Testicular histology in boys with Prader-Willi syndrome: fertile or infertile?

The Journal of Urology
Annick VogelsGuy A Bogaert

Abstract

Prader-Willi syndrome is associated with hypogonadism. Cryptorchidism is found in 93% of cases and considered a phenotypic criterion. Men with Prader-Willi syndrome are thought to be infertile. To study the fertility probability in boys with Prader-Willi syndrome we analyzed testicular histology in 8 prepubertal boys and 1 man. Eight boys 16 months to 14 years old with a proven molecular diagnosis of Prader-Willi syndrome, including 6 with a deletion on chromosome 15 and 2 with uniparental maternal disomy of chromosome 15, underwent orchiopexy and the man underwent unilateral orchiectomy. Prepubertal testes were classified into 4 Nistal categories according to mean tubular diameter, the tubular fertility index (average percent of tubules containing spermatogonia) and the Sertoli's cell index. Two of 8 prepubertal boys showed a favorable Nistal score of I, 1 showed a Nistal score of II and 5 showed a Nistal score of III. The testis in the man showed diffuse tubular atrophy with tubular hyalinization, a Sertoli's cell nodule, vacuolized Leydig cells, peritubular hyalinization and small tubuli. Prader-Willi syndrome appears to be a heterogenic disorder with respect to testicular histology. Although most boys showed absent spermato...Continue Reading

References

Nov 1, 1975·Anesthesiology·R A Milliken, D M Weintraub
Mar 18, 1977·European Journal of Pediatrics·M L KatcherJ M Opitz
Jan 1, 1987·European Journal of Pediatrics·F HadziselimovicM Buser
May 1, 1968·American Journal of Diseases of Children·H Zellweger, H J Schneider
Jul 1, 1980·The Journal of Urology·D Uehling
Nov 1, 1980·Human Pathology·M NistalJ A Díez-Pardo
Jan 1, 1980·Clinical Endocrinology·W J JeffcoateG M Besser
Dec 10, 1997·Journal of Medical Genetics·S B Cassidy
Nov 27, 1999·Developmental Medicine and Child Neurology·A AkefeldtC Gillberg
Jun 26, 2001·Hormone Research·D S HuffH M Snyder
Dec 18, 2003·European Journal of Human Genetics : EJHG·Annick VogelsJ P Fryns
Dec 15, 2005·The Journal of Clinical Endocrinology and Metabolism·Urs EiholzerManuela Simoni
Dec 2, 2006·The Journal of Pediatrics·Marc FillionGuy Van Vliet
Feb 7, 2008·Hormone Research·Maithé Tauber, Wayne Cutfield

❮ Previous
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Citations

Aug 13, 2011·American Journal of Medical Genetics. Part a·Margje SinnemaConstance T R M Schrander-Stumpel
Mar 9, 2011·Annals of the New York Academy of Sciences·Kathleen HwangDolores J Lamb
Jun 13, 2015·Journal of Endocrinological Investigation·M A AnguloM E Cataletto
May 3, 2016·Pediatric and Developmental Pathology : the Official Journal of the Society for Pediatric Pathology and the Paediatric Pathology Society·Manuel NistalMiguel Reyes-Múgica
Aug 22, 2013·International Journal of Pediatric Endocrinology·Jill E Emerick, Karen S Vogt
Nov 19, 2011·American Journal of Clinical Pathology·Tatjana AnticJerome B Taxy
Jul 14, 2018·Molecular Genetics & Genomic Medicine·Talia Eldar-GevaRachel Eiges
Dec 23, 2011·The Journal of Clinical Endocrinology and Metabolism·Elbrich P C SiemensmaAnita C S Hokken-Koelega
Apr 30, 2009·The Journal of Clinical Endocrinology and Metabolism·Harry J HirschVarda Gross-Tsur
May 27, 2014·Journal of Pediatric Endocrinology & Metabolism : JPEM·Letícia E SewaybrickerGil Guerra-Júnior
Jan 23, 2020·AACE Clinical Case Reports·Elena MonaiRikke Beck Jensen
Mar 1, 2019·Endocrinology, Diabetes & Metabolism·Satoko MatsuyamaAtsushi Mizokami
Mar 2, 2021·The Lancet. Diabetes & Endocrinology·Maithé Tauber, Charlotte Hoybye
Mar 7, 2021·International Journal of Molecular Sciences·Luigi NapolitanoMassimiliano Creta
Apr 4, 2021·International Journal of Molecular Sciences·Cees NoordamUrs Eiholzer

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