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


OBJECTIVE
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.


METHODS
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.
Department of Pediatrics, University of Messina, Messina, Italy.
Maria Francesca
MF
Messina
Department of Pediatrics, University of Messina, Messina, Italy.
Tommaso
T
Aversa
Department of Pediatrics, University of Torino, Torino, Italy.
Luisa
L
de Sanctis
Department of Pediatrics, University of Messina, Messina, Italy.
Malgorzata
M
Wasniewska
Department of Pediatrics, University of Messina, Messina, Italy.
Mariella
M
Valenzise
Department of Pediatrics, University of Messina, Messina, Italy.
Giovanni Battista
GB
Pajno
Department of Pediatrics, University of Messina, Messina, Italy.
Filippo
F
De Luca
Department of Pediatrics, University of Messina, Messina, Italy.
Fortunato
F
Lombardo
D000293:Adolescent; D000726:Androgen Antagonists / Q000494:pharmacology / Q000627:therapeutic use; D001827:Body Height / Q000187:drug effects*; D002648:Child; D002675:Child, Preschool; D017373:Cyproterone Acetate / Q000494:pharmacology / Q000627:therapeutic use*; D004359:Drug Therapy, Combination; D005359:Fibrous Dysplasia, Polyostotic / Q000188:drug therapy*; D006801:Humans; D007654:Ketoconazole / Q000494:pharmacology / Q000627:therapeutic use*; D016729:Leuprolide / Q000494:pharmacology / Q000627:therapeutic use*; D008297:Male; D016896:Treatment Outcome
D000726:Androgen Antagonists; D017373:Cyproterone Acetate; D016729:Leuprolide; D007654:Ketoconazole