Are adolescent boys with Klinefelter syndrome androgen deficient? A longitudinal study of Finnish 47,XXY boys

Pediatric Research
Anne M WikströmTaneli Raivio

Abstract

Testosterone (T)-substitution therapy is widely used in adult patients with Klinefelter syndrome (KS) to prevent symptoms and sequels of androgen deficiency, but it is currently unknown if adolescent boys with KS benefit from early T therapy. To evaluate the optimal age to start T substitution, we searched for signs of androgen deficiency in pubertal boys with KS. 14 nonmosaic 47,XXY boys, aged 10-13.9 y, were followed up for 4-37 mo with staging of puberty and frequent reproductive hormone measurements. Furthermore, indices reflecting androgen action (serum SHBG, leptin, and prostate-specific antigen (PSA) levels) were studied. Both onset and progression of puberty according to Tanner stages were normal in boys with KS. Consistently, serum T concentrations increased as expected and remained normal throughout follow-up. Changes in the indices of androgen action (decreases in serum SHBG and leptin, and increase in serum PSA concentrations) occurred normally, except that average leptin levels were higher in the boys with KS (KS boys 11.8 +/- 7.0 microg/L; controls 7.6 +/- 4.7 microg/L; p = 0.033). Despite normal T concentrations, the KS boys displayed from the age of 13 y elevated serum FSH and LH levels, and exaggerated gonadotr...Continue Reading

References

Jan 27, 1990·BMJ : British Medical Journal·J N MatthewsP Royston
Apr 1, 1988·Clinical Genetics·J NielsenK Sørensen
Jan 1, 1985·Pediatric Research·J A SalbenblattJ S Winter
Feb 1, 1970·The Journal of Pediatrics·S A MyhreV C Kelley
May 1, 1971·The Journal of Clinical Endocrinology and Metabolism·Z Laron, I H Hochman
Sep 1, 1982·European Journal of Pediatrics·E TopperZ Laron
Aug 15, 1997·The Journal of Clinical Investigation·M WabitschH Hauner
Jun 30, 1998·Archives of Internal Medicine·C M Smyth, W J Bremner
May 18, 1999·Archives of Disease in Childhood·S Ratcliffe
Jun 21, 2001·European Journal of Endocrinology·C Ankarberg-LindgrenE Norjavaara
Feb 8, 2003·The Journal of Clinical Endocrinology and Metabolism·Anders BojesenClaus Højbjerg Gravholt
Nov 14, 2003·Genetics in Medicine : Official Journal of the American College of Medical Genetics·Joe Leigh SimpsonC Alvin Paulsen
Mar 6, 2004·The Journal of Clinical Endocrinology and Metabolism·Taneli RaivioOlli A Jänne
Apr 9, 2004·The Journal of Clinical Endocrinology and Metabolism·Najiba LahlouMarc Roger
May 6, 2004·The Journal of Clinical Endocrinology and Metabolism·Anne M WikströmLeo Dunkel
Jul 21, 2004·Lancet·Fabio LanfrancoEberhard Nieschlag
Aug 10, 2005·Hormone Research·Judith L RossAndrew Zinn

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Citations

Jan 21, 2010·Human Reproduction·G FullertonA Maheshwari
Mar 19, 2013·European Journal of Endocrinology·L Aksglaede, A Juul
Aug 29, 2013·Case Reports in Pediatrics·Anju Sukumaran, John Buchlis
Feb 5, 2011·Journal of Endocrinological Investigation·A F RadicioniA Lenzi
May 3, 2014·Annales d'endocrinologie·Ingrid PlottonHervé Lejeune
Apr 1, 2014·The Journal of Urology·Akanksha MehtaDarius A Paduch
Nov 30, 2013·The Urologic Clinics of North America·James M Hotaling
Aug 13, 2011·Gynécologie, obstétrique & fertilité·I PlottonH Lejeune
Nov 17, 2015·Endocrinology and Metabolism Clinics of North America·Shanlee M DavisJudith L Ross
May 19, 2009·Schizophrenia Research·Sophie Van RijnHanna Swaab
Apr 22, 2008·The Urologic Clinics of North America·Robert D Oates
Apr 16, 2008·The Journal of Pediatrics·Martha P D ZegerJudith L Ross
Jun 12, 2009·International Journal of Andrology·L AksglaedeA Juul
Jan 11, 2011·Acta Paediatrica·Rodolfo A ReyIgnacio Bergadá
Jan 22, 2011·Acta Paediatrica·Martha Z BardsleyJudith L Ross
May 6, 2015·American Journal of Medical Genetics. Part C, Seminars in Medical Genetics·Carole Samango-SprouseAndrea L Gropman
Jul 25, 2015·The Journal of Pediatrics·Sharron CloseNancy Reame
Jan 21, 2014·La Presse médicale·Hervé LejeuneIngrid Plotton
Jan 30, 2016·Clinical Anatomy : Official Journal of the American Association of Clinical Anatomists & the British Association of Clinical Anatomists·Rebecca J Bird, Bradley J Hurren
Jul 15, 2016·International Journal of Endocrinology·Stefano StagiMaurizio de Martino
Sep 21, 2016·Journal of Endocrinological Investigation·M BonomiUNKNOWN Klinefelter ItaliaN Group (KING)
May 28, 2008·Hormone Research·Anne M Wikström, Leo Dunkel
May 1, 2014·Expert Review of Endocrinology & Metabolism·Sara Brilli, Gianni Forti
May 18, 2020·American Journal of Medical Genetics. Part C, Seminars in Medical Genetics·Michael Zitzmann, Julia Rohayem
Feb 1, 2020·Current Opinion in Obstetrics & Gynecology·Carole A Samango-SprouseAndrea L Gropman
Aug 24, 2006·The Journal of Clinical Endocrinology and Metabolism·Anne M WikströmLeo Dunkel
Jan 25, 2013·American Journal of Medical Genetics. Part C, Seminars in Medical Genetics·Lise AksglaedeAnders Juul
Dec 12, 2019·Human Reproduction Update·Nicholas A DeebelHooman Sadri-Ardekani
Jun 14, 2019·The Journal of Clinical Endocrinology and Metabolism·Yee-Ming ChanElfa Jonsdottir-Lewis
Sep 16, 2021·Clinical Endocrinology·Mila TannerTaneli Raivio

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