scholarly journals A Clinico-Pathological Correlation of Childhood Primary Nephrotic Syndrome in Jakarta

2019 ◽  
Vol 33 (7-8) ◽  
pp. 150-8
Author(s):  
I. G. N. Wila Wirya
Keyword(s):  
Nephrotic Syndrome ◽  
Kidney Disease ◽  
Child Health ◽  
Medical School ◽  
Membranoproliferative Glomerulonephritis ◽  
Clinical Laboratory ◽  
International Study ◽  
Histopathological Changes ◽  
Primary Nephrotic Syndrome ◽  
Pathological Characteristics

Three hundred and sixty-four out of 547 children with primary nephrotic syndrome (PNS) treated at the Department of Child Health, Medical School, University of Indonesia between January 1970 and December 1979, were biopsied. The clinical, laboratory, and pathological characteristics of these patients were evaluated and compared with those reported by the International Study of Kidney Disease in Children (ISKOC, 1978) comprising children of Western countries with PNS. Results of this study showed that the spectrum of the histopathological changes in the 2 study populations was slightly different. Clinical and laboratory characteristics of certain types of PNS also showed some differences compared with those of ISKDC report. Patients with non-minimal changes nephrotic syndrome (NMCNS) were more frequently found in this study, and they were older than those of ISKDC report, i.e., more children < 6 years of age were affected with this disease in Jakarta. Similarly, this study showed that patients with minimal changes nephrotic syndrome (MCNS) were older than those of ISKDC report; the peak age of Western patients was less than 6 years. Patients with focal segmental glomerulosclerosis (FSGS) and membranoproliferative glomerulonephritis (MPGN) reported by ISKDC were more severe than those of this study as far as clinical and laboratory characteristics were concerned. The causes of these clinical and histopathological discrepancies between the two studies need to be further elucidated.

scholarly journals C3 and C4 Complements in Glomerular Disorders in Children

2017 ◽  
Vol 18 (3-4) ◽  
pp. 75
Author(s):  
H. Alatas ◽  
I.G.N. Wila Wirya ◽  
T. Tambunan
Keyword(s):  
Nephrotic Syndrome ◽  
Child Health ◽  
Normal Level ◽  
Membranoproliferative Glomerulonephritis ◽  
Kidney Diseases ◽  
Immunosuppressive Treatment ◽  
The Other ◽  
Poststreptococcal Glomerulonephritis ◽  
Acute Poststreptococcal Glomerulonephritis ◽  
Almost All

Seventy children who were hospitalized for kidney diseases in the Nephrological ward Department of Child Health, University of Indonesia, Jakarta were used in this study. Thirty seven patients sufferfng from acute poststreptococcal Glomerulonephritis (A.G.N.), 3 patients with Membranoproliferative Glomerulonephritis (M.P.G.N.) and 30 patients with Nephrotic Syndrome due to other causes were examined for complement concentration. A total of 80 samples were examined for C3 and 25 samples for C4 concentration using the immunediffusion plates. Almost all patients with A.G.N. and M.P.G.N. showed depression of C3. C4 concentration was normal except in 2 patients, 1 with A.G.N. and the other With M.P.G.N. This suggest activation of complement at the C3 level by the alternating pathway in most of the patients. C3 concentration in A.G.N. patients returned to normal after 8-10 weeks. In MPGN the depression was persistent in 2 patients, while in 1 patient it returned to normal level after 3 months of Immunosuppressive treatment.

scholarly journals Portal Vein Thrombosis in a 21-Year-Old Man with Membranoproliferative Glomerulonephritis and Nephrotic Syndrome

2019 ◽  
Vol 2019 ◽  
pp. 1-3
Author(s):  
Ilya Seleznev ◽  
Dinara Jumadilova ◽  
Assiya Naushabayeva ◽  
Kairat Kabulbayev ◽  
Gulaiym Karashasheva ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Portal Hypertension ◽  
Liver Disease ◽  
Kidney Disease ◽  
Portal Vein ◽  
Membranoproliferative Glomerulonephritis ◽  
Microscopic Haematuria ◽  
Vein Thrombosis ◽  
Increased Risk ◽  
Portosystemic Collaterals

