Objective:To investigate the clinicopathological features and the prognosis of IgA nephropathy (IgAN) in children with massive proteinuria.Methods:It was a retrospective cohort study. Clinical data of IgAN children with massive proteinuria admitted to the Department of Nephrology, Children's Hospital Affiliated to Capital Institute of Pediatrics from January 2008 to December 2021 were retrospectively analyzed. Patients were divided into effective group and ineffective group according to whether urine protein turned negative after 6 months of initial treatment. The follow-up endpoint event was defined as a reduction in proteinuria of less than 50% or end-stage renal disease (ESRD) achievement. MedCalc software was used to perform Kaplan-Meier survival analysis, and Log-rank test was used to compare the difference of renal survival between the two groups.Results:A total of 127 patients were diagnosed as primary IgAN by renal biopsy, of whom 57 patients with IgAN showed massive proteinuria. These 57 IgAN patients with macroproteinuria accounted for 44.9% of the total IgAN patients and were enrolled in the study. Among the 57 cases, 33 cases (57.9%) were Lee's grade Ⅲ, 11 cases (19.3%) were below Lee's grade Ⅲ, and 13 cases (22.8%) were above Lee's grade Ⅲ. The follow-up time was 4.0 (3.0,5.8) years. In the initial treatment, among 57 patients, 46 (80.7%) were effective (effective group) and 11 (19.3%) were ineffective (ineffective group). Compared with the effective group, the ineffective group had a higher proportion of concurrent AKI at the onset of disease and longer recovery time of renal function, with significant difference (7/11 vs. 13/46, χ2=4.878, P=0.027). Compared with the effective group, the proportion of Lee grade Ⅲ or above was higher in the ineffective group, and the difference was statistically significant (5/11 vs. 8/46, χ2=3.971, P=0.046). There were significant differences in endocapillary hypercellularity (E1), segmental glomerulosclerosis or adhesion (S1) and cellular/fibrocellular crescents (C2) of Oxford classification between IgAN children with Lee grade Ⅲ or below and those over Lee grade Ⅲ (11/13 vs. 20/44, χ2=6.204, P=0.013; 12/13 vs. 17/44, χ2=11.566, P=0.001; 9/13 vs. 7/44, χ2=14.131, P=0.001). Among 57 patients, endpoint events occurred in 2 patients who both were urinary protein unmitigated, and none of the children progressed to ESRD. There was no significant difference in cumulative renal survival between the two groups by Kaplan-Meier survival analysis and Log-rank test ( χ2=0.537, P=0.460) after addition of calcineurin inhibitors (CNIs) to the initial treatment ineffective group. Conclusions:Macroproteinuria is the prominent manifestation of IgAN in children. The pathological type is mainly Lee grade Ⅲ. Children with macroproteinuria have a good prognosis in the short and medium term after active treatment. For IgAN with macroproteinuria that does not respond well to initial treatment, AKI is more common at onset, and renal function recovery time is longer. The application of CNIs may have a certain effect on improving the renal outcome of IgAN with massive proteinuria.

Objective:To summarize and analyze the clinical features and risk factors of acute focal bacterial nephritis (AFBN) in children.Methods:It was a retrospective cohort study. The clinical data of patients diagnosed with upper urinary tract infection in Children's Hospital Affiliated to Capital Institute of Pediatrics from July 1, 2016 to July 31, 2021 were collected, and the patients all received abdominal enhanced CT examination. According to the imaging examination results, the patients were divided into AFBN group and acute pyelonephritis (APN) group, and the clinical manifestations, laboratory and imaging examination between the two groups were compared. Logistic regression model and receiver operating characteristic curve were used to analyze the risk factors of AFBN.Results:A total of 135 patients with upper urinary tract infection were enrolled in this study, with age of 2.5 (0.5, 3.7) years old, and 68 males (50.4%). There were 67 patients (49.6%) in AFBN group and 68 patients (50.4%) in APN group. There were statistically significant differences in the highest fever temperature, duration of fever after treatment, proportion of lower urinary tract irritation symptoms, proportion of urinary tract malformation or abnormality, white blood cell count, neutrophil count, procalcitonin, C-reactive protein, proportion of pyuria, urinary β2 microglobulin and proportion of using carbapenem antibiotics between the two groups (all P<0.05). Multivariate logistic regression analysis result showed that urinary tract malformation/abnormality ( OR=3.34, 95% CI 1.23-9.10) and leukocytosis ( OR=1.25, 95% CI 1.03-1.51) were the independent risk factors of AFBN. Conclusions:The children with urinary tract infection who have high peak fever, long duration, obvious increase of inflammatory indexes and urinary β2 microglobulin may suggest AFBN. Urinary tract malformation/abnormality and high white blood cells are risk factors of AFBN.

