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

2014; Springer Science+Business Media; Linguagem: Inglês

10.14310/horm.2002.1515

2520-8721

Maria Francesca Messina, Tommaso Aversa, Luisa De Sanctis, Małgorzata Waśniewska, Mariella Valenzise, Giovanni Battista Pajno, Filippo De Luca, Fortunato Lombardo,

Bone health and treatments

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 acetateleuprolide depot may be well tolerated and effective, as demonstrated by the very good adult height outcome recorded in our patient.