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Title: | Karyomegalic nephropathy: An uncommon cause of progressive renal failure. | Authors: | Kerr P. ;Collett P.;Cooke B.E.;Newland R.;Dowling J.;Horvath J.;Kalowski S.;Bhandari S. | Institution: | (Bhandari, Kalowski, Collett, Horvath) Statewide Renal Services, Royal Prince Alfred and Concord Repatriation Hospitals, Australia (Newland) Department of Anatomical Pathology, Royal Prince Alfred and Concord Repatriation Hospitals, Canada (Cooke) Department Anatomical Pathology, Prince of Wales Hospital, Sydney, NSW, Australia (Kerr) Department of Pathology, Alfred Hospital, Australia (Dowling) Monash Medical Centre, Melbourne, Vic., Australia (Bhandari) Hull and East Yorkshire Hospitals NHS Trust, Department of Renal Medicine, Hull Royal Infirmary, East Yorkshire HU3 2JZ, United Kingdom | Issue Date: | 19-Nov-2002 | Copyright year: | 2002 | Publisher: | Oxford University Press Oxford University Press (Great Clarendon Street, Oxford OX2 6DP, United Kingdom) |
Place of publication: | United Kingdom | Publication information: | Nephrology Dialysis Transplantation. 17 (11) (pp 1914-1920), 2002. Date of Publication: 01 Nov 2002. | Journal: | Nephrology Dialysis Transplantation | Abstract: | Background. Karyomegalic nephropathy, first identified in 1974, represents an increasingly recognized, but perhaps underdiagnosed condition associated with interstitial nephritis. It undoubtedly leads to end-stage renal disease requiring renal support. Methods and results. We present a series of six cases of karyomegalic nephropathy. The age at diagnosis was 9-51 years, median 33 years. Impaired renal function, proteinuria, and haematuria were present in the majority of cases. Non-specific elevated liver enzymes were present in three cases. Two patients died, soon after transplantation from overwhelming respiratory sepsis. The classical histological features of large, abnormal hyperchromatic nuclei with irregular outlines within epithelial cells were present in renal epithelial cells. Abnormality of DNA ploidy distributions compared with age- and sex-matched controls, and characterized by the presence of significant numbers of cells with high ploidy values was present in cases but not in controls. Mitotic figures were absent. Proliferation markers, Ki-67 and proliferating cell nuclear antigen/cyclin were not significantly elevated in those cases examined. Human leukocyte antigen analysis did not support the clustering of A9 or B35, in the cases or their families. Conclusions. The presence of significant renal impairment, positive urine sediment, abnormal liver enzymes, and early age of onset should alert one to the presence of karyomegalic nephropathy. It represents an under-diagnosed disorder with a high degree of ploidy indicative of karyotypic abnormality. | DOI: | http://monash.idm.oclc.org/login?url=http://dx.doi.org/10.1093/ndt/17.11.1914 | PubMed URL: | 12401846 [http://www.ncbi.nlm.nih.gov/pubmed/?term=12401846] | ISSN: | 0931-0509 | URI: | https://repository.monashhealth.org/monashhealthjspui/handle/1/27238 | Type: | Article | Subjects: | kidney function lung infection/co [Complication] male mitosis ploidy priority journal proteinuria school child sepsis/co [Complication] cell antigen/ec [Endogenous Compound] cycline/ec [Endogenous Compound] cyclosporin/ae [Adverse Drug Reaction] cyclosporin/dt [Drug Therapy] Ki 67 antigen/ec [Endogenous Compound] leukocyte antigen/ec [Endogenous Compound] liver enzyme/ec [Endogenous Compound] kidney transplantation adult article cause of death clinical article clinical feature diagnostic procedure disease course epithelium cell female glomerulonephritis/di [Diagnosis] hematuria hemolytic uremic syndrome/si [Side Effect] histology human karyotyping kidney biopsy *kidney disease/di [Diagnosis] kidney epithelium *kidney failure/co [Complication] *kidney failure/dt [Drug Therapy] *kidney failure/su [Surgery] karyotyping diagnostic procedure *kidney disease / *diagnosis kidney epithelium *kidney failure / *complication / *drug therapy / *surgery kidney function kidney transplantation lung infection / complication male mitosis ploidy priority journal proteinuria school child sepsis / complication clinical feature clinical article cause of death article adult kidney biopsy disease course epithelium cell female glomerulonephritis / diagnosis hematuria hemolytic uremic syndrome / side effect histology human |
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