Objective:To explore the related factors of poor prognostis in children with Henoch-Sch?nlein purpura nephritis (HSPN), and provide reference for predicting and improving the prognosis of children with HSPN.Methods:The clinical and pathological data of children with HSPN hospitalized in the Department of Nephrology, Children's Hospital Affiliated to Capital Institute of Pediatrics from May 2007 to June 2019 were retrospectively reviewed. According to the prognosis, the patients were divided into complete remission group and persistent abnormal group.Results:(1) Among 108 cases, there were 73 males and 35 females, with the onset age ranging from 5 to 16 years and average age of (9.5±2.8) years. The interval time from the first clinic in our hospital to the last follow-up was 2-131 months, with average of 24.8 months. Renal involvement occurred in the course of Henoch-Sch?nlein purpura from 1 day to 51 months, and the renal biopsy time was 5 days to 60 months after renal involvement. (2) Hematuria with proteinuria type and nephrotic syndrome type were predominant, and there was no significant difference between the two groups. The proportion of gross hematuria in the persistent abnormal group were significantly higher than that in the complete remission group (52.6% vs 31.4%, χ2=4.659, P=0.031). There were significant differences in serum creatinine and urea between the two groups (both P<0.05). The proportion of hyperuricemia in the persistent abnormal group was higher than that in the complete remission group (39.5% vs 21.4%, χ2=3.998, P=0.046). After clinical treatment, though there was no significant difference in proteinuria between the two groups at the beginning of the disease, the negative transformation rate of proteinuria in the complete remission group was higher than that in the persistent abnormal group after 3 months (55.7% vs 34.2%, χ2=4.562, P=0.033). (3) According to International study of Kidney Disease in Children (ISKDC) pathology classification, 14 cases (36.8%), 21 cases (55.3%), 3 cases (7.9%) withⅡ, Ⅲ, Ⅳ level in the persistent abnormal group and 21 cases (30.0%), 49 cases (70.0%), 0 case with Ⅱ, Ⅲ, Ⅳ level (70.0%) in the complete remission group after (20.16±24.86) months of follow-up, and the difference between the two groups was not statisticcally significant ( Z=-0.135, P=0.892). According to the Oxford Classification of IgA nephropathy, 36(33.3%) children had tubule-interstitial lesions (T1, 26%-50% tubular atrophy or interstitial fibrosis), and the proportion in the persistent abnormal group was significantly higher than that in the complete remission group (50.0% vs 24.3%, Z=-2.695, P=0.007). (4) Compared with T0 (0-25% tubular atrophy or interstitial fibrosis), the incidence of gross hematuria and hyperuricemia in the T1 tubule-interstitial lesion were both higher than that (respectively 63.9% vs 27.8%, χ2=13.061, P<0.001; 38.9% vs 22.2%, χ2=3.983, P=0.046). (5) Multivariate logistic regression analysis showed that renal tubule-interstitial lesion was a risk factor for poor prognosis of HSPN ( OR=2.580, 95% CI 1.055-6.310, P=0.038). Conclusions:Renal tubule-interstitial lesion is a risk factor for the persistent abnormal of HSPN. Gross hematuria and hyperuricemia are related to tubule-interstitial lesions.

