Mycophenolate mofetil treatment for primary glomerular diseases
Michael ChoiJoseph A. EustaceLuis F. GimenezMohamed G. AttaPaul J. ScheelRenuka SothinathanWilliam A. Briggs
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Mycophenolate
Minimal change disease
Minimal change disease
Glomerulosclerosis
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Mycophenolate mofetil has been reported to be effective in the treatment of steroid-responsive minimal change disease. We report a case of steroid- and cyclosporin-resistant minimal change disease with severe nephrotic syndrome that responded to mycophenolate mofetil with complete remission. The patient remains in complete remission 1 year after the discontinuation of mycophenolate mofetil. The presented case argues for the need of a prospective controlled study with the goal of evaluating the efficacy of mycophenolate mofetil in the therapy of steroid-resistant minimal change disease.
Mycophenolate
Minimal change disease
Discontinuation
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Abstract Background: Although minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS) have been described as two separate forms of nephrotic syndrome(NS), they are not completely independent. We report a patient presenting a transition from MCD to FSGS, review the literature and explore the relationship between the two diseases. Case presentation: A 42-year-old male welder, Asian, presenting lower extremity edema and elevated serum creatinine, had laboratory exams indicating NS and end-stage renal disease(ESRD). The patient had a kidney biopsy 20 years earlier for NS, which indicated MCD, and this repeated kidney biopsy suggested FSGS. After treatment follow-up, the patient was eventually admitted to renal replacement therapy. Conclusions: MCD and FSGS may be different stages of the same disease. The transition from MCD to FSGS in this case indicates the progression of the disease, which may be related to the excessive metal caused by occupation.
Minimal change disease
Glomerulosclerosis
Nephrosis
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Treatment of nephrotic syndrome due to minimal change disease and focal segmental glomerulosclerosis remains a challenge since steroid dependence, steroid resistance and a relapsing disease course exhibits a high cumulative steroid dosage. The necessity of using alternative steroid-sparing immunosuppressive agents with potential toxic side effects also restricts their long-term use. Rituximab, a monoclonal antibody targeting CD20, has been increasingly used in the therapy of difficult-to-treat nephrotic syndrome. A clinical response has been shown for patients with steroid-dependent or frequently relapsing nephrotic syndrome, whereas the benefit seems to be limited in steroid-resistant patients, especially those with underlying focal segmental glomerulosclerosis. No potentially life-threatening adverse events have been observed in the treatment of adult minimal change disease and focal segmental glomerulosclerosis following rituximab administration. Since most reports are retrospective and evidence of efficacy is derived from small case series, more prospective trials in a controlled, randomized manner are highly desirable to delineate the use of rituximab or other B cell-depleting agents in steroid-dependent, frequently relapsing or steroid-resistant patients.
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To specify clinical and laboratory characteristics of minimal change disease (MCD), focal mesangioproliferative glomerulonephritis (MPGN), focal-segmental glomerulosclerosis (FSGS).A retrospective analysis of 45 case histories of children (renal biopsy for nephrotic syndrome) has shown that morphologically 13 of them had MCD, 15--FSGS and 17--focal MPGN. Clinical, laboratory, immunofluorescent and electron microscopy findings typical for each of the morphological types were studied.The data obtained suggest that MCD, FSGS and focal MPGN may represent independent forms of glomerulonephritis.If a differential diagnosis by renal biopsy is difficult, information from the disease history and clinico-laboratory evidence should be used.
Minimal change disease
Glomerulosclerosis
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Minimal change disease (MCD) and focal and segmental glomerulosclerosis (FSGS) are often studied together, because both present with heavy proteinuria and the nephrotic syndrome. The precise distinction between MCD and FSGS is sometimes difficult because of inadequate number of glomeruli for definite diagnosis. Some evidence suggests that markers of lipid peroxidation, such as malondialdehyde (MDA) is an index of free radical mediated injury and may be involved in the pathogenesis of FSGS. In this study, we assessed the immunoreactivity of MDA, the end product of lipid peroxidation in glomeruli of patients with idiopathic FSGS, MCD as well as normal controls (NC). Our results showed that the immunostaining level of MDA was significantly higher in patients with FSGS (mean = 1.5) than in either patients with MCD (mean = 0.16) or normal controls (mean = 0.11) with P value < 0.001. Glomerular MDA level correlated well with the degree of glomerulosclerosis in patients with idiopathic FSGS. Our data demonstrates that the glomerular level of MDA is higher in idiopathic FSGS than MCD. We suggest that MDA immunostaining can be helpful in differentiating between FSGS and MCD in problematic cases and when we do not have enough glomeruli for definite and correct diagnosis.
Minimal change disease
Glomerulosclerosis
Malondialdehyde
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Mycophenolate
Minimal change disease
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Minimal change disease
Immunosuppression
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