Ann Pediatr Endocrinol Metab.  2016 Sep;21(3):136-142. 10.6065/apem.2016.21.3.136.

Etiology and therapeutic outcomes of children with gonadotropin-independent precocious puberty

Affiliations
  • 1Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Korea. hwyoo@amc.seoul.kr

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.

Keyword

Precocious puberty; Gonadotropin-independent; Etiology; Treatment outcome

MeSH Terms

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
Androgens
Aromatase Inhibitors
Chorionic Gonadotropin
Dehydroepiandrosterone Sulfate
Estradiol
Gonadal Steroid Hormones
Gonadotropin-Releasing Hormone
Luteinizing Hormone
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