Objective: To analyze the relationship of clinical manifestations and pathological characteristics of Henoch-Schönlein purpura nephritis combined with hyperuricemia in children. Methods: A retrospective study was conducted in 50 children with Henoch-Schönlein purpura nephritis who hospitalized at Department of Nephrology, Affiliated Children′s Hospital, Capital Institute of Pediatrics from January 2014 to May 2018.The differences between the hyperuricemia group(19 cases)and the normal uric acid group(31 cases), were compared in age, sex, blood pressure, serum albumin, 24-hour urinary protein, serum creatinine, triglyceride, cholesterol, high density lipoprotein, low density lipoprotein, serum uric acid, estimated glomerular filtration rate, and renal pathological characteristics, and the short-term prognosis was analyzed. Results: (1)The average urinary protein in the hyperuricemia group and the normal uric acid group was (91.67±90.37) mg/(kg·d) and (64.62±43.28) mg/(kg·d), respectively and the difference was statistically significant between the both groups(t=2.04, P=0.047); and the morbidity with massive proteinuria in hyperuricemia group and normal uric acid group was 18/19 cases(94.7%)and 17/31 cases(54.8%), respectively and the difference was statistically significant between the both groups(χ²=8.930, P=0.003). (2)In all cases, there were 4 cases of glomerular pathological grade Ⅱ, 43 cases of grade Ⅲ and 3 cases of grade Ⅳ.The pathological grading of hyperuricemia group and normal uric acid group was mainly grade Ⅲ, including 16/19 cases (84.2%) in hyperuricemia group and 27/31 cases (87.1%) in normal uric acid group, 4 cases of grade Ⅱ in normal uric acid group and 3 cases of grade Ⅳ in hyperuricemia group, the pathological grade of hyperuricemia group was relatively severe (χ²=7.358, P=0.025). There was no significant difference about the degree of global sclerosis and mesangial proliferation between hyperuricemia group and normal uric acid group(χ²=2.426, P=0.119, χ²=0.043, P=0.836, respectively); 7/19 cases (36.8%) had severe foot process lesions in hyperuricemia group, which was significantly higher than that in normal uric acid group [4/31 cases(12.9%)](χ²=3.934, P=0.047). In hyperuricemia group, tubulointerstitial lesions were found in 9/19 cases (47.4%) of (+ ) grade and 10/19 cases (52.6%) of (+ + ) grade, and 12/31 cases (38.7%) had normal tubulointerstitium in normal uric acid group, (+ )and (+ + )grade lesions were also less than those in the hyperuricemia group(χ²=10.694, P=0.005). The mean scores of tubular atrophy and interstitial fibrosis were significantly higher in hyperuricemia group than that in normal uric acid group(t=2.36, P=0.001). (3) The interval from renal biopsy to final visit was 10.0 months and 10.5 monthsin hyperuricemia group and normal uric acid group respectively (P=0.85). In hyperuricemia group, complete remission was found in 5/19 cases (26.3%), slight abnormality in 10/19 cases (52.6%), severe abnormality in 4/19 cases (21.1%). Howe-ver, in normal uric acid group, complete remission was found in 19/31 cases (61.3%), 10/31 cases (32.3%) of slight abnormalities and 2/31 cases (6.5%)of severe abnormalities.The non-remission cases in the hyperuricemia group were significantly higher than those in the normal uric acid group(χ²=7.878, P=0.042). Conclusions Urinary protein was higher in children with Henoch-Schönlein purpura nephritis complicated with hyperuricemia, the pathological of renal tubulointerstitium and glomerulus and the foot process change are more serious than those of patients with normal uric acid.Therefore, hyperuricemia may be used as a risk factor for poor prognosis.

