Objective: To summary the clinical presentation and prognosis of primary nephrotic syndrome (PNS) in teenagers. Methods: The clinical data, renal pathological types and prognosis of 118 children over 10-year-old with PNS treated in the Department of Nephrology of the Children's Hospital Affiliated to Capital Institute of Pediatrics from January 2010 to December 2020 were retrospectively analyzed, with 408 children ≤10-year-old as control group synchronously. Chi-square test was used to compare the difference of clinical types, pathologic types, response to steroids and tubulointerstitial changes between the groups. The teenagers with steroid resistant nephrotic syndrome (SRNS) were divided into initial non-responder group and late non-responder group. Kaplan-Meier method was used to compare the difference of persistent proteinuria, and Fisher's exact test for the histological types. Results: There were 118 children >10-year-old, including 74 males and 44 females, with the onset age of 12.1 (10.8, 13.4) years; and 408 children ≤10-year-old with the onset age of 4.5 (3.2, 6.8) years. The proportion of SRNS was significantly higher in patients >10-year-old than those ≤10-year-old (24.6% (29/118) vs. 15.9% (65/408), χ²=4.66, P=0.031). There was no statistical difference in the pathological types between >10-year-old and ≤10-year-old (P>0.05), with minimal change disease the most common type (56.0% (14/25) vs. 60.5% (26/43)). The percentage of cases with renal tubulointerstitial lesions was significantly higher in children >10-year-old compared to those ≤10-year-old (60.0% (15/25) vs. 23.3% (10/43), χ²=9.18, P=0.002). There were 29 cases presented with SRNS in PNS over 10-year-old, including 19 initial non-responders and 10 late non-responders. Analyzed by Kaplan-Meier curve, it was shown that the percentage of persistent proteinuria after 6 months of immunosuppressive treatments was significantly higher in initial non-responders than those of the late non-responders ((22±10)% vs. 0, χ²=14.68, P<0.001); the percentage of minimal change disease was significantly higher in patients of late non-responders than those of the initial non-responders (5/6 vs. 3/13, P=0.041). Of the 63 >10-year-old with steroid-sensitive nephrotic syndrome followed up more than one year, 38 cases (60.3%) had relapse, and 14 cases (22.2%) were frequent relapse nephrotic syndrome and steroid dependent nephrotic syndrome. Among the 45 patients followed up over 18-year-old, 22 cases (48.9%) had recurrent proteinuria continued to adulthood, 3 cases of SRNS progressed to kidney insufficiency, and one of them developed into end stage kidney disease and was administrated with hemodialysis. Conclusions: Cases over 10-year-old with PNS tend to present with SRNS and renal tubulointerstitial lesions. They have a favorable prognosis, but are liable to relapse in adulthood.
Male ; Female ; Adolescent ; Child ; Humans ; Nephrotic Syndrome/pathology* ; Retrospective Studies ; Nephrosis, Lipoid/drug therapy* ; Prognosis ; Proteinuria/etiology* ; Recurrence

Nephrotic syndrome (NS) is the most common glomerular disorder in childhood, and a vast majority of cases are idiopathic. The precise cause of this common childhood disease is not fully elucidated despite significant advancements in our understanding of podocyte biology. Idiopathic NS has been considered “a disorder of T-cell function” mediated by a circulating factor that alters podocyte function resulting in massive proteinuria since the last four decades. Several circulatory factors released from T-cells are considered to be involved in pathophysiology of NS; however, a single presumptive factor has not been defined yet. Extended evidence obtained by advances in the pathobiology of podocytes has implicated podocytes as critical regulator of glomerular protein filtration and podocytopathy. The candidate molecules as pathological mediators of steroid-dependent NS are CD80 (also known as B7-1), hemopexin, and angiopoietin-like 4. The “two-hit” hypothesis proposes that the expression of CD80 on podocytes and ineffective inhibition of podocyte CD80 due to regulatory T-cell dysfunction or impaired autoregulation by podocytes results in NS. Recent studies suggest that not only T cells but also other immune cells and podocytes are involved in the pathogenesis of MCNS.
Biology ; Filtration ; Hemopexin ; Homeostasis ; Nephrosis, Lipoid ; Nephrotic Syndrome ; Pathology ; Podocytes ; Proteinuria ; T-Lymphocytes

