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001-es BibID:BIBFORM061600
Első szerző:Fülöp Tibor (kardiológus)
Cím:Resolution of C1q deposition but not of the clinical nephrotic syndrome after immunomodulating therapy in focal sclerosis / Tibor Fülöp, Éva Csongrádi, Anna A. Lerant, Matthew Lewin, Jack R. Lewin
Dátum:2015
Megjegyzések:Background: The natural evolution of C1q nephropathy (C1qNP) during immunosuppressivetreatment is relatively little studied or understood.Case Presentation: A 30 year-old Caucasian female was referred to us for further managementof biopsy-proven C1qNP and severe nephrotic syndrome. Serologic work-up remainednegative, including complement C3 and C4 levels and repeated testing for antinuclearantibodies. A renal biopsy revealed minimal change nephropathy vs. focal sclerosis onlight microscopy and C1qNP on immunopathology. She has failed trials of high-dose oralprednisone, mycophenolate mofetil 1,500 mg twice a day and a subsequent regimen ofmonthly IV cyclophosphamide 750 mg ? 9 cycles. She also received the maximum toleratedangiotensin-converting enzyme inhibitor and spironolactone therapy. Random urine proteinto-creatinine (UPC) ratio predicted proteinuria in the range between 5-35 gm/day, whileserum creatinine rose progressively from 1.0 mg/dL to 1.4 mg/dL (to convert to ?mol/L,multiply by 88.4). A decision was made to repeat renal biopsy to reassess the underlyinghistology. The biopsy revealed focal sclerosis but no C1q deposition.Conclusions: Our case illustrates at least two points: first, an established pathologic diagnosisdoes not obviate the need for repeated renal biopsy later on, should diagnostic uncertaintypersist. Second, histological diagnoses may evolve over time, especially in a patient receivingactive and powerful immune-modulating treatment. In our case, the clinical nephrosis didnot change with immunosuppressive therapy while C1q deposition ceased, making this latterentity likely the immunologically mediated process.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
Megjelenés:Journal of Nephropathology. - 4 : 2 (2015), p. 54-58, -
További szerzők:Csongrádi Éva (1969-) (szakorvos) Lerant, Anna (orvos) Lewin, Matthew (orvos) Lewin, Jack R.
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