The paucity of minimal change disease in adolescents with primary nephrotic syndrome

1998 ◽  
Vol 12 (2) ◽  
pp. 105-107 ◽  
Author(s):  
Noosha Baqi ◽  
Anup Singh ◽  
Shivaiha Balachandra ◽  
Hadi Ahmad ◽  
Anthony Nicastri ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Minimal Change Disease ◽  
Minimal Change ◽  
Primary Nephrotic Syndrome

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.

MO1021HYPERTENSION IN CHILDHOOD MINIMAL CHANGE DISEASE: IGNORED, MISSED OR UNDER-DIAGNOSED?

2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Sourabh Sharma ◽  
Neha Sharma
Keyword(s):  
Body Mass Index ◽  
Nephrotic Syndrome ◽  
Family History ◽  
Body Mass ◽  
Minimal Change Disease ◽  
Angiotensin Receptor Blockers ◽  
Tertiary Care ◽  
Minimal Change ◽  
Primary Nephrotic Syndrome ◽  
Steroid Dependent

Abstract Background and Aims Minimal change disease (MCD) is most common form of primary nephrotic syndrome (76.6% as per ISKDC) in children aged 1 to 10 years. As per current literature, blood pressure (BP) is usually normal in childhood MCD while in adults, 50% MCD cases are hypertensive. We hypothesized that hypertension is prevalent in childhood MCD also and most of cases are missed or under-diagnosed due to lack of BP monitoring, inappropriate BP cuff size (larger cuff will inaccurately show lower BP; cuff should cover 75% to 80% of arm circumference between antecubital fossa and axilla) or lack of knowledge about childhood hypertension criteria (systolic or diastolic BP> 95th percentile for age on ≥2 clinical visits at least 1 week apart). Method This cross-sectional study was performed from March 2018 to December 2020 at a tertiary care centre in India. Newly diagnosed and relapses of primary nephrotic syndrome (presumed MCD) in children aged 1 to 10 years were included in the study. All cases were subjected to careful BP monitoring and charting using appropriate BP cuff, while child is calm and relaxed, in quiet room with comfortable temperature and empty bladder. Three measurements were taken at 1 minute interals and taking average of last 2 measurements. Percentile charts for age and height were followed. Results fifty four cases of childhood MCD were studied. Thirty six cases (66.67%) were male. Mean age was 5.48 years (SD ±2.31). Thirty two cases (59.26%) were found to be hypertensive. Fourteen cases (43.75%) were relapses. Seven cases (21.87%) were steroid resistant nephrotic syndrome while three cases were steroid dependent. Five cases were already on calcineurin inhibitors when diagnosed of hypertension. There was family history of hypertension in 9 cases diagnosed of hypertension (p value 0.77). There was no correlation between occurrence of hypertension and degree of proteinuria, hypoalbuminemia or body mass index. All hypertensives were managed with angiotensin receptor blockers and regularly followed-up. Conclusion Results of our study are contrary to traditional belief that hypertension is not common in childhood MCD. Significant number of cases are hypertensive. Correct BP measurement and interpretation is necessary in each childhood MCD case. Its occurrence is not correlated with family history or degree of proteinuria, hypoalbuminemia or body mass index.

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 Minimal Change Disease as a Secondary and Reversible Event of a Renal Transplant Case with Systemic Lupus Erythematosus

2015 ◽  
Vol 2015 ◽  
pp. 1-6 ◽  
Author(s):  
Elena Gkrouzman ◽  
Kyriakos A. Kirou ◽  
Surya V. Seshan ◽  
James M. Chevalier
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Nephrotic Syndrome ◽  
Renal Transplant ◽  
Transplant Recipient ◽  
Lupus Erythematosus ◽  
Minimal Change Disease ◽  
Iliac Vein ◽  
Minimal Change ◽  
Systemic Lupus ◽  
Vein Stenosis

Secondary causes of minimal change disease (MCD) account for a minority of cases compared to its primary or idiopathic form and provide ground for consideration of common mechanisms of pathogenesis. In this paper we report a case of a 27-year-old Latina woman, a renal transplant recipient with systemic lupus erythematosus (SLE), who developed nephrotic range proteinuria 6 months after transplantation. The patient had recurrent acute renal failure and multiple biopsies were consistent with MCD. However, she lacked any other features of the typical nephrotic syndrome. An angiogram revealed a right external iliac vein stenosis in the region of renal vein anastomosis, which when restored resulted in normalization of creatinine and relief from proteinuria. We report a rare case of MCD developing secondary to iliac vein stenosis in a renal transplant recipient with SLE. Additionally we suggest that, in the event of biopsy-proven MCD presenting as an atypical nephrotic syndrome, alternative or secondary, potentially reversible, causes should be considered and explored.

