Beckwitt-Wiedemann's syndrome with medullary sponge kidneys, agonadism and persistent testosterone production.

Abstract:

:A patient with Beckwitt-Wiedemann's syndrome is described in which associated malformations were hemihypertrophy of the body, medullary sponge kidneys and absence of testes and internal ducts. On the other hand, we demonstrated biochemically the existence of testosterone secretion, with active response to a 3-day HCG stimulation as well as prepuberty levels LH and FSH. This demonstrated the existence of functioning Leydig's cells. The diagnosis of agonadism and anorchia could not be entirely true since they lead to an erroneous conclusion that there always are some disseminated Leydig's cells.

journal_name

Eur Urol

journal_title

European urology

authors

Salgado AL,Macia AL,Freire A,Paramo P,Fernandez-Cruz A

doi

10.1159/000472070

subject

Has Abstract

pub_date

1977-01-01 00:00:00

pages

108-10

issue

2

eissn

0302-2838

issn

1873-7560

journal_volume

3

pub_type

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