Objective To explore the activation of renin-angiotensin system (RAS),efficiency and safety of Captopril,and the predictor of therapeutic activity for Henoch-Sch(o)nlein purpura nephritis (HSPN) characterized by mild proteinuria.Methods A total of 71 children who were hospitalized in Children's Hospital Affiliated to Capital Institute of Pediatrics from July 2014 to January 2017 were involved,with the diagnosis of HSPN and the characteristic of mild proteinuria.The cases were divided into 2 groups,one as Captopril group,the other as case control group.The patients were followed up for 6 months.Forty healthy children were assigned as healthy control group.Blood pressure,urinary protein excretion,levels of urinary angiotensinogen (AGT) and transforming growth factor β1 (TGF-β1),and the side effects of Captopril were surveyed.The therapeutic effects of these groups were analyzed by Kaplan-Meier survival curve.Results (1) Clinical characteristics:in the 71 cases,43 cases were male,28 cases were female,aged from 3 years to 14 years and 7 months.A total of 32 cases (45.1％) had manifested with isolated proteinuria,39 cases (54.9％) were with hematuria and proteinuria.The volume of 24 hours' urinary protein was 4.2-23.5 mg/(m2 · h) [median 9.6(7,12) mg/(m2 · h)] at the beginning.(2) The level of urinary AGT:the levels of urinary AGT in the children with HSPN were significantly higher than those of the healthy control group(Z =-3.010,P =0.003).(3) Curative effect:there was no significant difference in age,disease staging,mean arterial pressure(MAP),levels of urinary of proteinuria and estimated glomerular filtration rate (eGFR) between the patients with or without Captopril.The proteinuria was relieved in 88.57％ cases of Captopril group(35 cases),and the proportion was 80.55％ in the case control group(36 cases),and there was no significant difference between the 2 groups.The levels of proteinuria were decreased significantly in the children of Captopfil group 2 months after the enrollment,and there was a statistical significance (Z =2.010,P =0.044).But in the patients of each group,the levels of urinary protein excretion (Z =-2.127,P=0.030;Z=-2.639,P=0.010),TGF-β1(Z=-2.126,P=0.030;Z=-2.058,P=0.040) at theonset were significantly higher in the children with persistent proteinuria compared to those with remission of proteinuria completely,and there was a statistical significance.(4)Side effect:among 35 cases with therapy of Captopril,4 cases (11.42％) were verified to have adverse reaction (hypotension,dry cough and abnormal renal function),with mild symptom.Conclusion The overall prognosis of children of HSPN presenting as mild proteinuria are not improved completely by Captopril.The occurrence of adverse effects for Captopril is seldom and less severe.The level of urinary protein excretion,TGF-31 and AGT at the onset have some relevance with the prognosis of the patients of HSPN.

Objective: To compare the efficacy and safety of mycophenolate mofetil versus cyclosporine A in treating children with primary refractory nephrotic syndrome. Methods: Conducted a prospective randomized controlled clinical trial in 62 pediatric patients (including 44 boys and 18 girls), age ranged from 2.1 to 17.0 years; 32 cases presented with frequently relapsing nephrotic syndrome (FRNS) and 30 cases presented with steroid-resistant nephrotic syndrome (SRNS), who were admitted to department of Nephrology, Children's Hospital Affiliated to Capital Institute of Pediatrics from October 2013 to October 2015. The patients received either mycophenolate mofetil (20-30)mg/(kg·d) or cyclosporine A (3-5)mg/(kg·d) randomly, on the basis of prednisone treatment. Follow-up interview was conducted regularly for at least one year. Efficacy rate, relapse rate, time required for induction of remission, relapse-free period and prednisone dosage were compared between the two groups. Results: (1) Renal histologic examination, which was available for 17 patients, revealed minimal change disease in 8 patients, mesangial proliferative glomerulonephritis (MsPGN) in five, membranous nephropathy in two, and focal segmental glomerulosclerosis (FSGS) in two. (2) Comparison of mycophenolate mofetil versus cyclosporine A in children with FRNS: There were 14 patients with FRNS in mycophenolate mofetil group and 18 patients with FRNS in cyclosporine A group respectively. The relapse