scholarly journals Difficult Renal Pathological Classification in a Case of Pediatric Nephrotic Syndrome

2017 ◽  
Vol 7 (3) ◽  
pp. 161-166
Author(s):  
Hiroshi Yamaguchi ◽  
Atsutoshi Shiratori ◽  
Taku Nakagawa ◽  
Kyoko Kanda ◽  
Shigeo Hara ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Complete Remission ◽  
Renal Biopsy ◽  
Clinical Course ◽  
Minimal Change ◽  
Albumin Infusion ◽  
Dense Deposits ◽  
Old Japanese ◽  
Foot Process ◽  
The Impact

The underlying histopathology is very important in determining patient management, as the histopathology usually has direct repercussions on the treatment response and clinical course. However, the impact of the method used to assess renal biopsies, i.e., light microscopy (LM), immunofluorescence (IF), and electron microscopy (EM), on the occurrence of a difficult biopsy classification in the native kidneys of pediatric nephrotic patients is unknown. A 12-month-old Japanese boy was diagnosed with nephrotic syndrome (NS); he was administered prednisolone (60 mg/m2/day), and a continuous albumin infusion was started. A renal biopsy using LM revealed minimal change. However, an IF study showed granular staining for immunoglobulin G along the glomerular basement membrane. Therefore, he was diagnosed with membranous nephropathy (MN). As his proteinuria was so severe, we started immunosuppressant therapy and continued the albumin infusion for more than 2 months. However, he did not attain complete remission. A month later, EM examination of his renal biopsy showed extensive foot process fusion without electron-dense deposits. Although the result of the IF study suggested MN, the results of the LM and EM studies indicated minimal change. We finally diagnosed the patient with minimal change NS, in consideration of his clinical condition and course. Because of the failure of previous treatments, pulse steroid therapy was started. After five rounds of therapy the patient attained complete remission. A difficult renal biopsy finding classification, dependent on the diagnostic method used, might occur in the native kidneys of pediatric nephrotic patients. Therefore, a diagnosis should be made after considering all renal biopsy findings and the clinical course.

Minimal change disease

2019 ◽  
pp. 175-224
Author(s):  
Claudio Ponticelli ◽  
Richard J Glassock ◽  
Rosanna Coppo
Keyword(s):  
Electron Microscopy ◽  
Nephrotic Syndrome ◽  
Light Microscopy ◽  
Renal Biopsy ◽  
Minimal Change Disease ◽  
The Elderly ◽  
Minimal Change ◽  
Common Cause ◽  
Foot Process

This chapter discusses minimal change disease (MCD), which is chiefly characterized clinically by episodes of nephrotic syndrome (NS) and presents with massive proteinuria, hypo-albuminaemia, hyperlipidaemia, and generalized oedema, Morphologically, it is characterized by no or only minimal glomerular abnormalities in a renal biopsy examined by light microscopy and immunofluorescence, while there is diffuse effacement of the podocyte foot process by electron microscopy. MCD is the most common cause of NS in children but it may also develop at any age, including in the elderly. This chapter covers the pathology, presentation, and treatment of MCD, including practical tips for the practitioner.

A case of minimal change disease during pregnancy – Benefits of early diagnosis and use of corticosteroids

2021 ◽  
pp. 1753495X2199021
Author(s):  
Priyanka S Sagar ◽  
Eddy Fischer ◽  
Muralikrishna Gangadharan Komala ◽  
Bhadran Bose
Keyword(s):  
Nephrotic Syndrome ◽  
Early Diagnosis ◽  
Renal Biopsy ◽  
Early Pregnancy ◽  
Minimal Change Disease ◽  
Minimal Change ◽  
Risk Of Bleeding ◽  
Increased Risk ◽  
Prompt Diagnosis ◽  
In Pregnancy

Nephrotic syndrome presenting in pregnancy is rare and poses a diagnostic and therapeutic challenge. Timing of renal biopsy is important given the increased risk of bleeding and miscarriage, and the choice of immunosuppression is limited due to the teratogenicity profiles of standard drugs. We report and discuss a case of minimal change disease diagnosed by renal biopsy during early pregnancy and treated with corticosteroids throughout the pregnancy. Prompt diagnosis and treatment of glomerular disease in pregnancy are vital to prevent poor maternal and fetal outcomes.

scholarly journals Clinical manifestations of focal segmental glomerulosclerosis in Japan from the Japan Renal Biopsy Registry: age stratification and comparison with minimal change disease

