Renal transplantation in the management of bilateral Wilms' tumour (BWT) and of Denys-Drash syndrome (DDS)

Wilms' tumour (WT) occurs bilaterally in approximately 5-7% of affected children. In some patients, complete surgical removal of the malignant tissue cannot be achieved without bilateral total nephrectomy. In Denys-Drash syndrome (DDS), bilateral nephrectomy is indicated both because of the ass...

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Veröffentlicht in:Nephrology, dialysis, transplantation dialysis, transplantation, 1998-06, Vol.13 (6), p.1506-1510
Hauptverfasser: RUDIN, C, PRITCHARD, J, FERNANDO, O. N, DUFFY, P. G, TROMPETER, R. S
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container_issue 6
container_start_page 1506
container_title Nephrology, dialysis, transplantation
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creator RUDIN, C
PRITCHARD, J
FERNANDO, O. N
DUFFY, P. G
TROMPETER, R. S
description Wilms' tumour (WT) occurs bilaterally in approximately 5-7% of affected children. In some patients, complete surgical removal of the malignant tissue cannot be achieved without bilateral total nephrectomy. In Denys-Drash syndrome (DDS), bilateral nephrectomy is indicated both because of the associated nephropathy usually progressing rapidly to end-stage renal failure and because of the high risk of WT development in any residual renal tissue. Case records of patients with a diagnosis of either bilateral WT (BWT) or DDS, who underwent bilateral nephrectomy and subsequent renal transplantation between 1980 and 1996 at the Hospital for Sick Children, London, were reviewed. Allogeneic renal transplantation was performed in two children with BWT and four with DDS, three of whom had developed unilateral WT by the time their kidneys were removed. Renal transplantation was performed 15-49 months after bilateral nephrectomy at a mean age of 45 (26-76) months, with a minimum of 1 year tumour-free survival after completion of chemotherapy in those with WT. One patient died after renal transplantation. Five children had a favourable outcome, with a mean follow-up of 80 (29-121) months post-renal transplantation. Advances in dialysis and transplantation programmes for young children offer the potential for a marked improvement in the prognosis for patients with BWT and for those with DDS.
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Allogeneic renal transplantation was performed in two children with BWT and four with DDS, three of whom had developed unilateral WT by the time their kidneys were removed. Renal transplantation was performed 15-49 months after bilateral nephrectomy at a mean age of 45 (26-76) months, with a minimum of 1 year tumour-free survival after completion of chemotherapy in those with WT. One patient died after renal transplantation. Five children had a favourable outcome, with a mean follow-up of 80 (29-121) months post-renal transplantation. 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Case records of patients with a diagnosis of either bilateral WT (BWT) or DDS, who underwent bilateral nephrectomy and subsequent renal transplantation between 1980 and 1996 at the Hospital for Sick Children, London, were reviewed. Allogeneic renal transplantation was performed in two children with BWT and four with DDS, three of whom had developed unilateral WT by the time their kidneys were removed. Renal transplantation was performed 15-49 months after bilateral nephrectomy at a mean age of 45 (26-76) months, with a minimum of 1 year tumour-free survival after completion of chemotherapy in those with WT. One patient died after renal transplantation. Five children had a favourable outcome, with a mean follow-up of 80 (29-121) months post-renal transplantation. 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Graft diseases</topic><topic>Surgery of the urinary system</topic><topic>Syndrome</topic><topic>Wilms Tumor - genetics</topic><topic>Wilms Tumor - surgery</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>RUDIN, C</creatorcontrib><creatorcontrib>PRITCHARD, J</creatorcontrib><creatorcontrib>FERNANDO, O. N</creatorcontrib><creatorcontrib>DUFFY, P. G</creatorcontrib><creatorcontrib>TROMPETER, R. S</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Nephrology, dialysis, transplantation</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>RUDIN, C</au><au>PRITCHARD, J</au><au>FERNANDO, O. N</au><au>DUFFY, P. G</au><au>TROMPETER, R. S</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Renal transplantation in the management of bilateral Wilms' tumour (BWT) and of Denys-Drash syndrome (DDS)</atitle><jtitle>Nephrology, dialysis, transplantation</jtitle><addtitle>Nephrol Dial Transplant</addtitle><date>1998-06-01</date><risdate>1998</risdate><volume>13</volume><issue>6</issue><spage>1506</spage><epage>1510</epage><pages>1506-1510</pages><issn>0931-0509</issn><issn>1460-2385</issn><eissn>1460-2385</eissn><coden>NDTREA</coden><abstract>Wilms' tumour (WT) occurs bilaterally in approximately 5-7% of affected children. In some patients, complete surgical removal of the malignant tissue cannot be achieved without bilateral total nephrectomy. 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source MEDLINE; Oxford University Press Journals All Titles (1996-Current); EZB-FREE-00999 freely available EZB journals; Alma/SFX Local Collection
subjects Biological and medical sciences
Child, Preschool
Female
Genitalia - abnormalities
Humans
Infant
Kidney Diseases - genetics
Kidney Diseases - surgery
Kidney Neoplasms - genetics
Kidney Neoplasms - surgery
Kidney Transplantation
Male
Medical sciences
Neoplasms, Multiple Primary - genetics
Neoplasms, Multiple Primary - surgery
Nephrectomy
Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases
Surgery of the urinary system
Syndrome
Wilms Tumor - genetics
Wilms Tumor - surgery
title Renal transplantation in the management of bilateral Wilms' tumour (BWT) and of Denys-Drash syndrome (DDS)
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