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Review
. 2020 Aug 4:11:522.
doi: 10.3389/fendo.2020.00522. eCollection 2020.

Auxological and Endocrinological Features in Children With McCune Albright Syndrome: A Review

Maria Tufano  1 , Daniele Ciofi  2 , Antonella Amendolea  3 , Stefano Stagi  2
Affiliations
Review

Auxological and Endocrinological Features in Children With McCune Albright Syndrome: A Review

Maria Tufano et al. Front Endocrinol (Lausanne). .

Abstract

McCune-Albright syndrome is a rare and challenging congenital sporadic disease involving the skin and skeletal and endocrine systems with a prevalence ranges from one in 100,000 to 1,000,000. In addition to the classical triad of fibrous dysplasia of bone, café au lait pigmented skin lesions and precocious puberty, other multiple endocrinological features, including hyperthyroidism, growth hormone excess, hypercortisolism, and hypophosphatemic rickets, have been reported. A brief review of the syndrome in children is here reported.

Keywords: McCune–Albright syndrome; café au lait skin pigmentation; endocrinopathies; fibrous dysplasia of bone; precocious puberty.

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Figures

Figure 1
Figure 1
Clinical and radiological features of children diagnosed with McCune Albright syndrome. (A) Classic Café-au-lait skin pigmented lesion in a 25-months-old boy with McCune-Albright Syndrome. The spots with smooth borders, respect the midline and follow the developmental lines of Blaschko. At follow up, fibrous dysplasia of the both tibias has been revealed. (B) When he was 3-years-old, a light testicular asymmetry has been noted and testicular microlithiasis has been revealed on ultrasound. (C) Unilateral ovarian cyst in a 7-years-old girl with vaginal bleeding diagnosed with McCune Albright syndrome.
See this image and copyright information in PMC

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