Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2024 Mar-Apr;34(2):189-190.
doi: 10.4103/ijn.ijn_364_22. Epub 2023 Aug 4.

Karyomegalic Interstitial Nephritis in the Allograft Kidney - A Case Report

Jansi Prema K S  1 , Mahesh R K  2 , Anila A Kurien  1
Affiliations
Case Reports

Karyomegalic Interstitial Nephritis in the Allograft Kidney - A Case Report

Jansi Prema K S et al. Indian J Nephrol. 2024 Mar-Apr.

Abstract

Karyomegalic interstitial nephritis is a rare progressive renal disease. We report a 36-year-old male patient who developed kidney failure due to this condition, underwent kidney transplantation from his sister, and developed the same condition in the graft. Genetic testing of the donor revealed autosomal recessive compound heterozygous mutation of Fanconi anemia-associated nuclease1 (FAN1) gene. Karyomegalic interstitial nephritis is most probably donor derived in our patient. It should not be mislabeled as viral nephropathy.

Keywords: Immunohistochemistry; interstitial nephritis; karyomegaly; kidney biopsy.

PubMed Disclaimer

Conflict of interest statement

There are no conflicts of interest.

Figures

Figure 1:
Figure 1:
(a and b) Native kidney biopsy. KIN has focal enlarged tubular epithelial cell nuclei. There is interstitial inflammation and fibrosis along with tubular atrophy (hematoxylin and eosin stain in (a) 20×, in (b) 40×). (c and d) Allograft biopsy. Acute cell-mediated rejection with interstitial inflammation and tubulitis is also seen. Some of the tubular epithelial cells have enlarged nuclei. The biopsy shows large, pleomorphic, and hyperchromatic tubular epithelial cell nuclei with tubular epithelial cell injury (hematoxylin and eosin stain in (c) 20×, in (d) 40×). KIN = karyomegalic interstitial nephritis.
See this image and copyright information in PMC

References

    1. Isnard P, Rabant M, Labaye J, Antignac C, Knebelmann B, Zaidan M. Karyomegalic interstitial nephritis. Medicine (Baltimore) 2016;95:3349. doi: 10.1097/MD.0000000000003349. doi: 10.1097/MD.0000000000003349. - DOI - PMC - PubMed
    1. Burry AF. Extreme dysplasla in renal epithelium of a young woman dying from hepatocarcinoma. J Pathol. 1974;113:147–50. doi: 10.1002/path.1711130303. - DOI - PubMed
    1. Mihatsch MJ, Gudat F, Zollinger HU, Heierli C, Thölen H, Reutter FW. Systemic karyomegaly associated with chronic interstitial nephritis. A new disease entity? Clin Nephrol. 1979;12:54–62. - PubMed
    1. Ravindran A, Cortese C, Larsen CP, Wadei HM, Gandhi MJ, Cosio FG, et al. Karyomegalic interstitial nephritis in a renal allograft. Am J Transplant. 2019;19:285–90. doi: 10.1111/ajt.15035. - DOI - PubMed
    1. Zhou W, Otto EA, Cluckey A, Airik R, Hurd TW, Chaki M, et al. FAN1 mutations cause karyomegalic interstitial nephritis, linking chronic kidney failure to defective DNA damage repair. Nat Genet. 2012;44:910–5. doi: 10.1038/ng.2347. - DOI - PMC - PubMed

Publication types

LinkOut - more resources