OBJECTIVE To analyze the use of Cyclosporine (CyA) in nephrotic syndrome. METHODS Thirty five children of mean age of 5.9 years with steroid dependent (n = 26) or steroid resistant (n = 9) primary nephrotic syndrome with normal renal functions and who received CyA were studied. CyA was used at a dosage of 6-7 mg/kg/day orally in two divided doses. The mean duration of therapy was 9.6 weeks. A...

Nephrotic syndrome is an important chronic disease in children, characterized by minimal change disease in the majority. Research on pathogenesis has emphasized the importance of T lymphocyte dysregulation and vascular permeability factors that might alter podocyte function and permselectivity. While mutations in genes that encode important podocyte proteins have also been identified, a hypothe...

A 27-year-old Japanese female developed nephrotic syndrome and impaired renal function during pregnancy. A renal biopsy performed at 21 weeks of gestation revealed crescentic IgA nephropathy. She was treated with steroid pulse therapy followed by conventional steroid therapy. Although the nephrotic syndrome was persistent, the impaired renal function did not deteriorate after the treatment. A l...

Advances in podocytology and genetic techniques have expanded our understanding of the pathogenesis of hereditary steroid-resistant nephrotic syndrome (SRNS). In the past 20 years, over 45 genetic mutations have been identified in patients with hereditary SRNS. Genetic mutations on structural and functional molecules in podocytes can lead to serious injury in the podocytes themselves and in adj...

BACKGROUND Nephrotic patients are prone to atherosclerosis in consequence of frequent exposures to hyperlipidemia, hypertension, and immunosuppressive drugs. OBJECTIVES We studied the carotid parameters as early indicators of atherosclerosis in children with nephrotic syndrome. METHODS Between 2008 and 2011, 51 children with history of nephrotic syndrome enrolled in the study. The inclusion...

Online first: March 14, 2017 SUMMARY Introduction Congenital nephrotic syndrome (CNF) is manifested at birth or within the first three months of life. The Finnish-type of CNF is caused by the mutation of the NPHS1 gene, which encodes nephrin in the podocyte slit diaphragm. It is a very severe disease, for which immunosuppressive therapy is not advised. Here we describe a patient with CNF who re...

Childhood nephrotic syndromes are most commonly caused by one of two idiopathic diseases: minimal-change nephrotic syndrome (MCNS) and focal segmental glomerulosclerosis (FSGS). A third distinct type, membranous nephropathy, is rare in children. Other causes of isolated nephrotic syndrome can be subdivided into two major categories: rare genetic disorders, and secondary diseases associated with...

BACKGROUND The deliberate use of ketoconazole to reduce the need for cyclosporine (CsA) is not new, but it is particularly relevant because of the high cost of CsA. Many studies have documented this benefit in renal and cardiac transplants, but this co-administration has not been reported in patients with nephrotic syndrome. METHODS This retrospective study included 207 nephrotic patients who...

Background: Nephrotic syndrome (NS) is a common glomerular disease in children, for which there are currently no noninvasive markers predicting responsiveness to steroid treatment. Objectives: This study aimed analyze the relationship between clinical-laboratory presentations and subsequent response therapy evaluate role of urinary vitamin D binding protein (uVDBP) as predictor resistance (SR)....