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