Wednesday, January 27, 2010

- Sertoli-cell-only syndrome (germinal cell aplasia).

Sertoli-cell-only syndrome (SCO)
-Def:- It is a condition in which only Sertoli cells line the seminiferous tubules of the testes and characterized by male sterility (due to azoospermia) without sexual abnormality.
- Synonyms:-
  • Del Castillo syndrome.
  • Germ cell aplasia.
-The etiology (1):-
  • Most causes of SCO syndrome are idiopathic. A congenital absence of germ cells due to failure of migration of gonocytes is theoretically possible.
  • Massive deletions in the azoospermia factor (AZF) region of the Y chromosome. found.
  • Exposure to chemicals and toxins may cause SCO; however, direct cause-and-effect relationships in humans have been difficult to document.
  • Klinefelter syndrome, 47 XXY, results in a characteristic biopsy appearance of SCO and Leydig cell hyperplasia.

N.B:- Attempting to distinguish between primary (congenital) and secondary (acquired) SCO syndrome is of no prognostic significance.
- Clinical picture:-
Patients is normal and have normal secondary male features and have normal or small-sized testes.
- Diagnosis:-
  • Testicular biopsy would confirm the absence of spermatozoa .
  • It is characterized histologically by complete loss of the germinal epithelium in testicular tubules, and clinically by aspermia.

-No known effective treatment exists.

Return to list of medical syndromes here

*References:-
(1)Sertoli-Cell-Only Syndrome(emedicine)

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