Short-Term Steroid Regimen for Adult Steroid-Sensitive Minimal Change Disease

2018 ◽  
Vol 49 (1) ◽  
pp. 54-63 ◽  
Author(s):  
Takaya Ozeki ◽  
Takayuki Katsuno ◽  
Hiroki Hayashi ◽  
Sawako Kato ◽  
Yoshinari Yasuda ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Minimal Change Disease ◽  
Minimal Change ◽  
First Episode ◽  
Short Term ◽  
Short Term Treatment ◽  
Steroid Dose ◽  
Steroid Sensitive ◽  
Steroid Regimen ◽  
Frequent Relapse

Background: In pediatric patients with steroid-sensitive nephrotic syndrome, recent trials have revealed that a 2-month, short-term steroid regimen is not inferior to an extended steroid course. However, the optimal duration of initial steroid therapy for adult steroid-sensitive minimal change disease (MCD) remains unclear. Objectives: The aim of present study was to evaluate the effectiveness of a 2-month, short-term steroid regimen in the treatment of adult steroid-sensitive MCD patients. Method: This was a prospective observational study. Adult patients with steroid-sensitive MCD (n = 35) who were initiated on a short-term steroid regimen between January 2015 and June 2016 were included. The details of the regimen are as follows: (1) prednisolone was administered at an initial dose of 0.8–1.0 mg/kg/day and continued for 4–6 weeks and (2) dosage was reduced to 0.5–0.6 mg/kg/alternate day and continued for 4 weeks. Control patients (n = 140), who were treated using conventional steroid administration, were selected from our previous adult MCD cohort. All patients fulfilled the following criteria: biopsy-proven MCD, age ≥20 years, first episode of nephrotic syndrome, and attainment of complete remission within 4 weeks. The following parameters of patients who received short-term treatment regimen and control patients were compared: any relapse and frequent relapse, adverse events caused by steroid treatment and cumulative steroid dose. Results: Throughout the observation period (median: 17.3 months), 24 (68.6%) patients in the short-term group developed at least one relapse. The short-term regimen showed earlier occurrence of any relapse than the conventional regimen (adjusted hazard ratio [aHR] 2.45; 95% CI 1.51–3.97; p < 0.001), but there was no difference in frequent relapse (aHR 1.31; 95% CI 0.43–3.99; p = 0.63). None of the patients showed any symptoms of adrenal insufficiency after discontinuation of corticosteroids. The cumulative steroid dose during the observational period was significantly lower in the short-term group than in the conventional group. Conclusions: The short-term steroid regimen may represent an effective treatment option that ensures lower steroid exposure when treating adult steroid-sensitive MCD patients.

scholarly journals Clinical Characteristics and Outcomes of Adults with Nephrotic Syndrome Due to Minimal Change Disease

2021 ◽  
Vol 10 (16) ◽  
pp. 3632
Author(s):  
Sophia Lionaki ◽  
Evangelos Mantios ◽  
Ioanna Tsoumbou ◽  
Smaragdi Marinaki ◽  
George Makris ◽  
...  
Keyword(s):  
Acute Kidney Injury ◽  
Nephrotic Syndrome ◽  
Minimal Change Disease ◽  
Kidney Injury ◽  
Spontaneous Remission ◽  
Minimal Change ◽  
Adult Onset ◽  
Potential Risk Factors ◽  
The Mean ◽  
End Stage

Purpose: Minimal change disease (MCD) is considered a relatively benign glomerulopathy, as it rarely progresses to end-stage kidney disease. The aim of this study was to describe the characteristics and outcomes of adults with MCD and identify potential risk factors for relapse. Patients & Methods: We retrospectively studied a cohort of adults with biopsy-proven MCD in terms of clinical features and treatment outcomes. Baseline characteristics and outcomes were recorded and predictors of relapse were analyzed using logistic regression multivariate analysis. Results: 59 patients with adult-onset primary MCD with nephrotic syndrome were included. Mean serum creatinine at diagnosis was 0.8 mg/dL (±2.5) and estimated GFR (eGFR) was 87 mL/min/1.73 m2 (±29.5). Mean serum albumin was 2.5 g/dL (±0.8) and 24 h proteinuria 6.8 g (±3.7). Microscopic hematuria was detected in 35 (58.5%) patients. 42 patients received prednisone alone, six patients received prednisone plus cyclophosphamide, five patients received prednisone plus cyclosporine, one patient received prednisone plus rituximab and five patients did not receive immunosuppression at all since they achieved spontaneous remission. During a mean follow up time of 34.7(22.1) months, 46.1% of patients experienced at least one episode of relapse. The mean age of patients who did not experience a relapse was significantly higher than that of patients who relapsed while relapsers had a significantly longer duration of 24 h proteinuria prior to biopsy compared to non-relapsers. Overall, 10% of patients experienced acute kidney injury while the mean eGFR at the end was 82 mL/min/1.73 m2 (±29.1) and one patient ended up in chronic dialysis. Overall, the proportion of non-relapsers, who experienced acute kidney injury (17%) was significantly higher than the one recorded among relapsers (0%).Conclusion: In this series of patients, almost 46% of adult-onset nephrotic MCD patients experienced a relapse, although their renal progression was rare. Younger onset age was an independent risk factor for relapse in adult-onset MCD patients.

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.

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