Testicular degeneration in three patients with the persistent müllerian duct syndrome
| Autor: | S, Imbeaud, R, Rey, P, Berta, J L, Chaussain, J M, Wit, R H, Lustig, M A, De Vroede, J Y, Picard, N, Josso |
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| Rok vydání: | 1995 |
| Předmět: |
Male
endocrine system medicine.medical_specialty Pathology endocrine system diseases Mullerian Ducts Disorders of Sex Development Uterus Hernia Inguinal urologic and male genital diseases Testicular Diseases Internal medicine Cryptorchidism Testis Scrotum medicine Humans Testicular torsion Spermatic Cord Torsion Ultrasonography urogenital system business.industry Infant Newborn Infant Syndrome medicine.disease Inguinal canal Growth Inhibitors Testicular Hormones medicine.anatomical_structure Endocrinology Normal Virilization Chronic Disease Pediatrics Perinatology and Child Health Persistent Müllerian duct syndrome Testicular Regression business Gonadotropins |
| Zdroj: | European Journal of Pediatrics. 154:187-190 |
| ISSN: | 1432-1076 0340-6199 |
| Popis: | The presistent Mullerian duct syndrome, characterized by the presence of uterus and tubes in males, is a familial disorder due to defects of synthesis or action of anti-Mullerian hormone, a Sertoli cell glycoprotein responsible for the regression of Mullerian derivatives in normal male fetuses. Patients are normally virilized and testicular production of testosterone is normal. Both testes my be cryptorchild; alternatively, one may be descended into the inguinal canal or scrotum, together with the Mullerian derivatives, a condition known as “hernia uteri inguinalis”. We have recently observed three patients affected by the presistent Mullerian duct syndrome who experienced progressive degeneration of testicular tissue. In two, functional testicular tissue was still present some months after birth, but deteriortated progressively later. In one patient, testicular tissue was already absent at birth, but the normal virilization of external genitalia indicated that testicular degeneration must have occurred lat during fetal life, after the expected time of regression of male Mullerian ducts. The high incidence of degeneration of testicular tissue in the presistent Mullerian duct syndrome could be indirectly linked to anatomical abnormalities which could favour testicular torsion, known to induce testicular regression. |
| Databáze: | OpenAIRE |
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