum and assess the antialbuminuric response to angiotensin converting enzyme (ACE) inhibitor and/or angiotensin receptor blocker (ARB) therapy in a cohort of children with Dent disease. Methods The patients’ clinical findings, renal biopsy results, genetic and follow‐up data were analyzed retrospectively. Mutations in CLCN5 or OCRL were detected by next‐generation sequencing or Sanger sequencing. Results Of 31 Dent disease boys, 24 carried CLCN5 and 7 carried OCRL mutations. Low molecular weight proteinuria and albuminuria were detected in all cases. Nephrotic‐range proteinuria and severe albuminuria were identified in 52% and 62% of cases, respectively; by 7 years of age, 6 patients had hematuria and nephrotic‐range proteinuria, and 7 patients had hematuria and moderate to severe albuminuria. In addition to disease‐related renal features, patients with Dent‐1 disease also presented with congenital cataract (1/9) and developmental delay (2/7). Seventeen of 31 patients underwent renal biopsy. Glomerular changes included mild glomerular lesions, mesangial proliferative glomerulonephritis and focal segmental glomerular sclerosis. Thirteen of the 31 patients had follow‐up records and received ACE inhibitor and/or ARB treatment for more than 3 months. After a median 1.7 (range 0.3–8.5) years of treatment, a reduction in the urinary microalbumin‐to‐creatinine ratio was observed in 54% of children. Conclusions Hematuria with nephrotic‐range proteinuria or moderate to severe albuminuria was common in Dent disease patients. Extrarenal manifestations were observed in Dent‐1 patients, which extends the phenotypic spectrum. In addition, ACE inhibitors and ARBs are well tolerated, and they are partially effective in controlling albuminuria.
Objective: To assess the correlation of glomerular C1q or IgA deposition with clinical and pathological features of primary membranous nephropathy (PMN) in children. Methods: The clinical and pathological manifestations including (phospholipase A2 receptor, PLA2R) and IgG subclasses staining in renal biopsies, serum anti-PLA2R antibody and therapeutic response of 33 children diagnosed with PMN in Peking University First Hospital from December 2012 to December 2020 were retrospectively summarized and analyzed. According to results of PLA2R test and findings renal pathological, the patients were divided into PLA2R-related group and non-PLA2R-related group, typical MN group and atypical MN group, C1q deposit group and non-C1q deposit group, as well as IgA deposit group and non-IgA deposit group respectively. T-test, Mann-Whitney U test and Fisher's exact probability test were used for comparison between the groups. Results: Among the 33 children with PMN, there were 20 males and 13 females, of that the age of onset was 11 (8, 13) years, and 32 patients had nephrotic level proteinuria. Renal biopsies were performed at 4.6 (2.1, 11.6) months after onset, and 28 patients (85%) received glucocorticoid or immunosuppressive therapy prior to renal biopsy. There were 20 cases (61%) with PLA2R-related MN and 13 cases (39%) with non-PLA2R-related MN. Compared with the non-PLA2R-related group, the PLA2R-related group had an older age of onset (12 (10, 13) vs. 7 (3, 12) years, Z=-2.52, P=0.011), a lower preceding infection rate (45% (9/20) vs. 11/13, P=0.032) and lower spontaneous remission rate (0 vs. 4/13, P=0.017). Renal PLA2R positivity was significantly associated with predominant or co-deposition of IgG4 (13/17 vs. 5/15, P=0.031) and low albumin levels at renal biopsy ((25±6) vs. (29±7) g/L, t=2.14, P=0.041). There were 12 patients with typical PMN and 21 patients with atypical PMN, and no significant difference in clinical and pathological manifestations was found between these 2 groups (all P>0.05). There were 10 cases (32.3%) with glomerular C1q deposition, and their disease course before renal biopsy was significantly shorter than those without C1q deposition (1.8 (0.8, 5.9) vs. 6.0 (2.5, 22.3) months, Z=-2.27, P=0.023). Twelve cases (36.4%) had glomerular IgA deposition, and their course of disease,clinical and pathological manifestations were not significantly different from those without IgA deposition (all P>0.05). Conclusion: Glomerular C1q or IgA deposition may not affect the clinical manifestations, glomerular PLA2R and IgG subclasses staining pattern, or the response to treatment of PMN in children.目的： 探讨儿童原发膜性肾病（PMN）肾小球C1q或IgA沉积与临床和病理表现的关系。 方法： 回顾性总结并分析2012年12月至2020年12月北京大学第一医院儿科诊断的33例PMN患儿临床及病理表现［肾组织磷脂酶A2受体（PLA2R）抗原、IgG亚型检测］、血清抗PLA2R抗体及治疗反应等资料。分别根据PLA2R检测结果、肾脏病理表现、是否有C1q沉积、是否有IgA沉积分别分为PLA2R相关组和非PLA2R相关组、典型PMN组和不典型PMN组、C1q沉积组和无C1q沉积组、IgA沉积组和无IgA沉积组。组间比较采用t检验、Mann-Whitney U检验或Fisher确切概率法。 结果： 33例PMN患儿中男20例、女13例，起病年龄11（8，13）岁，临床表现为肾病水平蛋白尿者32例。病程4.6（2.1，11.6）个月时行肾活检，28例（85%）在肾活检前接受糖皮质激素和（或）免疫抑制剂治疗。PLA2R相关组20例（61%），非PLA2R相关组13例（39%）。与非PLA2R相关组相比，PLA2R相关组发病年龄偏大［12（10，13）比7（3，12）岁，Z=-2.52，P=0.011］、前驱感染率低［45%（9/20）比11/13，P=0.032］、自发缓解率低（0 比 4/13，P=0.017）。肾组织PLA2R抗原阳性与肾脏IgG4沉积为主或共沉积（13/17比5/15）以及肾活检时低白蛋白水平［（25±6）比（29±7）g/L，t=2.14］均显著相关（P=0.031、0.041）。肾脏病理表现为典型PMN者12例、不典型PMN者21例，两组间临床及肾脏病理表现差异均无统计学意义（均P>0.05）。肾小球C1q沉积者10例（32%），其肾活检前病程短于无C1q沉积者［1.8（0.8，5.9）比6.0（2.5，22.3）个月，Z=-2.27，P=0.023］；肾小球IgA沉积者12例（36%），其肾活检前病程、临床和病理表现与无IgA沉积者间差异均无统计学意义（均P>0.05）。 结论： 伴或不伴肾小球 C1q或IgA沉积不影响PMN患儿的临床表现、肾组织PLA2R和IgG亚型分布以及治疗反应。.
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