Wilms tumor in a pediatric renal transplant recipient with unexpected Denys–Drash syndrome

Bydder, S.; Charles, Adrian; Hewitt, Ian K.; Walpole, Ian; Algar, Elizabeth; Smith, Nicholas M.; Phillips, Marianne

doi:10.1016/s0041-1345(02)03683-7

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“…Bei dem hier be schriebe nen Jun gen hät te sich mög li cher wei se ohne die pro phy lak ti sche Ne phrek to mie noch ein voll stän di ges De nys-Drash-Syndrom mit Wilms-Tu mor ent wi ckelt. Es gibt be reits be schrie be ne Fäl le, in de nen die Di ag no se erst un er war tet im Rah men ei ner Nie ren trans plan ta ti on ge stellt wurde [8].…”

Section: Fallunclassified

Denys-Drash-Syndrom

Zugor

Zenker

Dötsch

et al. 2005

Urologe

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Denys-Drash syndrome is a rare symptom complex associated with obligatory childhood nephrotic syndrome, male pseudohermaphroditism, and Wilms' tumor. The etiology of Denys-Drash syndrome is attributed to a mutation of the WT1 gene. We report on two cases of Deny-Drash syndrome confirmed by genetic testing. Rapidly evolving terminal renal insufficiency was detected in both patients necessitating bilateral nephrectomies with prophylactic intent. In one of the patients, a Wilms' tumor had already been verified in one kidney so that chemotherapy had to be initiated.The risk of Wilms' tumor is very high in patients with a WT1 mutation, which leads to the need for removal of both kidneys during or before transplantation. It would be important to perform a diagnostic work-up for WT1 gene mutation in children who develop renal failure in the 1st year of life.

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Section: Fallunclassified