ISSN:
1437-9813
Keywords:
Persistent müllerian duct syndrome
;
Müllerian inhibiting substance
;
Gubernaculum
;
Testicular descent
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Abstract A 4-month-old phenotypic male with persistent müllerian duct syndrome (PMDS) is described. Serum levels of müllerian inhibiting substance (MIS) were undetectable in an enzyme immunoassay, consistent with a in an MIS gene. Normal germ cells were present in the testis, suggesting that subsequent azoospermia found in other patients with this abnormality is caused by a postnatal defect in germ cell development after 4 months of age. The testes were not attached by normal male gubernacula, but were loosely anchored by a long “round ligament” to the inguinal region. Failure of masculinasation of the gubernaculum in this syndrome suggests that MIS is responsible for early gubernacular development, and hence may have a role in testicular descent.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF00179676
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