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CC BY-NC-ND 4.0 · Ann Natl Acad Med Sci 2018; 54(01): 043-053
DOI: 10.1055/s-0040-1712821
Original Article

Therapies for Glomerular Diseases in Children

Arvind Bagga
Department of Pediatrics, Division of Nephrology, All India Institute of Medical Sciences, New Delhi
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Abstract

Nephrotic syndrome is an important chronic disease of childhood, with a steroid sensitive course in most patients. Research on pathogenesis has emphasized the importance of T-lymphocyte dysregulation and vascular permeability factors that alter podocyte function and glomerular permselectivity. Mutations in genes that encode important podocyte proteins and therapeutic targets within podocytes have been identified. A hypothesis unifying available evidence on pathogenesis is yet to be proposed. An important proportion of patients have difficult disease course, characterized by frequent relapses, steroid dependence or steroid resistance, requiring therapy with alternative immunosuppressive agents. Clinical studies support the use of levamisole, cyclophosphamide, mycophenolate mofetil, calcineurin inhibitors (CNIs) and rituximab in patients with frequent relapses or steroid dependence. The management of steroid-resistant nephrotic syndrome is difficult and patients failing to achieve remission show progressive renal damage. Prospective studies in patients with steroid sensitive and steroid resistant nephrotic syndrome are the basis of current guidelines while ongoing studies will help identify and formulate effective and safe therapies.

Keywords

Calcineurin inhibitors - focal segmental glomerulosclerosis - minimal change disease - Rituximab


Publication History

Article published online:
08 May 2020

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