A case of systemic karyomegaly associated with interstitial nephritis

A case of systemic karyomegaly is described for the first time in Greece. This rare disease entity was first described in 1979 by Mihatsh and so far only nine cases have been reported. Typical clinical features are progressive renal failure in the third decade of life and recurrent infections, mostly of the upper respiratory tract. Typical histologic findings are markedly enlarged and hyperchromic nuclei in tubular cells of the nephron and interstitial fibrosis surrounding the atrophic tubules.