Document Detail

Testicular and epididymal involvement in Fabry's disease.
MedLine Citation:
PMID:  6420529     Owner:  NLM     Status:  MEDLINE    
Autopsy specimens of the testes and epididymis of a 32-year-old male with Fabry's disease were studied by light and electron microscopy. The characteristic ceramide deposits (lamellar inclusions, myeloid-bodies, zebra-bodies) were found in the Leydig cells as well as in the epithelial lining of both the ductuli efferentes and the ductus of the epididymis. Similar myeloid-bodies were observed in the blood vessels, connective tissue cells and muscle cells of the testicular interstitium, tunica albuginea and epididymis. In contrast, myeloid-bodies were absent or scarce in both the seminiferous epithelium and the mediastinum testis. However, the seminiferous tubules were severely affected showing reduced diameter, thickening of the tunica propria, and a few degenerated spermatogonia and primary spermatocytes. The different degrees of involvement of the testicular structures might be related to their different functions, the absorptive and permeable structures being the most severely affected. The development of testicular alterations seems to have been slow, since the patient fathered three children.
M Nistal; R Paniagua; M L Picazo
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  The Journal of pathology     Volume:  141     ISSN:  0022-3417     ISO Abbreviation:  J. Pathol.     Publication Date:  1983 Oct 
Date Detail:
Created Date:  1984-03-14     Completed Date:  1984-03-14     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  0204634     Medline TA:  J Pathol     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  113-24     Citation Subset:  IM    
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MeSH Terms
Epididymis / pathology*,  ultrastructure
Fabry Disease / pathology*,  ultrastructure
Leydig Cells / ultrastructure
Microscopy, Electron
Seminiferous Tubules / ultrastructure
Testis / pathology*,  ultrastructure

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