rate (episodes/year) in cyclosporine A group was lower than that of mycophenolate mofetil group (1.0 (0.0, 1.0) vs. 1.0 (1.0, 3.0), Z=-2.405, P=0.016). The relapse-free period (months) in cyclosporine A group was longer than that of mycophenolate mofetil group (10.0 (5.7, 12.1) vs. 5.0 (1.0, 11.0), Z=-1.984, P=0.047). No significant difference in dosage of prednisone was found between cyclosporine A and mycophenolate mofetil groups when followed up for 1 year. (3) Comparison of mycophenolate mofetil versus cyclosporine A in children with SRNS: The efficacy rate was 6/14 in mycophenolate mofetil group and 13/16 in cyclosporine A group. The complete remission rate was 4/14 in mycophenolate mofetil group and 12/16 in cyclosporine A group (P<0.05). The time (months) required for induction of remission in cyclosporine A group was significantly shorter than that of mycophenolate mofetil group (1.0 (1.0, 2.0) vs. 3.0 (2.5, 4.0), Z=-2.529, P=0.011). No significant differences were found between the two groups with respect to relapse-free period and relapse rate. (4) Except that one patient developed hypertensive encephalopathy in cyclosporine A group, no other serious adverse events were recorded. There were no significant differences between two groups with respect to adverse events. Conclusion Our results indicated that both mycophenolate mofetil and cyclosporine A were effective in the treatment of children with refractory nephrotic syndrome. Cyclosporine A was superior to mycophenolate mofetil in preventing relapses in patients with FRNS and inducing complete remission in patients with SRNS. Although most patients were able to tolerate mycophenolate mofetil and cyclosporine A, but the toxicity and safety of cyclosporine A should be monitored closely.

Objective To analyze the pathogenesis,initially diagnosed symptoms and clinical manifestations of children with chronic kidney disease (CKD) at stage 2 to 5.Methods The data of 108 children who were hospitalized in Children's Hospital Affiliated to Capital Institute of Pediatrics from September 2007 to April 2016 with CKD stage 2 to 5 were retrospectively analyzed.The etiologies,clinical manifestations and examinations were summarized,and the clinical manifestations were compared between the congenital hereditary urinary diseases group and the acquired urinary diseases group.Results (1) In the 108 cases collected,66 cases were male,42 cases were female,aged from 3 months to 15 years and 1 month old.Twenty-four cases were diagnosed at stage 2,26 cases at stage 3,35 cases at stage 4,and 23 cases at stage 5.(2) Twenty-eight kinds of illness were involved in the cause of CKD.Among them,57 cases (52.8％) had congenital anomalies of the kidney and urinary tract,5 cases(4.6％) had hereditary kidney diseases,41 cases (38.0％) had other primary or secondary kidney diseases,and in 5 cases (4.6％) the causes of disease were unknown.(3) For the initially diagnosed symptoms,29 cases(26.9％) were due to complaints associated with kidney disease,36 cases (33.3％) were of other outside kidney symptoms,and 43 cases (39.8 ％) were of negative symptoms.The results of urinary ultrasound were abnormal in 79 cases(73.1％) and 87 cases(80.6％) showed abnormality in urinary analysis.There were 105 cases (97.2％) with abnormal manifestations either in urinary tract ultrasound or in urinary analysis.(4)The ages on diagnosis as CKD in children with congenital hereditary urinary diseases(5.89 years old) were younger than that of children with acquired urinary diseases (9.20 years old),and the difference was significant(Z =-3.434,P =0.001).The frequency of cases with short stature or lower-weight in group of congenital hereditary urinary diseases[66.1％ (41/622 cases),64.5％ (40/62 cases)] were significantly higher than those of the acquired urinary diseases group[43.9％ (18/41cases),43.9％ (18/41 cases)],and the differences were statistically significant(x2 =4.983,4.263,P =0.026,0.039).Conclusions The causes of CKD are complicated,and the congenital anomalies of kidney and urinary tract are the major causes of CKD at stage 2 to 5 in the cases.The initially diagnosed symptoms of CKD are insidious and atypical.The children with congenital hereditary urinary diseases tend to have more serious growth retardation.Urinary analysis and ultrasound may have an important significance for early diagnosis of CKD in children.

