Scientific conference in universities or research centers (Scientific conferences in universities or research centers)
le syndrome de Pasqualini (Fertile eunuch syndrome)
VALDES SOCIN, Hernan Gonzalo
2014
 

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Pasqualini&BurRevAMA1950.pdf
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Programa Aula Pasqualini.doc
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Homenaje Pasqualini
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Keywords :
lutheinizing hormone; hypogonadotropic hypogonadism; fertility; genetics; LHB; male; fertile eunuchs; hypoandrogenism; pasqualini syndrome; Luteinizing hormone mutation
Abstract :
[en] Pasqualini’s syndrome: hypoandrogenism with spermatogenesis Pasqualini and Bur published the first case of eunuchoidism with preserved spermatogenesis in 1950 in Revista de la Asociación Médica Argentina. The hypoandrogenism with spermatogenesis syndrome included: (a) eunuchoidism, (b) testis with normal spermatogenesis and full volume, with mature spermatozoids in a high proportion of seminiferous tubes and undifferentiated and immature Leydig cells (c) full functional compensation through the administration of chorionic gonadotropin hormone, while hCG is administered (d) total urinary gonadotrophins within normal limits (e) this definition supposes the normal activity of the pituitary and the absence of congenital malformations in general. A first step in the understanding of the physiopathogeny of Pasqualini syndrome or the so called “fertile eunuch" syndrome was the absence of LH in plasma and urine of patients. The second breakthrough was the functional and genetic studies that validated the hypothesis of a functional deficit of LH in these men: it will then also be described in some women. Different groups including ours demonstrated in these cases a LH with varying degrees of immunological activity but biologically inactive in most of the patients, due to one or more inactivating mutations in the LHB gene. Finally, the full comprehension of Pasqualini syndrome allowed to reverse the hypoandrogenic phenotype and to restore fertility in these patients through the use of chorionic gonadotropin and the modern in-vitro fertility techniques. This conference is an historical review and a tribute to the memory of Rodolfo Q. Pasqualini.
Disciplines :
Endocrinology, metabolism & nutrition
Author, co-author :
VALDES SOCIN, Hernan Gonzalo  ;  Centre Hospitalier Universitaire de Liège - CHU > Endocrinologie clinique
Language :
English
Title :
le syndrome de Pasqualini (Fertile eunuch syndrome)
Alternative titles :
[en] Pasqualini's syndrome (Fertile eunuch syndrome)
Publication date :
12 November 2014
Number of pages :
1
Event name :
Hommage au prof Pasqualini
Event organizer :
Servicio de Endocrinologia. Hospital de Clinicas jose de San martin
Event place :
Buenos Aires, Argentina
Event date :
12 novembre 2014
Name of the research project :
Fonds de Mobilté en Amérique du Sud
Funders :
Région wallonne [BE]
Available on ORBi :
since 24 November 2014

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