The Clinical Course of Minimal Change Nephrotic Syndrome With Onset in Adulthood or Late Adolescence: A Case Series

Maas, Rutger; Deegens, Jeroen K. J.; Beukhof, J. R.; Reichert, L.J.M.; Dam, Marc A. ten; Beutler, Jaap J.; Bake, A. W. L. Van Den Wall; Rensma, Pieter L.; Konings, Constantijn; Geerse, Daniël A.; Feith, Geert W.; Kuijk, Willi H. Van; Wetzels, Jack F.M.

doi:10.1053/j.ajkd.2016.10.032

Cited by 47 publications

(56 citation statements)

References 32 publications

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“…Comparable with the results of the present cohort study, previous retrospective cohort studies on MCD reported that infection was one of the most common adverse events [23][24][25][26][27] and one of the leading causes of mortality [28]. To suppress the incidence of infection, a lower dose of and/or shorter term immunosuppressive therapy is desirable.…”

Section: Discussionsupporting

confidence: 79%

Incidence of remission and relapse of proteinuria, end-stage kidney disease, mortality, and major outcomes in primary nephrotic syndrome: the Japan Nephrotic Syndrome Cohort Study (JNSCS)

et al. 2020

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Background Despite recent advances in immunosuppressive therapy for patients with primary nephrotic syndrome, its effectiveness and safety have not been fully studied in recent nationwide real-world clinical data in Japan. Methods A 5-year cohort study, the Japan Nephrotic Syndrome Cohort Study, enrolled 374 patients with primary nephrotic syndrome in 55 hospitals in Japan, including 155, 148, 38, and 33 patients with minimal change disease (MCD), membranous nephropathy (MN), focal segmental glomerulosclerosis (FSGS), and other glomerulonephritides, respectively. The incidence rates of remission and relapse of proteinuria, 50% and 100% increases in serum creatinine, end-stage kidney disease (ESKD), all-cause mortality, and other major adverse outcomes were compared among glomerulonephritides using the Log-rank test. Incidence of hospitalization for infection, the most common cause of mortality, was compared using a multivariable-adjusted Cox proportional hazard model. Results Immunosuppressive therapy was administered in 339 (90.6%) patients. The cumulative probabilities of complete remission within 3 years of the baseline visit was ≥ 0.75 in patients with MCD, MN, and FSGS (0.95, 0.77, and 0.79, respectively). Diabetes was the most common adverse events associated with immunosuppressive therapy (incidence rate, 71.0 per 1000 person-years). All-cause mortality (15.6 per 1000 person-years), mainly infection-related mortality (47.8%), was more common than ESKD (8.9 per 1000 person-years), especially in patients with MCD and MN. MCD was significantly associated with hospitalization for infection than MN. Conclusions Patients with MCD and MN had a higher mortality, especially infection-related mortality, than ESKD. Nephrologists should pay more attention to infections in patients with primary nephrotic syndrome.

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Section: Discussionsupporting

confidence: 79%

Incidence of remission and relapse of proteinuria, end-stage kidney disease, mortality, and major outcomes in primary nephrotic syndrome: the Japan Nephrotic Syndrome Cohort Study (JNSCS)

et al. 2020

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“…In this study, the most frequent reasons why patients with MCD or FSGS were rebiopsied were therapy-resistance or corticosteroid-dependent/frequently relapsing diseases. As reaffirmed by a recent study on the clinical course of MCD with onset in adulthood, 6 a missed diagnosis of FSGS is often suggested to explain corticosteroid resistance, which occurred for 6 patients in the OLD cohort and 7 in the CureGN study. Conversely, assessing whether worsening proteinuria and/or renal function were driven by active disease or the result of a chronic process was the primary reason for second biopsies of patients with MN and IgAN.…”

Section: Clinical Researchmentioning

confidence: 79%

“…Incidence rates of primary glomerular diseases have been well-documented, and long-term clinical outcomes for these conditions have been reported. [6][7][8][9][10] Nevertheless, the literature is comparably bare in reporting persistence of disease activity many years after clinical onset. Our study presents a unique approach to this population by analyzing patients in terms of disease activity rather than their chronic kidney disease or end-stage kidney disease status.…”