Objective To investigate the prevalence,missed diagnosis rate and causes of acute kidney injury (AKI) in hospitalized children,and its impact on hospitalization cost,length of stay and outcome.Methods The data of children admitted in Children's Hospital Affiliated to Capital Institute of Pediatrics from December 1st to 31st 2014 were collected,and those whose serum creatinine (Scr) were measured at least two times were selected.Patients were diagnosed as AKI according to the diagnostic criteria of 2012 Kidney Disease:Improving Global Outcomes,then divided into AKI group and non-AKI group,the former of which was further divided into AKI1 group (Scr peak value in normal range) and AKI2 group (Scr peak value above normal range).The causes and impact of AKI on hospitalization cost,length of stay and outcome in different groups were compared and analyzed.Results (1) Among 921 patients with at least two Scr results,170 patients met with the diagnostic criteria of AKI,including 100 males and 70 females.There were 112(65.9％) in AKI stage 1,43(25.3％) in stage 2,and 15(8.8％) in stage 3.The overall prevalence of AKI was 18.5％.With only 7cases getting diagnosed,the diagnostic rate was 4.1％,while 95.9％ of patients missed diagnosis.(2)Among AKI patients,67 cases had pre-renal causes,103 cases had intra-renal causes and mixed factors.100(58.8％) cases got complete recovery,34(20.0％) cases recovered partially and 36(21.2％)cases did not improve,including 4 cases of death.(3) The prevalence of AKI among those below 1-year old was higher than children elder than 1-year (23.0％ vs 15.5％,P=0.004).The prevalence of AKI in surgical ward was higher than medical ward (30.7％ vs 15.8％,P ＜ 0.001).(4) Compared with those in non-AKI group,there was lower age [1.1(0.2,3.5) year vs 2.0(0.3,4.9) year] and higher hospitalization time[12.5(8.0,20.0) d vs 8.0(6.0,11.0) d],hospitalization costs [25 279.2(13 822.8,48 856.7) yuan vs 12 616.9(8680.1,19 345.1) yuan] and mortality (2.4％ vs 0.3％) in AKI group (all P ＜ 0.05).(5) There were 126 cases in AKL group and 44 cases in AKI2 group.The costs of hospitalization,outcome and mortality showed no difference between two groups (all P ＞ 0.05).The hospitalization time in AKI2 group was shorter than that in AKL group (P=0.038).Conclusions Among hospitalized children the missed diagnosis rate of AKI is high.Pre-renal factor is the main cause of AKI.Children younger than 1-year old are more susceptible to AKI.AKI children have lower age and higher hospitalization time,hospitalization costs and mortality than non-AKI children.The effect of Scr fluctuation within normal levels needs to be further studied.

