2018
DOI: 10.1186/s13052-018-0577-7
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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 biochemi… Show more

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Cited by 4 publications
(3 citation statements)
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References 22 publications
(56 reference statements)
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“…Testicular autonomous hyperfuction in MAS may also be secondary to Sertoli cells activation with a consequent isolated macroorchidism usually not followed by signs of sexual precocity and prepubertal testosterone levels ( 30 , 31 ). A higher incidence of testicular abnormalities on ultrasound (microlithiasis, hypoecoic lesions, focal calcifications) has also been described ( 33 , 50 , 51 ) ( Figure 1B ). None of the patients with these lesions, followed up for several years, developed any signs of malignant transformation, so a conservative approach has been recommended ( 33 ).…”
Section: Endocrinological Featuresmentioning
confidence: 77%
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“…Testicular autonomous hyperfuction in MAS may also be secondary to Sertoli cells activation with a consequent isolated macroorchidism usually not followed by signs of sexual precocity and prepubertal testosterone levels ( 30 , 31 ). A higher incidence of testicular abnormalities on ultrasound (microlithiasis, hypoecoic lesions, focal calcifications) has also been described ( 33 , 50 , 51 ) ( Figure 1B ). None of the patients with these lesions, followed up for several years, developed any signs of malignant transformation, so a conservative approach has been recommended ( 33 ).…”
Section: Endocrinological Featuresmentioning
confidence: 77%
“…Few data about the natural evolution of sexual development and the adult fertility prognosis in male patients can be recovered. According to available data, males seemed to be able to maintain their autonomous testicular function ( 33 , 64 ).…”
Section: Long-term Health Problemsmentioning
confidence: 99%
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