Minimal change disease

2018 ◽  
Author(s):  
Patrick Niaudet ◽  
Alain Meyrier
Keyword(s):  
Differential Diagnosis ◽  
Nephrotic Syndrome ◽  
Renal Biopsy ◽  
Minimal Change Disease ◽  
Minimal Change ◽  
Direct Effects ◽  
Glomerular Filtration Barrier ◽  
Common Cause ◽  
Filtration Barrier ◽  
Steroid Responsiveness

Minimal change disease is the most common cause of nephrotic syndrome in childhood but is not rare in adults. The factors altering permeability of the glomerular filtration barrier are not known, but podocyte structure is significantly altered in the condition and it seems certain that this cell is the target of whatever factors are responsible for the condition. It is still not clear that it is immunologically mediated and many of the agents used to treat it have direct effects on the podocyte. The differential diagnosis includes any other disease causing nephrotic syndrome, and a renal biopsy narrows this down. In children, steroid unresponsiveness is often used as a diagnostic test, and consideration of genetic or other pathologies reserved for patients who show no or poor steroid responsiveness.

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 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.

Minimal change nephropathy

2009 ◽  
pp. 179-214 ◽  
Author(s):  
Rosanna Coppo ◽  
Claudio Ponticelli
Keyword(s):  
Nephrotic Syndrome ◽  
Light Microscopy ◽  
Renal Biopsy ◽  
Minimal Change Disease ◽  
Minimal Change ◽  
Minimal Change Nephropathy ◽  
The Usa

Minimal change nephropathy (MCN), also called ‘minimal change disease’ in the USA, is chiefly characterized by episodes of nephrotic syndrome—presenting with massive proteinuria, hypoalbuminemia, generalized edema, hyperlipidemia—and no lesions or only minimal glomerular abnormalities in the renal biopsy examined by light microscopy.

scholarly journals RENAL BIOPSY IN PAEDIATRIC POPULATION

1969 ◽  
Vol 3 (2) ◽  
pp. 314-317
Author(s):  
AHMAD ZEB KHAN ◽  
RIAZ GUL ◽  
AZIZ AHMAD
Keyword(s):  
Nephrotic Syndrome ◽  
Renal Biopsy ◽  
Focal Segmental Glomerulosclerosis ◽  
Minimal Change Disease ◽  
Paediatric Population ◽  
Minimal Change ◽  
Segmental Glomerulosclerosis ◽  
Renal Biopsies ◽  
A Prospective Study ◽  
Nephrotic Range Proteinuria

OBJECTIVE: To find out the pattern of glomerulopathies in paediatric population, undergoing renalbiopsy at Khyber Teaching Hospital, Peshawar.METHODS: This was a prospective study carried out at the department of Nephrology at Khyber TeachingHospital, Peshawar from June 2010 till June 2012. Ultrasound guided percutaneous renal biopsies werecarried out in patients with the finding of; 1 ) Nephrotic range proteinuria in children. 2) Non-Nephroticrange proteinuria with evidence of hypertension / haematuria / deranged renal function or active sedimentson urine microscopy. 3) Steroid resistant nephrotic syndrome in children (patients not responding to steroidin eight weeks time) and 4) Children with nephrotic syndrome who were not tolerant of steroid therapy orwere considered for immunosuppressive drugs.RESULT: A total of 155 renal biopsies were done. Out of these 90 were male patients and 65 were females.The most common histopathological lesion among children population was minimal change disease(42.66%) followed by focal segmental glomerulosclerosis (25.33%) and membranous GN (16.0%). Weobserved that nephrotic range proteinuria was most prevalent in minimal change disease and membranousGN followed by focal segmental glomerulosclerosis. While non-nephrotic range proteinuria was mostlyseen in patients with membranoprolifirative GN.CONCLUSION: In paediatric population, minimal change disease is the most common encounteredglomerulopathy, followed by focal segmental glomerulosclerosisand membranous GN.KEY WORDS: Nephrotic syndrome, Renal biopsy, Proteinuria, Glomerulopathy

Discovery of Autoantibodies Targeting Nephrin in Minimal Change Disease Supports a Novel Autoimmune Etiology