Humans ; Kidney Neoplasms ; diagnosis ; pathology ; Lymphoma ; diagnosis ; pathology ; Nephrotic Syndrome ; diagnosis ; pathology

Diabetic nephropathy is a chronic microvascular complication of type 2 diabetes and the leading cause of end-stage renal disease. We report the case of a 34-year-old male, newly diagnosed with type 2 diabetes mellitus, who had advanced-stage nephropathy with glomerular crescents. A moderately-to-severely decreased glomerular filtration rate with nephrotic syndrome was seen at the time of diagnosis of diabetes. Proliferative diabetic retinopathy was detected, but there was no positive finding in serology tests for glomerulonephritis. Non-necrotizing cellular crescents and nodular glomerulosclerosis were observed in a kidney biopsy, and renal function declined rapidly to the end stage. We review data on diabetic glomerulosclerosis with cellular crescents and the rapid progression of nephropathy.
Adult ; Biopsy ; Diabetes Mellitus, Type 2 ; Diabetic Nephropathies* ; Diabetic Retinopathy ; Diagnosis ; Disease Progression ; Glomerular Filtration Rate ; Glomerulonephritis ; Humans ; Kidney ; Kidney Failure, Chronic* ; Male ; Nephrotic Syndrome ; Pathology

REVERSIBLE posterior leukoencephalopathy syndrome (RPLS) is a rare neurological syndrome characterized by headache, altered mental status, seizures, and visual disturbance, associated with reversible white matter changes.1 It has been commonly reported in patients with severe hypertension and pre-eclampsia. Here we report a case with nephrotic syndrome complicated by RPLS.
Brain ; diagnostic imaging ; drug effects ; pathology ; Child ; Female ; Humans ; Magnetic Resonance Imaging ; Nephrotic Syndrome ; complications ; diagnosis ; drug therapy ; Posterior Leukoencephalopathy Syndrome ; complications ; diagnosis ; drug therapy ; Tomography, X-Ray Computed ; Treatment Outcome

Female ; Humans ; Infant ; Male ; Nephrotic Syndrome ; congenital ; pathology ; therapy

OBJECTIVETo study the characteristics of clinicopathology and prognosis of 3 pediatric cases diagnosed as C3 glomerulopathy, and to improve the understanding of C3 glomerulopathy in children.

METHODThe medical record, plasma complement C3, Factor H (FH) and its autoantibody, and therapeutic response of the 3 cases were analyzed, and their prognosis were followed up.

RESULTOf the 3 cases, 2 were male and 1 was female, the age of onset was 9 years, 12 years, 5 years 4 months, the duration from onset to renal biopsy was 3 months, 7 months and 20 days, and the follow-up period were 2.6 years, 8 months and 1.5 years respectively.

CLINICAL MANIFESTATIONSAll the 3 cases showed microscopic hematuria, with or without gross hematuria and proteinuria. Two showed persistently decreased plasma complement C3, in the other one C3 was in normal lower limit, all presented with decreased FH concertration, in 1 case anti-FH antibody was positive. Their clinical diagnosis was post-streptococcal glomerulonephritis, nephrotic syndrome (NS) nephritis type, and mesangial proliferative glomerulonephritis respectively.

PATHOLOGICAL FINDINGSAll showed evident deposition of C3 on glomerular basement membrance (GBM) and mesangial region by immunofluorescence (IF) and electron dense deposit in GBM, mesangial region or para-mesangial region by Electron microscopic (EM) examination Treatment and prognosis: The case with NS showed no response to steroid, so steroid was gradually stopped after renal biopsy and replaced by angiotensin converting enzyme inhibitors (ACEI) and angiotensin receptor antagonist (ARB). The other two cases were treated with ACEI and renal protective treatment. Of the 3 cases, one gradually showed elevated serum creatinine (Scr) and decreased creatinine clearance rate (Ccr), the other two were normal, but slightly increased indications for early kidney injury.

CONCLUSIONC3 glomerulopathy is characterized by evident C3 deposition under IF. Its clinical and pathological manifestations vary a lot. The decreased plasma C3 and FH suggest that the abnormal regulation of complement system play an importment role in its pathogenesis.