Section: Discussionmentioning

confidence: 99%

Persistent Disease Activity in Patients With Long-Standing Glomerular Disease

Delbarba

Marasà

Canetta

et al. 2020

Kidney International Reports

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Introduction: Glomerular diseases are characterized by variable disease activity over many years. We aimed to analyze the relationship between clinical disease activity and duration of glomerular disease. Methods: Disease activity in adults with chronic minimal change disease, focal segmental glomerulosclerosis, membranous nephropathy, and IgA nephropathy (IgAN; first diagnostic biopsy >5 years before enrollment; Of Longstanding Disease [OLD] cohort, n ¼ 256) followed at Columbia University Medical Center (CUMC), was compared with disease activity of an internal and external cohort of patients with first diagnostic biopsy <5 years before enrollment drawn from the Cure Glomerulonephropathy Network (CureGN cohort, n ¼ 1182; CUMC-CureGN cohort, n ¼ 362). Disease activity was defined by (i) Kidney Disease: Improving Global Outcomes-recommended threshold criteria for initiation of immunosuppression in primary glomerulonephropathy (GN) and (ii) CureGN's Disease Activity Working Group definitions for activity. Results: No significant differences were detected among the 3 cohorts in terms of age, sex, serum creatinine, and urinary protein-to-creatinine ratio. For each GN subtype, disease activity in the OLD cohort was comparable with disease activity in the entire CureGN and the CUMC-CureGN cohort. When limiting our comparisons to disease activity in incident CUMC-CureGN patients (first diagnostic biopsy within 6 months of enrollment), OLD patients demonstrated similar activity rates as incident patients. Conclusion: Disease activity did not differ among patients with shorter versus longer duration of disease. Such survivor patients, with long-term but persistent disease, are potentially highly informative for understanding the clinical course and pathogenesis of GN and may help identify factors mediating more chronic subtypes of disease.

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“…Although some case reports showed TLV was effective in patients with nephrotic syndrome 7,8 , it has not been fully elucidated which nephrotic patients are responsive to TLV. Also, nephrotic patients sometimes have renal impairment 9,10 , which is one of the major risk factors for resistance to TLV 11 . Thus, it is required to evaluate whether nephrotic syndrome is responsive to TLV and whether the response to TLV can be predicted by urine, blood or pathological test.…”

Section: Introductionmentioning

confidence: 99%

Efficacy of tolvaptan on nephrotic patients with diuretic-resistant edema: a pilot study

Matsuura

Ohtake

Mochida

et al. 2020

Preprint

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Background Tolvaptan (TLV), a vasopressin type 2 receptor antagonist, is an effective drug for heart failure without worsening renal function. However, the effect of TLV on nephrotic syndrome remains unknown. This study aims to assess the association between response to TLV and clinical parameters including pathological evaluation in nephrotic patients with diuretics-resistance.Methods For prospectively enrolled patients, TLV was added for 14 days after renal biopsy performed. We evaluated the effect of TLV on urine output (UO) and body weight (BW) and the correlation of UO change with BW, urine and blood measurements and pathological evaluation. Pathological evaluation included glomerular sclerosis, tubulointerstitial injury, interstitial fibrosis and the degree of aquaporin 2 (AQP2) positivity in collecting ducts.Results Ten nephrotic patients were enrolled. Two patients already received 140 and 160mg furosemide and others 40mg furosemide before TLV. After TLV administration, no patients showed worsening renal function or hypernatremia (>145 mEq/L) and two patients without any improvement of symptoms discontinued the trial. TLV significantly increased UO at day 1 and decreased BW overall. UO increase at day 1 was significantly correlated with BW decrease at the last follow-up. UO increase at day 1 was correlated only with AQP2 positivity in collecting ducts on biopsied kidney (r2=0.59, p=0.01) while other urine and blood measurements were not. Conclusions TLV was effective on nephrotic patients. UO increase by TLV was correlated with AQP2 positivity in the collecting ducts while biochemistry in urine and blood was not.

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The Clinical Course of Minimal Change Nephrotic Syndrome With Onset in Adulthood or Late Adolescence: A Case Series

Cited by 47 publications

References 32 publications

Incidence of remission and relapse of proteinuria, end-stage kidney disease, mortality, and major outcomes in primary nephrotic syndrome: the Japan Nephrotic Syndrome Cohort Study (JNSCS)

Incidence of remission and relapse of proteinuria, end-stage kidney disease, mortality, and major outcomes in primary nephrotic syndrome: the Japan Nephrotic Syndrome Cohort Study (JNSCS)

Persistent Disease Activity in Patients With Long-Standing Glomerular Disease

Efficacy of tolvaptan on nephrotic patients with diuretic-resistant edema: a pilot study

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