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Background Primary nephrotic syndrome (NS) is a common disease in children. Lipid nephrotoxicity and cellular immune dysfunction are known features of this disease. Recently, CXCL16 was found to participate in inflammation and mediate cellular uptake of lipids. Here, we investigated the involvement of CXCL16 in the occurrence and development of primary NS. Methods Serum CXCL16, blood lipids and albumin, 24-hour urine protein, interferon-γ and immune cells were detected in 25 children with steroid sensitive NS during their active nephrotic and remissive stages. Twenty healthy children served as the control group. Results Levels of serum CXCL16, blood lipids, interferon-γ and CXCR6+ T cells were significantly increased and albumin and NK cell number were significantly decreased in the active status group compared with remissive status and control groups. Correlation analysis showed that serum CXCL16 was positively correlated with blood lipids, 24-hour urine protein, interferon- γ and CXCR6+ T cells but negatively correlated with albumin in patients with active NS. Conclusion Serum CXCL16 was increased in patients with active NS and correlated with blood lipids, urine protein and immune and inflammation responses, suggesting that CXCL16 may serve as a useful index or biomarker for disease activity in children with nephrotic syndrome. Virtual slides The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1120468411154766. [ABSTRACT FROM AUTHOR]