Angiotensin Receptor Antagonists ; therapeutic use ; Angiotensin-Converting Enzyme Inhibitors ; therapeutic use ; Child ; Child, Preschool ; Complement C3 ; metabolism ; Complement Factor H ; deficiency ; metabolism ; Female ; Fluorescent Antibody Technique ; Glomerulonephritis ; complications ; drug therapy ; metabolism ; pathology ; Hematuria ; etiology ; pathology ; Humans ; Kidney Glomerulus ; metabolism ; pathology ; Male ; Nephrotic Syndrome ; etiology ; pathology ; Proteinuria ; etiology ; pathology

OBJECTIVETo study the clinical and pathological features of Denys-Drash syndrome (DDS).

METHODThree DDS cases who were treated in our department from December 2009 to June 2011 were subjected to this study by reviewing of literature.

RESULTBoth case 1 and case 2 were female, with karyotype 46, XX. Case 3 was male with bilateral cryptorchidism. The ages of nephropathy onset of the three cases were 1 year and 9 months, 2 years and 8 moths, and 3 months respectively. Proteinuria in case 2 and case 3 were evidenced to be resistant to steroid. Case 1 was partially responsive to tacrolimus, plasma albumin and cholesterol were improved, although proteinuria was persistent after Tacrolimus was administered. Remission was achieved in case 2 after administration of cyclosporine A and later tacrolimus, and her renal function remains normal till present (4 years and 9 months). Residue renal histology revealed diffused mesangial sclerosis (DMS) in all three patients. All of the three patients had developed right unilateral Wilms tumor. A novel WT1 missense mutation exon 9 c.1213C > G was detected in case 1. WT1 exon 9 c.1168C > T nonsense mutation and exon 8 c.1130A > T missense mutation were detected in case 2 and case 3, respectively.

CONCLUSIONThe clinical manifestation of nephropathy in DDS is variable. The majority present with early onset nephropathy and reach renal failure before the age of 4 years. But in a few patients, nephropathy can also be present much later and progress slowly. Proteinuria in DDS is resistant to steroid but is responsive to calcineurin inhibitors, including Cyclosporine A. The effectiveness of tacrolimus was also observed in this study. DDS is evidently caused by WT1 mutation. DMS is the characteristic renal pathological change in DDS.

Cyclosporine ; therapeutic use ; Denys-Drash Syndrome ; drug therapy ; genetics ; pathology ; Fatal Outcome ; Female ; Genes, Wilms Tumor ; Heterozygote ; Humans ; Infant ; Male ; Mutation ; Nephrotic Syndrome ; drug therapy ; genetics ; pathology ; Proteinuria ; drug therapy ; Sclerosis ; drug therapy ; genetics ; pathology ; Tacrolimus ; therapeutic use ; Treatment Outcome ; WT1 Proteins ; genetics ; Wilms Tumor ; drug therapy ; genetics ; pathology

OBJECTIVETo study the clinical significance of urinary podocyte marker protein podocalyxin (PCX) in the diagnosis of primary nephrotic syndrome (PNS) in children and the evaluation of disease severity.

METHODSPCX levels in the first morning urine were measured by turbidimetric immunoassay (TIA) in 175 children, including 53 children with acute PNS [36 cases of simple nephrotic syndrome (NS) and 17 cases of nephritic NS], 56 children with PNS in the remission stage (relapsed: 42 cases) and 66 healthy children (control group). Twenty-four hour urinary protein was measured in the 53 children with acute PNS. The optimal operating points for the diagnosis of acute PNS and nephritic NS were determined using the receiver-operating characteristic curve (ROC curve).

RESULTSSignificant increasd levels of urinary PCX were found in children with acute PNS compared with those in the remission stage and the control group (P<0.01). A positive correlation was found between urinary PCX and 24 hour urinary protein in children with acute PNS (r=0.39, P<0.01). In children with acute PNS, urinary PCX levels were significantly higher in the nephritic NS group than in the simple NS group (P<0.05). In children in the remission stage, a significant increase in levels of urinary PCX was found in children who had relapsed compared with those who had not (P<0.05). The area under the ROC curve for the diagnosis of acute PNS and nephritic NS was 0.915 and 0.784 respectively. The optimal operating point for the diagnosis of acute PNS and nephritic NS was 7.97 and 10.28 ng/mL respectively, with a sensitivity and specificity of 81.1% and 93.4% respectively for acute PNS and of 94.1% and 52.8% respectively for nephritic NS.

