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 Nephrotic syndrome associated with hepatointestinal schistosomiasis

1992 ◽  
Vol 34 (4) ◽  
pp. 273-276 ◽  
Author(s):  
H. Abensur ◽  
I. Nussenzveig ◽  
L.B. Saldanha ◽  
M.S.C. Petalozzi ◽  
M.T. Barros ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Membranoproliferative Glomerulonephritis ◽  
Idiopathic Nephrotic Syndrome ◽  
Membranous Glomerulonephritis ◽  
Proliferative Glomerulonephritis ◽  
Control Group ◽  
Mesangial Proliferative Glomerulonephritis ◽  
Renal Lesions ◽  
Histological Types

Schistosomal nephropathy has long been related to the hepatosplenic form of schistosomiasis. In the last few years, 24 patients with hepatointestinal schistosomiasis and the nephrotic syndrome were studied. Aiming at evaluating a possible etiologic participation of schistosomiasis in the development of the nephropathy, this group was comparatively studied with a group of 37 patients with idiopathic nephrotic syndrome. Both groups had a different distribution of the histologic lesions. In the group with schistosomiasis there was a statistically significant prevalence of proliferative mesangial glomerulonephritis (33.3%), whereas in the control group there was prevalence of membranous glomerulonephritis (32.4%). On immunofluorescence, IgM was positive in 94.4% of the patients with schistosomiasis versus 55.0% in the control group (p<0.01). In the group with schistosomiasis, 8 patients evidenced mesangial proliferative glomerulonephritis and 5, membra-noproliferative glomerulonephritis. In both histological types immunofluorescence showed IgM and C3 granular deposits in the glomeruli. The data in this study suggests that mesangial proliferative and membranoproliferative glomerulonephritis, with glomerular granular IgM and C3 deposits, represent the renal lesions of the schistosomiasis associated nephropathy.

scholarly journals Steroid treatment for the first episode of childhood nephrotic syndrome: comparison of the 8 and 12 weeks regimen using an individual patient data meta-analysis

2021 ◽  
Author(s):  
Anne M. Schijvens ◽  
Nynke Teeninga ◽  
Eiske M. Dorresteijn ◽  
Steven Teerenstra ◽  
Nicholas J. Webb ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Individual Patient Data ◽  
Meta Analysis ◽  
Steroid Treatment ◽  
First Episode ◽  
Childhood Nephrotic Syndrome ◽  
Steroid Sensitive Nephrotic Syndrome ◽  
Steroid Sensitive ◽  
First Relapse ◽  
Steroid Regimen

AbstractSteroids are the cornerstone of the treatment of childhood nephrotic syndrome. The optimal duration for the first episode remains a matter of debate. The aim of this study is to determine whether the 8 weeks International Study of Kidney Disease in Children (ISKDC) regimen is equally effective as the 12 weeks steroid regimen from the German society of pediatric nephrology (Arbeitsgemeinschaft für Pädiatrische Nephrologie [APN]). An individual patient data (IPD) meta-analysis of randomized controlled trials reporting on prednisolone treatment for a first episode of childhood nephrotic syndrome was conducted. European trials aimed at investigating the ISKDC and/or APN steroid regimen were selected. The lead investigators of the selected trials were requested to provide the IPD of the specific treatment groups. Four trials included European cohorts using dosing schedules according to the regimens studied. IPD of two trials were available. A significant difference was found in time to first relapse after cessation of steroid treatment between the 8 and 12 weeks treatment group with a median time to relapse of 29 and 63 days, respectively. Moreover, relapse rate ratios during total follow-up were 51% higher for the 8 weeks regimen. Finally, younger children have a significantly lower time to first relapse and frequently relapsing nephrotic syndrome.Conclusions: The results of this IPD meta-analysis suggest that the 8 weeks steroid regimen for a first episode of steroid-sensitive nephrotic syndrome may not be equally effective as the 12 weeks steroid regimen. Moreover, this study highlights the importance of using uniform definitions to enable accurate comparison and interpretation of trial results.Trial registration: Registration number: CRD42020199244, date of registration 16-08-2020 What is Known:• Steroids are the cornerstone of the treatment of childhood nephrotic syndrome, however the optimal duration for the first episode remains a matter of debate.• Currently, the 8 weeks ISKDC protocol and 12 weeks APN protocol are among the most frequently used protocols in Europe. What is New:• The 8 weeks steroid regimen for a first episode of steroid-sensitive nephrotic syndrome may not be equally effective as the 12 weeks steroid regimen for the treatment of a first episode of nephrotic syndrome.• Younger children have a significantly shorter time to first relapse and time to frequent relapsing nephrotic syndrome.

