Linked Life Data

19794382

Source:http://linkedlifedata.com/resource/pubmed/id/19794382

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
5
pubmed:dateCreated
2009-10-1
pubmed:abstractText
Two girls with central precocious puberty (CPP) associated with hypothalamic hamartoma (HH) and non classical form of congenital adrenal hyperplasia (NCAH), are reported. Case 1. The first patient, who showed at age around 4 years the onset of CPP, was submitted in view of some organic lesion to magnetic resonance (MRI) of the brain which documented the presence of HH. The remarkable acceleration of bone age (BA) advanced of 3 SD and some clinical signs of hyperandrogenism suggested the coexistence of NCAH, proved by adrenocorticotropic hormone (ACTH) test and molecular analysis. She resulted carrier of partial 21-hydroxylase deficiency. Case 2. In the second girl with CPP, aged 6.5 years, the remarkable advancement (4 SD) of bone age (BA) alerted to adrenal involvement. ACTH stimulation test and molecular analysis showed NACH due to 21-hydroxylase deficiency. Brain MRI, performed mainly for severe headache, showed the presence of HH. Yearly brain MRI to monitor HH dimensions and neurological examination with EEG, in order to exclude anomalies referable to gelastic epilepsy are advisable, in both cases. The authors' observation emphasizes the need to be careful in young patients with CPP, with fast progression of pubertal development and remarkable BA advancement. The association of CPP with HH and NCAH should be considered, performing not only MRI of the brain, but also ACTH test, beside LHRH test for the diagnosis of CPP. At the authors' knowledge this association has not been reported so far. Further observations are needed to understand if this rare combination is occasional or genetically determined.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Oct
pubmed:issn
0026-4946
pubmed:author
pubmed:issnType
Print
pubmed:volume
61
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
561-4
pubmed:meshHeading
pubmed:year
2009
pubmed:articleTitle
Precocious puberty with hypothalamic hamartoma and non classical form of congenital adrenal hyperplasia. Report of two cases.
pubmed:affiliation
Pediatric Department, La Sapienza University, Rome, Italy. annamaria.pasquino@virgilio.it
pubmed:publicationType
Journal Article, Case Reports