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Pulmonary manifestations of systemic karyomegaly.

Journal article
Authors Levent Akyürek
Aziz Hussein
Andrew G Nicholson
Nils-Johan Mauritz
Johan Mölne
Published in Respiratory Medicine Case Reports
Volume 29
Pages 101032
ISSN 2213-0071
Publication year 2020
Published at
Pages 101032
Language en
Subject categories Respiratory Medicine and Allergy, Clinical Laboratory Medicine


Over 40 years ago, abnormal enlargement of the nucleus of tubular epithelial cells was reported in a rare distinct hereditary chronic interstitial nephritis, karyomegalic interstitial nephritis (KIN). Here, we report the second case of systemic karyomegaly with pulmonary manifestations and present a detailed characterization of the karyomegalic cells in lung parenchyma. A 59-year-old woman who was diagnosed with KIN developed renal failure and eventually received a renal transplant later evaluated for chronic and progressive restrictive lung disease. The KIN diagnosis prompted us to carefully examine her lung parenchyma. Karyomegalic cells were identified in the alveolar epithelium, interstitium, as well as, in the vascular wall. Viral serological and biochemical blood analyses were negative. We consider that the pulmonary manifestations of karyomegaly expands the differential diagnosis of interstitial lung disease in patients with KIN.

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Utskriftsdatum: 2020-08-14