Membranoproliferative glomerulonephritis, one of the main causes of nephrotic syndrome, is associated with a state of hypercoagulability that leads to increased risk of thrombotic events. Portosystemic collaterals may reopen due to reversal of the flow within the existing veins and be a presenting feature of thrombosis. We describe a patient who presented with large portosystemic collaterals and signs of portal hypertension and was subsequently found to be affected by membranous proliferative glomerulonephritis. Proteinuria and microscopic haematuria in a patient with signs of portal hypertension and no pre-existing liver disease should raise the suspicion of an underlying kidney disease.

scholarly journals An Update of Management of Idiopathic Nephrotic Syndrome: A Review Article

2013 ◽  
Vol 37 (2) ◽  
pp. 102-121
Author(s):  
MH Rahman ◽  
T Jesmin ◽  
G Muinuddin
Keyword(s):  
Nephrotic Syndrome ◽  
Kidney Disease ◽  
Child Health ◽  
Idiopathic Nephrotic Syndrome ◽  
Indian Subcontinent ◽  
Minimal Change Nephrotic Syndrome ◽  
Review Article ◽  
Minimal Change ◽  
Historical Background

Nephrotic syndrome (NS) is a common childhood kidney disease characterized by protein leakage from the blood to the urine through the glomeruli, resulting of proteinuria, hypoalbuminemia, generalized edema and hypercholesterolemia. The prevalence of minimal change nephrotic syndrome is higher in Indian subcontinent. Such incidence in Bangladesh is yet unknown. This review article discusses historical background, epidemiology, pathogenesis, pathophysiology and classification of nephrotic syndrome. In this review article focuses have been made on management of children aged between 1 to 18 years with idiopathic nephrotic syndrome. This article also provides information of different guideline recommendations and a brief review of relevant treatment trials related to each recommendation. DOI: http://dx.doi.org/10.3329/bjch.v37i2.17268 BANGLADESH J CHILD HEALTH 2013; VOL 37 (2) : 102-121

scholarly journals Histopathological Features of Primary Nephrotic Syndrome in Children

2017 ◽  
Vol 38 (1-2) ◽  
pp. 20
Author(s):  
M P Damanik ◽  
N Yoshikawa
Keyword(s):  
Nephrotic Syndrome ◽  
Renal Biopsy ◽  
Membranoproliferative Glomerulonephritis ◽  
Membranous Glomerulonephritis ◽  
Steroid Treatment ◽  
Mesangial Proliferative Glomerulonephritis ◽  
Primary Nephrotic Syndrome ◽  
Biopsy Specimens ◽  
Significant Difference ◽  
Steroid Sensitive

 Renal biopsy was performed on 28 out of 50 children with primary nephrotic syndrome encountered during the period January 1994 - December 1995. Light microscope (LM) and immunofluorescence microscope QM) examinations were performed on all biopsy specimens. LM examination indicated minimal changes (MC) in 13 cases (46.4%), focal segmental glomerulosclerosis (FSGS) in 10 (35.7%), membranous glomerulonephritis (MG) in 2 (7.1%), mesangial proliferative glomerulonephritis (MPG) in 7 (7.1 %), and membranoproliferative glomerulonephritis (MPGN) in 1 (3.6%). On IM examination, immunoglobulin deposit was not detected in any MC patients, whereas in FSGS, lgG, lgM, C3 and fibrinogen deposits were found. In the MG group, IgG deposition was detected in one case. In the MPG cases, depositions of lgA, IgG, lgM, C3 and fibrinogen were detected and in the case of MPGN, deposits of lgM and C3 were found. Regarding to response to steroid treatment in the MC group, there was a significant difference between the steroid sensitive and steroid insensitive (p<0.05). For the FSGS abnormality in the steroid treatment of U1e insensitive patients, there was found significant difference with the steroid sensitive abnormality (p<0.05). In conclusion, nephritic symptoms (hematuria, proteinuria, azothemia) are possibly the non minimal group and hence, it would be necessary to carry out renal biopsy to prove this.

scholarly journals Association of Kidney Biopsy Findings With Short- and Medium-Term Outcomes in Children With Moderate-to-Severe Iga Vasculitis Nephritis

2021 ◽  
Author(s):  
Stéphanie Clavé ◽  
Maud Sordet ◽  
Michel Tsimaratos ◽  
Stéphane Decramer ◽  
Marc Fila ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Kidney Disease ◽  
Kidney Biopsy ◽  
Immunoglobulin A ◽  
International Study ◽  
Clinical Severity ◽  
Grade Ii ◽  
Kidney Impairment ◽  
Grade Iii

