Ester Cholbi Vives, Javier Martín Benlloch, Josselyn Hernández Chinchilla, Vicent Martínez Cózar, Judith Pérez Rojas, Pedro Ortega López
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引用次数: 0
Abstract
A 12-year-old girl presented with proteinuria and peripheral edema. Initial evaluation did not identify a clear underlying etiology. Upon the diagnosis of nephrotic syndrome, corticosteroid therapy was initiated. However, the therapeutic response was suboptimal, with persistent proteinuria, the onset of de novo microhematuria, and progressive kidney dysfunction. A kidney biopsy was performed, which led to the diagnosis of fibrillary glomerulopathy, a rare pediatric condition characterized histologically by the presence of fibrillary deposits and positive immunostaining for DNAJB9. There is no established effective treatment for this condition. In our case, the patient was treated with antiproteinuric and calcineurin inhibitors, resulting in complete resolution of microhematuria, normalization of kidney function, and substantial improvement in proteinuria.
期刊介绍:
International Pediatric Nephrology Association
Pediatric Nephrology publishes original clinical research related to acute and chronic diseases that affect renal function, blood pressure, and fluid and electrolyte disorders in children. Studies may involve medical, surgical, nutritional, physiologic, biochemical, genetic, pathologic or immunologic aspects of disease, imaging techniques or consequences of acute or chronic kidney disease. There are 12 issues per year that contain Editorial Commentaries, Reviews, Educational Reviews, Original Articles, Brief Reports, Rapid Communications, Clinical Quizzes, and Letters to the Editors.