McCune-Albright syndrome: A longitudinal clinical study of 32 patients

Carlo De Sanctis, Roberto Lala, Patrizia Matarazzo, Antonio Balsamo, Rosalba Bergamaschi, Marco Cappa, Mariangela Cisternino, Vincenzo De Sanctis, Marco Lucci, Adriana Franzese, Lucia Ghizzoni, Anna Maria Pasquino, Maria Segni, Franco Rigon, Giuseppe Saggese, Silvano Bertelloni, Fabio Buzi

Research output: Contribution to journalArticle

Abstract

We report the diagnostic clinical features and their long term evolution in 32 patients with McCune-Albright syndrome. Patient data are made up of two periods: the first, classified as personal history, is from birth until the time when the diagnosis of McCune-Albright syndrome was made; the second, classified as clinical observation, is from the first observation until the end of follow up. The total duration of these two periods was 9.6 ± 2.9 yr; mean age at first observation was 5.7 yr (range 0.7-11 yr). The probability of manifesting main clinical signs according to age was calculated: almost all had skin dysplasia at birth, 50% probability of peripheral precocious puberty in females at 4 years and 50% of bone dysplasia at 8 years of age were found. Other clinical signs had diagnostic relevance when preceding the main signs leading to diagnosis of McCune-Albright syndrome even without specific genetic investigation. The most important clinical manifestations have different evolutions: skin lesions increase in dimensions according to body growth; precocious puberty in females evolves rapidly but periods of regression can be seen in some patients; bone dysplasia in most patients evolves with an increase both in the number of affected bones and in the severity of lesions.

Original languageEnglish
Pages (from-to)817-826
Number of pages10
JournalJournal of Pediatric Endocrinology and Metabolism
Volume12
Issue number6
Publication statusPublished - 1999

Keywords

  • Bone fibrous dysplasia
  • Cutaneous cafe-au-lait spots
  • McCune-Albright syndrome
  • Precocious puberty

ASJC Scopus subject areas

  • Endocrinology
  • Pediatrics, Perinatology, and Child Health

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  • Cite this

    De Sanctis, C., Lala, R., Matarazzo, P., Balsamo, A., Bergamaschi, R., Cappa, M., Cisternino, M., De Sanctis, V., Lucci, M., Franzese, A., Ghizzoni, L., Pasquino, A. M., Segni, M., Rigon, F., Saggese, G., Bertelloni, S., & Buzi, F. (1999). McCune-Albright syndrome: A longitudinal clinical study of 32 patients. Journal of Pediatric Endocrinology and Metabolism, 12(6), 817-826.