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Karyomegalic tubulointerstitial nephritis with primary focal segmental glomerulosclerosis in a young female: A rare form of steroid-nonresponsive nephrotic syndrome

Karyomegalic interstitial nephropathy (KIN) is a rare form of chronic tubulointerstitial nephritis initially described as a familial nephropathy in adults. We present a case of KIN with focal segmental glomerulosclerosis in a 15-year-old young female who became late nonresponder and resistant to steroid therapy. To our knowledge, only one case has been reported in literature.