The persistent müllerian duct syndrome: a rare cause of cryptorchidism

The persistent Müllerian duct syndrome is characterized by the retention of Müllerian derivatives in patients otherwise normally virilized. Clinically, the persistence of uterus and tubes leads either to cryptorchidism or inguinal hernia, depending on whether or not the Müllerian derivatives can be...

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Bibliographic Details
Published in:European journal of pediatrics 1993-01, Vol.152 Suppl 2 (S2), p.S76-S78
Main Authors: Josso, N, Picard, J Y, Imbeaud, S, Carré-Eusèbe, D, Zeller, J, Adamsbaum, C
Format: Article
Language:English
Subjects:
Adolescent
Animals
Anti-Mullerian Hormone
Child
Child, Preschool
Chronic Disease
Cryptorchidism - blood
Cryptorchidism - etiology
Disorders of Sex Development - blood
Disorders of Sex Development - complications
Disorders of Sex Development - diagnosis
Disorders of Sex Development - genetics
Glycoproteins
Growth Inhibitors - blood
Humans
Infant
Infant, Newborn
Male
Molecular Biology
Mullerian Ducts - metabolism
Mutation
Sex Differentiation
Syndrome
Testicular Hormones - blood
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Summary:The persistent Müllerian duct syndrome is characterized by the retention of Müllerian derivatives in patients otherwise normally virilized. Clinically, the persistence of uterus and tubes leads either to cryptorchidism or inguinal hernia, depending on whether or not the Müllerian derivatives can be mobilized during testicular descent. The condition is usually discovered at surgery, however preoperative sonography could allow the diagnosis to be made preoperatively. The molecular basis of the persistent Müllerian duct syndrome is heterogeneous, and is reflected by wide variations in the serum concentration of anti-Müllerian hormone. Some cases are apparently due to end-organ resistance, and are associated with normal serum levels of the hormone. Others, characterized by absent or low hormone concentrations, can be explained by mutations of the gene coding for anti-Müllerian hormone, which are distributed along the whole length of the coding region.
ISSN:0340-6199
1432-1076
DOI:10.1007/BF02125444