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Chronic Cyclosporine Toxicity in Pediatric Nephrotic Syndrome

Pediatric Nephrotic Syndrome, childhood nephrotic syndrome, CSA, CNI, juxtaglomerular apparatus
This biopsy is from a 9-year-old boy who developed pediatric nephrotic syndrome in the first year of life and has been maintained on cyclosporine (CSA) due to frequent relapses. He had not had a biopsy before and was clinically on remission, considering switching to rituximab. The biopsy was performed to assess chronic changes and the presence of histologic signs of chronic calcineurin inhibitor (CNI) toxicity CNI toxicity. His serum creatinine was 0.6 mg/dL. He was also on lisinopril for proteinuria control. He was not hypertensive. The biopsy contained a total of 61 glomeruli, four of which were globally sclerotic. No...

Juxtaglomerular Apparatus Hyperplasia

The arrow in this image points to hyperplasia of the juxtaglomerular apparatus (JGA), which was seen diffusely in this biopsy. The JGA consists of vascular components (portions of the afferent and efferent arterioles), mesangial cell components (modified extraglomerular and intraglomerular smooth muscle cells), and a tubular component (the macula densa). The differential diagnosis for JGA hyperplasia includes causes of chronic renal ischemia (e.g. renal artery stenosis or cardiac failure), various drugs (e.g. angiotensin II receptor antagonists and cyclosporine), and Bartter syndrome. This biopsy was taken from a child who had been diagnosed with Bartter syndrome approximately two years earlier –...