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Takaya Ozeki ◽  
Shoichi Maruyama ◽  
Toshiyuki Imasawa ◽  
Takehiko Kawaguchi ◽  
Hiroshi Kitamura ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Renal Biopsy ◽  
Focal Segmental Glomerulosclerosis ◽  
Clinical Diagnosis ◽  
Clinical Characteristics ◽  
Minimal Change Disease ◽  
Multivariable Analysis ◽  
Laboratory Data ◽  
Minimal Change ◽  
Segmental Glomerulosclerosis

AbstractFocal segmental glomerulosclerosis (FSGS) is a serious condition leading to kidney failure. We aimed to investigate the clinical characteristics of FSGS and its differences compared with minimal change disease (MCD) using cross-sectional data from the Japan Renal Biopsy Registry. In Analysis 1, primary FSGS (n = 996) were stratified by age into three groups: pediatric (< 18 years), adult (18–64 years), and elderly (≥ 65 years), and clinical characteristics were compared. Clinical diagnosis of nephrotic syndrome (NS) was given to 73.5% (97/132) of the pediatric, 41.2% (256/622) of the adult, and 65.7% (159/242) of the elderly group. In Analysis 2, primary FSGS (n = 306) and MCD (n = 1303) whose clinical diagnosis was nephrotic syndrome (NS) and laboratory data were consistent with NS, were enrolled. Logistic regression analysis was conducted to elucidate the variables which can distinguish FSGS from MCD. On multivariable analysis, higher systolic blood pressure, higher serum albumin, lower eGFR, and presence of hematuria associated with FSGS. In Japanese nationwide registry, primary FSGS patients aged 18–64 years showed lower rate of NS than those in other ages. Among primary nephrotic cases, FSGS showed distinct clinical features from MCD.

scholarly journals P0338CRUMBS HOMOLOG2-EZRIN SIGNALING INDUCED PODOCYTE INJURY, LEADING TO MINIMAL-CHANGE DISEASE(MCD) AND FOCAL SEGMENTAL GLOMERULOSCLEROSIS(FSGS)

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Ichiro Hada
Keyword(s):  
Electron Microscopy ◽  
Nephrotic Syndrome ◽  
Cell Line ◽  
Focal Segmental Glomerulosclerosis ◽  
Minimal Change Disease ◽  
Immunofluorescence Microscopy ◽  
Minimal Change ◽  
Podocyte Injury ◽  
Heavy Proteinuria ◽  
Foot Process

Abstract Background and Aims The etiology and cellular pathogenesis of podocyte injury leading to minimal-change disease (MCD) and focal segmental glomerulosclerosis (FSGS) remain largely obscure. Genetic mutation of crumbs homolog 2 (CRB2) is a cause of congenital nephrotic syndrome. Type-1 transmembrane proteins including CRB2 transduce outside-in signals that are involved in various cellular events including changes in the cytoskeletal network. The aim of the present study is to determine whether alteration of CRB2-mediated signaling in podocytes causes MCD and FSGS. Method Mice were immunized with a partial recombinant protein including the extracellular part of mouse CRB2. Urinalysis was obtained, and the kidney was subjected to histopathology. Kidney samples were also subjected to immunofluorescence microscopy and glomerular isolation to determine whether activation of the ezrin/radixin/moesin (ERM) family of cross-linkers between plasma membrane proteins and the actin cytoskeleton is involved in the pathogenesis of this nephrotic model. A CRB2-expressing mouse podocyte cell line was generated and incubated with anti-CRB2 antibody, and cell lysates were subjected to immunoblot analysis of ERM phosphorylation. The presence of anti-CRB2 antibody in the serum was determined by Western blot analysis. Results Apparent anti-CRB2 antibody was detected in the serum from 4 weeks onward. Immunized mice developed proteinuria at 4 weeks, which continued at least until 29 weeks. Mice developing extremely heavy proteinuria also developed hematuria from 18 weeks onward. Light microscopy revealed MCD in mice with proteinuria alone and FSGS in mice with heavy proteinuria and hematuria. Immunofluorescence microscopy revealed positive granular IgG staining in podocyte foot processes, but not complement C3. Electron microscopy and immuno-electron microscopy revealed alteration of actin organization associated with prominent foot process effacement. Strong phosphorylation of ezrin was observed in the glomerulus from the proteinuric stage and in the cellular lysates from the CRB2-expressing podocyte cell line incubated with anti-CRB2 antibody. Conclusion The current data revealed that binding of anti-CRB2 antibody to the extracellular domain of CRB2 on the podocyte foot process activated the ezrin-cytoskeleton network, leading to podocyte injury. Our data also indicated that signaling by this one molecular can induce two different phenotypes of glomerular injury: MCD and FSGS. In our model, the signaling was activated by anti-CRB2 antibody, but in patients with nephrotic syndrome the CRB2 ligands remain unknown. Therefore, it will be important to identify the soluble factors interacting with CRB2, which may be novel factors contributing to the pathogenesis of MCD and FSGS.