2021 ◽  
pp. ASN.2021060794
Author(s):  
Andrew Watts ◽  
Keith Keller ◽  
Gabriel Lerner ◽  
Ivy Rosales ◽  
A. Collins ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Animal Studies ◽  
Minimal Change Disease ◽  
Molecular Classification ◽  
Minimal Change ◽  
Filtration Barrier ◽  
Steroid Dependent ◽  
Renal Biopsies ◽  
End Stage ◽  
Autoimmune Etiology

Background Failure of the glomerular filtration barrier, primarily by loss of slit diaphragm architecture, underlies nephrotic syndrome in minimal change disease. The etiology remains unknown. The efficacy of B cell-targeted therapies in some patients, together with the known proteinuric effect of antinephrin antibodies in rodent models, prompted us to hypothesize that nephrin autoantibodies may be present in patients with minimal change disease. Methods We evaluated sera from patients with minimal change disease enrolled in the Nephrotic Syndrome Study Network (NEPTUNE) cohort and from our own institutions for circulating nephrin autoantibodies by indirect ELISA and by immunoprecipitation of full-length nephrin from human glomerular extract or a recombinant purified extracellular domain of human nephrin. We also evaluated renal biopsies from our institutions for podocyte-associated punctate IgG colocalizing with nephrin by immunofluorescence Results In two independent patient cohorts, we identified in a subset of patients with minimal change disease circulating nephrin autoantibodies during active disease that were significantly reduced or absent during treatment response. We correlated the presence of these autoantibodies with podocyte-associated punctate IgG in renal biopsies from our institutions. We also identified a patient with steroid-dependent childhood minimal change disease that progressed to end-stage kidney disease; she developed a massive posttransplant recurrence of proteinuria that was associated with high pretransplant circulating nephrin autoantibodies. Conclusions Our discovery of nephrin autoantibodies in a subset of adults and children with minimal change disease aligns with published animal studies and provides further support for an autoimmune etiology. We propose a new molecular classification of nephrin autoantibody minimal change disease to serve as framework for instigation of precision therapeutics for these patients.

scholarly journals Histopathological audit of renal biopsy in Nepalese children: two center experience

2018 ◽  
Vol 8 (1) ◽  
pp. 1244-1250
Author(s):  
Devendra Shrestha ◽  
Ajaya Kumar Dhakal ◽  
Nabin Bahadur Basnet ◽  
Shiva Raj KC ◽  
Rishi Kumar Kafle
Keyword(s):  
Nephrotic Syndrome ◽  
Renal Biopsy ◽  
Iga Nephropathy ◽  
Minimal Change Disease ◽  
Minimal Change ◽  
Proliferative Glomerulonephritis ◽  
Histopathological Diagnosis ◽  
Gross Hematuria ◽  
Nepalese Children ◽  
Diffuse Proliferative Glomerulonephritis

Background: Renal biopsy is an invaluable tool which provides histopathological description of renal disease in terms of severity of lesion and helps in formulating the long term plan. However such studies with histopathological description of renal biopsy among Nepalese children are scarce.Material and Methods: This was a retrospective study conducted among all children aged less than 18 years who underwent renal biopsy at two tertiary centers in Nepal between July 2015 and December 2017.Results: All 72 renal biopsies were done with Bard 18G spring loaded gun. Nephrotic syndrome and mixed nephritic-nephrotic features were the commonest indications for renal biopsy. IgA nephropathy was the most frequent histopathological diagnosis (20.8%) which was mainly observed in children of age group 11-18 years followed by minimal change disease (16.7%) and diffuse proliferative glomerulonephritis (16.7%). Majority of children with recurrent gross hematuria (6/7) or mixed nephritic-nephrotic features (6/8) had IgA nephropathy. Focal segmental glomerulosclerosis (6/8) was commonest in children with steroid resistant nephrotic syndrome and nephrotic syndrome with atypical features. Majority of children (10/12) with diffuse proliferative glomerulonephritis had features of poststreptococcal glomerulonephritis and 6/12 had crescents in glomeruli. Lupus nephritis (13.9%) was the commonest secondary cause of glomerular pathology and was observed predominantly in 11-18 years age.Conclusion: Renal biopsy is safe and not associated with clinically significant complications. Nephrotic syndrome was the commonest indication for renal biopsy and minimal change disease predominated. IgA nephropathy was the commonest histological diagnosis overall, as well as in children who presented with recurrent gross hematuria or mixed nephritic-nephrotic features.

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

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