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.
|Autori interni:||SAGGESE, GIUSEPPE|
|Autori:||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 AM; SEGNI M; RIGON F; SAGGESE G; BERTELLONI S; BUZI F|
|Titolo:||McCune-Albright syndrome: A longitudinal clinical study of 32 patients|
|Anno del prodotto:||1999|
|Appare nelle tipologie:||1.1 Articolo in rivista|