REVISITING THE PHENOTYPE OF ORGANIC AND IDIOPATHIC CENTRAL PRECOCIOUS PUBERTY IN A LARGE BRAZILIAN COHORT

Program: Abstracts - Orals, Featured Poster Presentations, and Posters
Session: SAT 596-621-Pediatric Endocrinology /Steroids and Puberty
Clinical
Saturday, June 15, 2013: 1:45 PM-3:45 PM
Expo Halls ABC (Moscone Center)

Poster Board SAT-619
Carlos Eduardo Seraphim*1, Mateus Rozalem Aranha1, Vinicius N. Brito1, Priscilla Cukier1, Ivo J Arnhold1, Berenice Bilharinho Mendonca1 and Ana Claudia Latronico2
1Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil, 2Hospital das Clinicas da Faculdade de Medicina da Universidade de São Paulo, Sao Paulo, Brazil
Context: Central precocious puberty (CPP) is due to the premature activation of the hypothalamic–pituitary–gonadal axis. The occurrence of CPP is more frequent in girls (3 to 23-folds), mainly the idiopathic form, i.e., without central nervous system lesions demonstrated by magnetic resonance imaging. In contrast, organic CPP is caused by central nervous system abnormalities.

Objective: To compare the clinical and hormonal features of a large cohort of patients with idiopathic and organic CPP.

Patients and Methods: This retrospective study consisted in the clinical and biochemical revision of medical records of 104 children with CPP (71 with idiopathic and 33 with organic forms). Data are presented as mean ± standard deviation (SDS) or median and range. Comparisons were performed using unpaired Student’s T test or the Mann-Whitney’s U test when appropriated. Statistical significance was set at p <0.05.

Results: Distinct congenital and acquired lesions were diagnosed in the 33 patients (23 girls) with organic CPP: hypothalamic hamartoma (12), congenital hydrocephalus (4), myelomeningocele (4), pineal cyst (3), cerebral palsy (2), pilocytic astrocytoma (2), acquired hydrocephalus (2), pinealoma (1), neuronal ceroid lipofuscinosis (1) and Alper’s disease (1). The remaining group had the idiopathic form of early sexual development. Girls with organic CPP had earlier onset of puberty when compared with idiopathic form (3.8 vs. 5.5 yrs, p<0.001), sought for medical assistance earlier (5.4 vs 7.4 yr, p<0.001) and had lower SDS height (0.6 vs 1.8, p=0.004) and lower bone age at first medical consultation (7.3 vs 9.9 yr, p=0.015). There was no significant statistical difference of bone age (BA) minus chronological age (CA) index between the two groups (2.0 vs 2.4 yrs). Regarding hormonal data, girls with organic CPP had significant higher GnRH-stimulated LH peak (29.7 vs 12.6 U/L, p=0.046) than those with idiopathic CPP. Neurological and ophthalmological symptoms were only identified in the organic group. The most common findings were epilepsy and neuro-psychomotor development retardation. In the small male group (n=10), clinical and biochemical parameters did not differ between idiopathic and organic forms.

Conclusion: We conclude that earlier onset of puberty, neurological and ophthalmological symptoms, and higher GnRH-stimulated LH peak may suggest the presence of an organic cause in girls with CPP.

*Both authors contributed equally as first authors

Nothing to Disclose: CES, MRA, VNB, PC, IJA, BBM, ACL

*Please take note of The Endocrine Society's News Embargo Policy at http://www.endo-society.org/endo2013/media.cfm