Abstract Assessing the initial severity of immunoglobulin A vasculitis nephritis (IgAV-N) is challenging important due to its determining effect on kidney management and outcomes. This study paper aims to describe describes a multicentre paediatric multicenter pediatric cohort of IgAV-N patients and discusses whilst investigating the relationships among between clinical presentation, histological features, and kidney outcome. A cohort consisting of 170 children requiring early kidney with biopsy because of IgAV-N, which was diagnosed between 2007 and 2017, was assessed including 27% of children with nephrotic syndrome (NS). One-quarter of the cohort (27%) presented with initial nephrotic syndrome (NS). Kidney biopsy revealed International Study of Kidney Disease (ISKDC) grade II or grade III in 83% of cases. An International Study of Kidney Disease (ISKDC) grade II or grade III was revealed through kidney biopsy in 83% of cases. Endocapillary proliferation was were observed in 73% of patients, and chronic lesions were observed in 25%of patients. Data analysis demonstrated showed a significant association between clinical severity (NS at onset and histological findings such asendocapillary proliferation and cellular crescents. After a median follow-up of 21 months (IQR 12-39), 30% of patients had persistent kidney impairment (proteinuria or decreased eGFR. WorseAt the end of follow-up, kidney outcome impairment was significantly associated more often observed in patients with NS at onset and those with cellular crescents and chronic lesions on initial kidney biopsy.Conclusions: This study highlights the relationship between the clinical and histological presentation of IgAV-N and the factors that affect kidney outcome. The ISKDC classification may be improved by including lesions that are more discriminating for disease severity and prognosis.

scholarly journals Primary Nephrotic Syndrome and Risks of End-Stage Kidney Disease, Cardiovascular Events, and Death: The Kaiser Permanente Nephrotic Syndrome Study

2021 ◽  
pp. ASN.2020111583
Author(s):  
Alan Go ◽  
Thida Tan ◽  
Glenn Chertow ◽  
Juan Ordonez ◽  
Dongjie Fan ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Kidney Disease ◽  
Membranous Nephropathy ◽  
Minimal Change Disease ◽  
Cardiovascular Outcomes ◽  
Minimal Change ◽  
End Stage Kidney Disease ◽  
Kaiser Permanente ◽  
Primary Nephrotic Syndrome ◽  
End Stage

Background Few population-based data exist about adults with primary nephrotic syndrome. Methods To evaluate kidney, cardiovascular, and mortality outcomes in adults with primary nephrotic syndrome, we identified adults within an integrated health care delivery system (Kaiser Permanente Northern California) with nephrotic-range proteinuria or diagnosed nephrotic syndrome between 1996-2012. Nephrologists reviewed medical records for clinical presentation, laboratory findings, and biopsy results to confirm primary nephrotic syndrome and assigned etiology. We identified a 1:100 time-matched cohort of adults without diabetes, diagnosed nephrotic syndrome, or proteinuria as controls to compare rates of end-stage kidney disease (ESKD), cardiovascular outcomes, and death through 2014, using multivariable Cox regression. Results We confirmed 907 cases of primary nephrotic syndrome (655 definite and 252 presumed cases of focal segmental glomerulosclerosis [FSGS, 40%], membranous nephropathy [40%], and minimal change disease [20%]). Mean age was 49 years; 43% were women. Adults with primary nephrotic syndrome had higher adjusted rates of ESKD (adjusted hazard ratio [aHR], 19.63; 95% confidence interval [95% CI], 12.76 to 30.20), acute coronary syndrome (aHR 2.58; 95% CI, 1.89 to 3.52), heart failure (aHR 3.01; 95%CI, 2.16 to 4.19), ischemic stroke (aHR 1.80; 95%CI,1.06 to 3.05), venous thromboembolism (aHR 2.56; 95%CI, 1.35 to 4.85), and death (aHR 1.34; 95%CI, 1.09 to 1.64) versus controls. Excess ESKD risk was significantly higher for FSGS and membranous nephropathy than for presumed minimal change disease. The three etiologies of primary nephrotic syndrome did not differ significantly in terms of cardiovascular outcomes and death. Conclusions Adults with primary nephrotic syndrome experience higher adjusted rates of ESKD, cardiovascular outcomes, and death, with significant variation by underlying etiology in risk for developing ESKD.

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