Source:http://linkedlifedata.com/resource/pubmed/id/10614538
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Predicate | Object |
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rdf:type | |
lifeskim:mentions | |
pubmed:issue |
6
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pubmed:dateCreated |
2000-1-13
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pubmed:abstractText |
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.
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pubmed:language |
eng
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pubmed:journal | |
pubmed:citationSubset |
IM
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pubmed:status |
MEDLINE
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pubmed:issn |
0334-018X
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pubmed:author |
pubmed-author:BalsamoAA,
pubmed-author:BergamaschiRR,
pubmed-author:BertelloniSS,
pubmed-author:BuziFF,
pubmed-author:CappaMM,
pubmed-author:CisterninoMM,
pubmed-author:FranzeseAA,
pubmed-author:GhizzoniLL,
pubmed-author:LallMM,
pubmed-author:LucciMM,
pubmed-author:MatarazzoPP,
pubmed-author:PasquinoA MAM,
pubmed-author:RigonFF,
pubmed-author:SaggeseGG,
pubmed-author:SegniMM,
pubmed-author:de SanctisCC,
pubmed-author:de SanctisVV
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pubmed:issnType |
Print
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pubmed:volume |
12
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
817-26
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pubmed:dateRevised |
2005-11-16
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pubmed:meshHeading |
pubmed-meshheading:10614538-Child,
pubmed-meshheading:10614538-Child, Preschool,
pubmed-meshheading:10614538-Female,
pubmed-meshheading:10614538-Fibrous Dysplasia, Polyostotic,
pubmed-meshheading:10614538-Humans,
pubmed-meshheading:10614538-Infant,
pubmed-meshheading:10614538-Longitudinal Studies,
pubmed-meshheading:10614538-Male
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pubmed:articleTitle |
McCune-Albright syndrome: a longitudinal clinical study of 32 patients.
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pubmed:affiliation |
Divisione di Endocrinologia Pediatrica, Ospedale Regina Margherita, Torino, Italy.
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pubmed:publicationType |
Journal Article,
Review
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