scholarly journals Effects of plasma albumin infusion on minimal change nephrotic syndrome.

1988 ◽  
Vol 77 (8) ◽  
pp. 1219-1223 ◽  
Author(s):  
Ashio YOSHIMURA ◽  
Terukuni IDEURA ◽  
Yasuki HASHIMOTO ◽  
Shozo KOSHIKAWA
Keyword(s):  
Nephrotic Syndrome ◽  
Minimal Change Nephrotic Syndrome ◽  
Minimal Change ◽  
Plasma Albumin ◽  
Albumin Infusion

A Patient with IgA Nephropathy: 5 Years after Complete Remission of Minimal Change Nephrotic Syndrome

2016 ◽  
Vol 39 (4) ◽  
pp. 118 ◽  
Author(s):  
Ji Won Kim ◽  
Jun Hyung Park ◽  
Da Hee Kim ◽  
Hyung Young Kim ◽  
Sang Hyun Kim ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Complete Remission ◽  
Iga Nephropathy ◽  
Minimal Change Nephrotic Syndrome ◽  
Minimal Change

scholarly journals Establishment of a novel nomogram for the clinically diagnostic prediction of minimal change disease, −a common cause of nephrotic syndrome

2020 ◽  
Vol 21 (1) ◽  
Author(s):  
Gaofei Yan ◽  
Guanzhi Liu ◽  
Xuefei Tian ◽  
Lifang Tian ◽  
Hao Wang ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Renal Biopsy ◽  
Minimal Change Disease ◽  
Clinical Manifestations ◽  
Minimal Change ◽  
Adult Patients ◽  
Lasso Regression ◽  
Glomerular Diseases ◽  
Laboratory Test Results ◽  
Primary Glomerular Diseases

Abstract Background Minimal change disease (MCD) is one of the major causes of nephrotic syndrome (NS). A confirmed MCD diagnosis mainly depends on renal biopsy at present, which is an invasive procedure with many potential risks. The overall incidence of complications caused by renal biopsy procedures has been reported as approximately 11 and 6.6% outside and within China, respectively. Unfortunately, there is currently no noninvasive procedure or practical classification method for distinguishing MCD from other primary glomerular diseases available. Method A total of 1009 adult patients who underwent renal biopsy between January 2017 and November 2019 were enrolled in this study. Twenty-five parameters extracted from patient demographics, clinical manifestations, and laboratory test results were statistically analysed. LASSO regression analysis was further performed on these parameters. The parameters with the highest area under the curve (AUC) were selected and used to establish a logistic diagnostic prediction model. Results Of the 25 parameters, 14 parameters were significantly different (P < 0.05). MCD patients were mostly younger (36 (22, 55) vs. 41 (28.75, 53)) and male (59% vs. 52%) and had lower levels of diastolic blood pressure (DBP) (79 (71, 85.5) vs. 80 (74, 89)) and IgG (5.42 (3.17, 6.36) vs. 9.38 (6.79, 12.02)) and higher levels of IgM (1.44 (0.96, 1.88) vs. 1.03 (0.71, 1.45)) and IgE (160 (46.7, 982) vs. 47.3 (19, 126)) than those in the non-MCD group. Using the LASSO model, we established a classifier for adults based on four parameters: DBP and the serum levels of IgG, IgM, IgE. We were able to clinically classify adult patients with NS into MCD and non-MCD using this model. The validation accuracy of the logistic regression model was 0.88. A nomogram based on these four classifiers was developed for clinical use that could predict the probability of MCD in adult patients with NS. Conclusions A LASSO model can be used to distinguish MCD from other primary glomerular diseases in adult patients with NS. Combining the model and the nomogram potentially provides a novel and valuable approach for nephrologists to diagnose MCD, avoiding the complications caused by renal biopsy.

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