Membranous glomerulonephritis

2018 ◽  
Author(s):  
Daniel C. Cattran ◽  
Heather N. Reich
Keyword(s):  
Nephrotic Syndrome ◽  
Hepatitis B ◽  
Renal Biopsy ◽  
Clinical Picture ◽  
Membranous Nephropathy ◽  
Membranous Glomerulonephritis ◽  
Hepatitis B Infection ◽  
Immunoglobulin G4 ◽  
Secondary Membranous Nephropathy ◽  
Secondary Causes

Membranous glomerulonephritis (MGN) usually presents as nephrotic syndrome, which may be severe. It is primarily a disease of adults, men more than women, with a peak incidence in the fourth and fifth decades. It is hoped that proven tests for the characteristic anti-PLA2R antibodies of primary MGN may become established, but the diagnosis currently rests on renal biopsy showing characteristic subepithelial granular immune deposits. These usually contain immunoglobulin G4 and complement. Other patterns may suggest secondary causes of MGN. Secondary membranous nephropathy occurs in lupus and some other immune or autoimmune disorders, in hepatitis B infection, after exposure to some drugs or toxins, and in some cancers. Secondary causes are more common at extremes of age, and are often made obvious by the history or clinical picture. How hard to look for malignancy is controversial, but malignancy is much more likely in patients over 60 years, and may be apparent at presentation.

scholarly journals HLA-B60 and HLA-DR4 alleles in Javanese children with steroid-sensitive primary nephrotic syndrome

2016 ◽  
Vol 46 (6) ◽  
pp. 246
Author(s):  
M P Damanik
Keyword(s):  
Nephrotic Syndrome ◽  
Strong Association ◽  
Control Sample ◽  
Hla Class I ◽  
Control Group ◽  
Healthy Children ◽  
Primary Nephrotic Syndrome ◽  
Steroid Sensitive Nephrotic Syndrome ◽  
Steroid Sensitive ◽  
Sequence Specific Oligonucleotide Probe

Background Steroid-sensitive nephrotic syndrome (SSNS) ofchildren is associated with several human leucocyte antigen (HLA)class I and class II.Objective To investigate the association between HLA-B60 andHLA-DR4 alleles and primary nephrotic syndrome (PNS) inJavanese children.Methods A case control study was conducted on 47 Javanesechildren with PNS who were typed for HLA-B60 and HLA-DR4 al-leles, using DNA sequence specific oligonucleotide probe (SSOP)as control sample, 47 healthy children were also typed for thoseHLA antigens using the same technique.Results Compared with control group, children with PNS had higherfrequency of both HLA-B60 (23.32% vs 4.3%; OR=6.85 [CI=1.32-35.65]; P<0.01) and HLA-DR4 (40.0% vs 2.1%; OR=30.67 [CI:3.71-253.33]; P<0.0002). There was association between HLA and PNSwith SSNS in children.Conclusion The strong association between PNS and HLA anti-gen support the immunogenetic background of the disease, whichseems to be stronger in young children with SSNS.

scholarly journals Serum and Urine ANGPTL8 Expression Levels are Associated with Hyperlipidemia and Proteinuria in Primary Nephrotic Syndrome

2020 ◽  
Author(s):  
Xia Gao ◽  
Qingju Liu ◽  
Chengdong Kang ◽  
Weijing Cui ◽  
Zichuan Xu ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
High Density Lipoprotein ◽  
Density Lipoprotein ◽  
Blood Lipid ◽  
Healthy Controls ◽  
Mesangial Proliferative Glomerulonephritis ◽  
Lipid Levels ◽  
Primary Nephrotic Syndrome ◽  
Serum And Urine ◽  
Blood Lipid Levels

Abstract Background: This study aimed to investigate the expression characteristics of ANGPTL8 in patients with primary nephrotic syndrome and its possible correlationwith hyperlipidemia and proteinuria.Methods: ANGPTL8 levels were determined using Enzyme‑linked immunosorbentassay in 133 subjects with PNS, and 60 subjects with healthy controls.Results: Subjects with primary nephrotic syndrome had higher levels of serum andurine ANGPTL8 than healthy controls subjects (P < 0.001). In primary nephroticsyndrome patients, serum ANGPTL8 was positively correlated with cholesterol (r =0.209, P < 0.05) and triglycerides (r = 0.412, P < 0.001), while no correlation with24hUTP. Urine ANGPTL8 was positively correlated with high density lipoprotein-cholesterol (r = 0.181, P < 0.05), while urine ANGPTL8 was significantly negativelycorrelated with creatinine (r = -0.323, P<0.001) and 24hUTP (r = -0.268, P = 0.002).Interestingly, urine ANGPTL8 concentrations were different between membranousnephropathy and mesangial proliferative glomerulonephritis pathological types.Conclusions: Serum and urine ANGPTL8 levels in primary nephrotic syndromepatients were correlated with blood lipid levels and proteinuria, respectively, suggestingthat ANGPTL8 may play a role in the development of primary nephrotic syndromehyperlipidemia and proteinuria.