CONCLUSIONSThe quantitative detection of urinary PCX is useful in the evaluation of podocyte dynamic changes. It is helpful in the diagnosis of acute PNS and in the differentiation of nephritic NS and simple NS.

Child ; Child, Preschool ; Female ; Humans ; Male ; Nephrotic Syndrome ; pathology ; urine ; Podocytes ; pathology ; ROC Curve ; Sialoglycoproteins ; urine

OBJECTIVEAcute kidney injury (AKI) was recently proposed for early recognition of renal function impairment and prompt interventions. Previous study revealed that AKI was highly associated with the prognosis. However, there was rare report of AKI in renal diseases, especially in children cohorts. Therefore, we performed the prospective clinical research in children with renal diseases in our hospital, aiming to study the prevalence, the clinical characteristics and the short-term prognosis of AKI.

METHODThe study was designed as a prospective, single-center observational study.

INCLUSION CRITERIA(1) the primary diagnosis was primary nephrotic syndrome (NS), Henoch-Schoenlein purpura nephritis (HSPN) or lupus nephritis (LN), (2) the duration from the onset of the renal diseases to the admission was less than 3 months. The serum creatinine and urine output of the subjects would be prospectively monitored. AKI was defined by the adult criteria and stratified by Acute Kidney Injury Network (AKIN) criteria. The patients were followed up at 6 months and 12 months after enrollment.

RESULTBetween October 2007 and April 2009, a total of 95 children were included, including 65 cases with NS, 15 HSPN and 15 LN. Mean age was (8.9 ± 3.9) years (range 2 - 16 years). Thirty-three of the 95 patients (34.7%) fulfilled the AKI criteria, 13 patients (13.7%) were diagnosed as acute renal failure (ARF). All the AKI in children with LN and HSPN presented with serum creatinine elevation. However, 65.4% of AKI in NS presented with decreasing urine output, only 19.2% accompanied with increasing creatinine, with higher stages of urine output. Regarding the etiology, only 26.9% of AKI in NS had definite cause, most of which resulted from side-effect of cyclosporine, hypovolemia or tubule-interstitial damage, independent of glomerular diseases. In contrast, the AKI in LN and HSPN were exclusively caused by glomerular diseases. The length and costs of hospitalization of AKI group were significantly higher than non-AKI [length of hospitalization (d), 28(6 to 94) vs. 21(7 to 100), Z = -1.971, P = 0.049; cost of hospitalization (yuan), 12 035.7 (1561.7 to 94 783.1) vs. 8594.3 (1390.1 to 98 876.5), Z = -1.993, P = 0.046]. There was no significant difference in the serum creatinine at 6-month and 12-month follow-up between AKI group and non-AKI [6-month, (60.4 ± 91.8) µmol/L vs. (42.8 ± 12.2) µmol/L, t = 0.937, P = 0.358; 12-month, (48.7 ± 18.1) µmol/L vs. (47.7 ± 14.2) µmol/L, t = 0.197, P = 0.845].

CONCLUSIONThe prevalence of AKI (34.7%) was higher than that of ARF (13.7%) in children with renal diseases. Most of the AKI in NS resulted from non-glomerular diseases. In contrast, most AKI in LN and HSPN were caused by underlying glomerular diseases. The length and costs of hospitalization were significantly higher in AKI group. However, there was no significant difference in serum creatinine between AKI and non-AKI group in the follow-up at 6 months and 12 months. Further investigations on criteria for the diagnosis of AKI in children with renal diseases are still needed.

Acute Kidney Injury ; etiology ; Adolescent ; Child ; Child, Preschool ; Female ; Humans ; Lupus Nephritis ; complications ; pathology ; Male ; Nephrotic Syndrome ; complications ; pathology ; Prospective Studies ; Purpura, Schoenlein-Henoch ; complications ; pathology ; Risk Factors