BACKGROUND: This study was carried out because of the rarity of peripheral precocious puberty (PPP) in boys with McCune-Albright syndrome (MAS) and the lack of data on adult height of treated MAS males, treatment for this disorder being not as yet standardized. AIMS: To report the adult height of a MAS boy with PPP who was treated with ketoconazole – cyproterone acetate – leuprolide depot and to describe some atypical aspects of MAS presentation and course in this boy. CASE HISTORY: The case concerns a boy presenting with unilateral macroorchidism, no signs of PPP or initially isolated Sertoli cell activation and MAS mutation that subsequently also activated Leydig cells, thus inducing a change in phenotypic expression. CONCLUSIONS: a) In a MAS boy presenting with unilateral macroorchidism and no other signs of PPP, a consecutive involvement of Leydig cells may follow the initially isolated activation of Sertoli cells; b) prolonged treatment with ketoconazole – cyproterone acetate – leuprolide depot may be well tolerated and effective, as demonstrated by the very good adult height outcome recorded in our patient

Adult height following a combined treatment of ketoconazole - cyproterone acetate - leuprolide depot in a boy with atypical McCune-Albright syndrome

MESSINA, Maria Francesca
Primo
;
AVERSA, TOMMASO;WASNIEWSKA, Malgorzata Gabriela;VALENZISE, Mariella;PAJNO, Giovanni Bat.;DE LUCA, Filippo
;
LOMBARDO, Fortunato
Ultimo
2015

Abstract

BACKGROUND: This study was carried out because of the rarity of peripheral precocious puberty (PPP) in boys with McCune-Albright syndrome (MAS) and the lack of data on adult height of treated MAS males, treatment for this disorder being not as yet standardized. AIMS: To report the adult height of a MAS boy with PPP who was treated with ketoconazole – cyproterone acetate – leuprolide depot and to describe some atypical aspects of MAS presentation and course in this boy. CASE HISTORY: The case concerns a boy presenting with unilateral macroorchidism, no signs of PPP or initially isolated Sertoli cell activation and MAS mutation that subsequently also activated Leydig cells, thus inducing a change in phenotypic expression. CONCLUSIONS: a) In a MAS boy presenting with unilateral macroorchidism and no other signs of PPP, a consecutive involvement of Leydig cells may follow the initially isolated activation of Sertoli cells; b) prolonged treatment with ketoconazole – cyproterone acetate – leuprolide depot may be well tolerated and effective, as demonstrated by the very good adult height outcome recorded in our patient
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11570/3094592
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