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Nephrol Dial Transplant (2003) 18: 2165-2169
© 2003 European Renal Association-European Dialysis and Transplant Association

Brief Report

Outcome of kidney transplantation in autosomal dominant medullary cystic kidney disease type 1

Christoforos Stavrou1, C. Constantinou Deltas2, Tasos C. Christophides3 and Alkis Pierides4

1Royal Artemis Medical Centre, Pafos, 2Cyprus Institute of Neurology and Genetics and Department of Biological Sciences, University of Cyprus, Nicosia, 3Department of Mathematics and Statistics, University of Cyprus, Nicosia and 4Nicosia General Hospital, Nicosia, Cyprus

Correspondence and offprint requests to: Dr Christoforos Stavrou, 8 Solon Street, 8577 Tala, Pafos, Cyprus. Email: royaldial{at}cytanet.com.cy

Background. Autosomal dominant medullary cystic kidney disease (ADMCKD) is an inherited, distinct, chronic, tubulointerstitial, cystic-type nephropathy, often described together with juvenile nephronophthisis as a single disease complex (NPH–MCD). However, since the recent localization of two genes responsible for ADMCKD, namely MCKD1 and MCKD2, ADMCKD has gained independent status. Unfortunately, there appears to be a distinct lack of up-to-date information in the currently available medical literature concerning worldwide patient and graft survival after renal transplantation in ADMCKD. This report is based on all 41 transplanted patients [19 suffering from autosomal dominant medullary cystic kidney disease type 1 (ADMCKD1) and 22 from other causes] who were referred for kidney transplantation from our centre in Pafos, Cyprus between 1976 and 2000. All patients had regular follow-up examinations. This report aims to present the results of kidney transplantation of the 19 ADMCKD1 patients and to compare them with those for the 22 non-ADMCKD patients.

Methods. Patient and graft survival times in both groups were recorded, analysed and compared 1 and 5 years post-transplant. Patient and graft survival times were calculated according to the Kaplan–Meier method and some descriptive statistical comparisons were based on the {chi}2-test.

Results. The 1 year patient and graft survival rates for ADMCKD1 (group A) were 100%, while the 5 year figures were 100% and 90%, respectively. For non-ADMCKD1 patients (group B) the 1 year figures were 95% for both parameters, while the 5 year figures were 93.3% for both parameters. There were no statistically significant differences in patient and graft survival times between the two groups.

Conclusions. Kidney transplantation is the treatment of choice for patients suffering from ADMCKD, with an excellent outcome and no specific complications.

Keywords: autosomal dominant medullary cystic kidney disease; kidney transplantation; nephronophthisis


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 Nephrol Dial TransplantHome page
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