Etiology and therapeutic outcomes of children with gonadotropin-independent precocious puberty.
10.6065/apem.2016.21.3.136
- Author:
Eungu KANG
1
;
Ja Hyang CHO
;
Jin Ho CHOI
;
Han Wook YOO
Author Information
1. Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Korea. hwyoo@amc.seoul.kr
- Publication Type:Original Article
- Keywords:
Precocious puberty;
Gonadotropin-independent;
Etiology;
Treatment outcome
- MeSH:
Adrenal Hyperplasia, Congenital;
Androgens;
Aromatase Inhibitors;
Child*;
Chorionic Gonadotropin;
Dehydroepiandrosterone Sulfate;
Diagnosis;
Estradiol;
Female;
Fibrous Dysplasia, Polyostotic;
Follow-Up Studies;
Gonadal Steroid Hormones;
Gonadotropin-Releasing Hormone;
Humans;
Luteinizing Hormone;
Male;
Ovarian Cysts;
Prognosis;
Puberty, Precocious*;
Retrospective Studies;
Treatment Outcome;
Uterine Hemorrhage
- From:Annals of Pediatric Endocrinology & Metabolism
2016;21(3):136-142
- CountryRepublic of Korea
- Language:English
-
Abstract:
PURPOSE: This study was performed to investigate the etiology, clinical features, and outcomes of patients with gonadotropin-independent precocious puberty (GIPP). METHODS: The study included 16 patients (14 female and 2 male patients) who manifested secondary sexual characteristics, elevated sex hormones, or adrenal androgens with prepubertal luteinizing hormone levels after gonadotropin releasing hormone stimulation diagnosed between May 1994 and December 2015. Patients with congenital adrenal hyperplasia were excluded. Clinical features, laboratory findings, treatment modalities, and outcomes were retrospectively reviewed. RESULTS: The median age at diagnosis was 2.6 years (range, 0.7–7.9 years) and median follow-up duration was 4.6 years (range, 1 month–9.8 years). Patients with McCune-Albright syndrome (n=5) and functional ovarian cysts (n=4) presented with vaginal bleeding and elevated estradiol levels (23.3±17.5 pg/mL); adrenocortical tumors (n=4) with premature pubarche and elevated dehydroepiandrosterone sulfate levels (87.2–6,530 µg/dL); and human chorionic gonadotropin (hCG)-producing tumor (n=1) with premature pubarche and elevated β-human chorionic gonadotropin levels (47.4 mIU/mL). Two patients were idiopathic. Six patients transited to gonadotropin-dependent precocious puberty median 3.3 years (range, 0.3–5.1 years) after the onset of GIPP. Initial and follow-up height standard deviation scores (0.99±0.84 vs. 1.10±1.10, P=0.44) and bone age advancement (1.49±1.77 years vs. 2.02±1.95 years, P=0.06) were not significantly different. CONCLUSION: The etiologies of GIPP are heterogeneous, and treatment and prognosis is quite different according to the etiology. Efficacy of treatment with aromatase inhibitors needs to be evaluated after long-term follow-up.
