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Phenotypic testicular abnormalities and pubertal development in boys with McCune-Albright syndrome.


ABSTRACT: Aim of this survey is to review the few available literature data on pathophysiologic and clinical aspects of pubertal development in boys with McCune-Albright syndrome (MAS). On the basis of such analysis, we concluded that:1) peripheral precocious puberty (PPP) is significantly more infrequent in boys than in girls; 2) the most common testicular abnormality at MAS presentation is macroorchidism, that may be either monolateral or bilateral; 3) macroorchidism is not always associated with clinical and biochemical evidence of PPP; 4) testicular microlothiasis is distinctly more frequent in boys with MAS than in those without MAS; 5) the available therapeutic schedules have to be adopted already at MAS presentation only in the cases with PPP.

SUBMITTER: Aversa T 

PROVIDER: S-EPMC6245937 | biostudies-literature | 2018 Nov

REPOSITORIES: biostudies-literature

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Phenotypic testicular abnormalities and pubertal development in boys with McCune-Albright syndrome.

Aversa Tommaso T   Zirilli Giuseppina G   Corica Domenico D   De Luca Filippo F   Wasniewska Malgorzata M  

Italian journal of pediatrics 20181119 1


Aim of this survey is to review the few available literature data on pathophysiologic and clinical aspects of pubertal development in boys with McCune-Albright syndrome (MAS). On the basis of such analysis, we concluded that:1) peripheral precocious puberty (PPP) is significantly more infrequent in boys than in girls; 2) the most common testicular abnormality at MAS presentation is macroorchidism, that may be either monolateral or bilateral; 3) macroorchidism is not always associated with clinic  ...[more]

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