Objective To investigate the prognosis and efficiency of glucocorticoid and immunosuppressor in the treatment of idiopathic membranous nephropathy(IMN)in children. Methods A retrospective analysis of 35 cases of biopsy - proven membranous nephropathy without secondary factors was performed,who were found present with ne-phrotic proteinuria and admitted to hospital from March 2004 to July 2013,to explore the efficiency of treatment with glucocorticoid and immunosuppressor and its prognosis. Results The 35 IMN cases included 18 boys and 17 girls,and the ratio was 1. 1∶ 1. 0. The mean age at onset was(11. 3 ± 0. 5)years with a range of 3. 0 - 17. 1 years. Five cases with gross hematuria,24 cases present with microscopic hematuria,8 cases with hypertension,1 case with chronic renal insufficiency,and 2 cases were complicated with thrombosis. According to membranous nephropathy staging criteria,9 cases(25. 7% )were in stage Ⅰ,16 cases(45. 7% )in stage Ⅱ,10 cases(28. 6% )in stage Ⅲ;about 94. 3%(33 / 35 cases)had mesangial cells and mesangial matrix with mild to moderate hyperplasia. They were all treated with glucocor-ticoid initially and one of them showed sensitive to flucocorticoid but developed flucocorticoid resistance after relapse, while all the others were flucocorticoid - resistant. Cyclophosphamide A(CsA)was introduced to 17 cases and at least lasted for 3 months,in which 13 cases(76. 5% )reached complete remission and 3 cases reached partial remission, while 1 case didn't achieve remission,and the mean time for proteinuria to disappear was(4. 9 ± 3. 7)months;5 cases were treated with Mycophenolate mefetil( MMF),among which 4 cases reached complete remission in 2 months,4 months,5 months,and 9 months separately,while 1 case reached partial remission. Cyclophosphamide(CTX)was intro-duced to 6 cases,in which the mean cumulative dosage was(91. 2 ± 46. 5)mg/ kg,among them 1 case(87 mg/ kg) reached complete remission,1 case(160 mg/ kg)partial remission,but 4 cases didn't achieve remission. One case reached remission after Rituximab(RTX)was introduced. One case got partial remission after Leflunomide(LEF)was introduced,and the complete remission rate was higher in those treated with combined therapy of glucocorticoid and CsA than those treated with glucocorticoid only(76. 5% vs 12. 5% ,P = 0. 004),but the total efficacy showed no difference (94. 2% vs 62. 5% ,P = 0. 081). The complete remission rate(76. 5% vs 38. 5% ,P = 0. 042)and total efficacy (94. 1% vs 61. 5% ,P = 0. 040)were higher in those with combined therapy of steroid and CsA than those treated with steroid and other immunosuppressor. The complete remission rate(76. 5% vs 16. 7% ,P = 0. 018)and total efficacy (94. 1% vs 33. 3% ,P = 0. 008)were also higher than those treated with steroid and CTX,but the complete remission rate(76. 5% vs 80. 0% ,P = 0. 687)and total efficacy(94. 1% vs 100. 0% ,P = 0. 773)showed no difference com-pared with those treated with steroid and MMF. Conclusions IMN shows glucocorticoid resistance mostly,while CsA had definite efficiency and may be better than CTX. And the efficiency of MMF should be noted.

OBJECTIVETo evaluate clinical manifestations and pathology in children with massive proteinuria with Henoch-Schonlein purpura nephritis (HSPN).

METHODThe data of the 52 children with massive proteinuria with Henoch-Schonlein purpura nephritis who were hospitalized in the department of nephrology in our hospital from January 2008 to January 2013 were retrospectively studied. Clinical manifestation and pathologic characteristics were summarized and compared. The relationship between clinical manifestations and pathologic characteristics were evaluated.

RESULT(1) Among the children, 16 (31%) cases had positive symptoms and signs including gross hematuria, edema, oliguria and hypertension, and in only 7 (13%) cases serum levels of albumin were below 25 g/L. (2) Children with pathological grade >III accounted for 72%. Children with crescent formation, glomerular capsule adhesion, segmental glomeralosclerosis, endocapillary proliferation and lesions in the walls of arterioles accounted for 56%, 52%, 19%, 67%, 62%, respectively, and 42% of the children suffered from severe mesangial proliferation or mesangial sclerosis. The frequencies of severe mesangial proliferation and changes in the walls of arterioles in children with pathological grade ≥ III was significantly higher than those in children with pathological grade

CONCLUSIONThe clinical manifestations of children with massive proteinuria with HSPN were relatively severe but the symptoms were not typical. The pathological manifestations wer variable, of which severe mesangial proliferation and lesions in arterioles are the most common in children with higher pathological grade. Clinical manifestations in children with HSPN are associated with formation of crescent, segmental glomeralosclerosis, glomerular capsule adhesion, and endocapillary proliferation.