scholarly journals Renal Histopathology in Childhood Nephrotic Syndrome at National Institute of Kidney Diseases and Urology, Dhaka, Bangladesh– an experience of one decade

2017 ◽  
Vol 8 (1) ◽  
pp. 165-169
Author(s):  
M Kabir Alam ◽  
Delwar Hossain ◽  
Anwar Hossain Khan ◽  
BH Nazma Yasmeen ◽  
Mahbub Ul Alam ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Renal Biopsy ◽  
Minimal Change Disease ◽  
Kidney Diseases ◽  
Minimal Change ◽  
Proliferative Glomerulonephritis ◽  
Mesangial Proliferative Glomerulonephritis ◽  
Childhood Nephrotic Syndrome ◽  
Renal Histopathology ◽  
Nephritic Syndrome

Background : Nephrotic syndrome (NS) is one of the most common renal diseases in children. The cause of idiopathic nephrotic syndrome is still unknown. Once the prevalence of minimal change nephritic syndrome occupied the three forth portion of the total renal pathology and most of them were steroid sensitive. But list of steroid insensitive nephritic syndrome become more longer today. Therefore renal biopsy is essential for histopathological diagnosis which guides the most accurate way for the treatment of such diseases.Objective : The objective of this study was to find out the pattern of renal histopathology of selected cases of Idiopathic nephritic syndrome.Materials and Method : This prospective study was conducted from January 2004 to December 2015 among children who were suffering from nephrotic syndrome admitted in Paediatric nephrology department, National Institute of Kidney Diseases and Urology(NIKDU), Dhaka. Renal biopsy were done who fulfil the inclusion criteria. Obtaining ultrasound-guided percutaneous renal biopsy specimens by means of an automated biopsy gun, were evaluated histopathologically under light and direct immunoflurescent(DIF) microscopy by an experienced histopathologist.Results : Total admitted childhood nephrotic syndrome during this period was 1512 and renal biopsy was done in 354 patient. Among the 354 children, histopathological findings were mesangial proliferative glomerulonephritis [MesPGN]was 92(25.98%),minimal change disease[MCD]was79 (22.32%), IgM nephropathy[IgMN]was69(19.49%), focal segmental glomerulosclerosis[FSGS] was 37(10.45%), membranoproliferative glomerulonephritis [MPGN] was 37(10.45%), IgA nephropathy [IgAN] was 20(5.65%),membranous nephropathy[MN] was 08(2.27%) and others were 12(3.39%).Conclusion : In this study we found that theselected patient for renal biopsy in the last 12 years showed that minimal change disease had been decreasing but the other histological types are increasing gradually such as mesangial proliferative glomerulonephritis, FSGS and IgM nephropathy.Northern International Medical College Journal Vol.8(1) July 2016: 165-169

Outcomes of primary nephrotic syndrome in elderly Japanese: retrospective analysis of the Japan Renal Biopsy Registry (J-RBR)

2014 ◽  
Vol 19 (3) ◽  
pp. 496-505 ◽  
Author(s):  
Hitoshi Yokoyama ◽  
Hitoshi Sugiyama ◽  
Ichiei Narita ◽  
Takao Saito ◽  
Kunihiro Yamagata ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Renal Biopsy ◽  
Retrospective Analysis ◽  
Primary Nephrotic Syndrome ◽  
Elderly Japanese

scholarly journals Glomerular Diseases in Children - A Review of 27 Cases Recorded at a Single Centre in Eastern Nepal

1970 ◽  
Vol 29 (1) ◽  
pp. 26-29 ◽  
Author(s):  
P Shrestha ◽  
NK Bhatta ◽  
A Tiwari ◽  
R Singh ◽  
BK Kalakhetti ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Medical Records ◽  
Membranoproliferative Glomerulonephritis ◽  
Kidney Biopsy ◽  
Clinical Presentation ◽  
Age At Onset ◽  
Single Centre ◽  
Glomerular Diseases ◽  
Biopsy Specimens ◽  
Nephrotic Range Proteinuria

Glomerular disease remains an important cause of renal disease in children. We retrospectively assessed the clinical features and histopathological diagnoses in kidney biopsy specimens in children (n=27). The medical records and histopathological diagnoses between 2003 and 2006 were reviewed. In each patient, clinical presentation, age at onset of disease, gender, presence of nephrotic range proteinuria, hematuria, hypertension and histopathological diagnoses were evaluated. SLE nephritis (n=8) and membranoproliferative glomerulonephritis (MPGN) (n=8) were the most frequent glomerular diseases and nephrotic syndrome (n=21) was the frequent clinical manifestation. Key words: Renal biopsy, Glomerular diseases doi:10.3126/jnps.v29i1.1597 J. Nepal Paediatr. Soc. Vol.29(1) p.26-29

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