An 11-month-old girl with central precocious puberty caused by hypothalamic hamartoma

Abstract

Central precocious puberty (CPP) is caused by premature activation of the hypothalamic-gonadal axis, and must be treated adequately. In particular, CPP that occurs at a relatively young age or in boys is likely to be caused by an organic lesion. Hypothalamic hamartoma (HH) is the most common organic cause of CPP. The present case report describes an 11-month-old female infant who presented with vaginal bleeding and rapidly progressive secondary sex characteristics from the age of 6 months. She was diagnosed with CPP following the detection of HH via magnetic resonance imaging. The infant girl was successfully treated with gonadotropin-releasing hormone agonist. After 6 months, her breast had regressed and clinical and radiological follow-up demonstrated stable findings with no evidence of tumor growth or secondary sexual characteristics until the fourth year after the initiation of treatment. This patient is the one of the youngest infants presenting with CPP and HH in Korea; treatment was successful over a relatively long follow-up period.

Hipotalamik hamartoma bağlı santral puberte prekoks ve faktör 7 eksikliği birlikteliği

“…Sonuç olarak, anormal endokrin fonksiyonunu tetikleyen aşırı GnRH salgılanması, tubersinerium yoluyla hipotalamusa aktarılmaktadır. (4,6) Hipotalamik hamartom teşhisi için en uygun tetkik, yüksek çözünürlüklü beyin MRG'dir. HH semptomları, HH'nin konumu ve hipotalamus gibi çevre dokularla olan bağlantıları ile yakından ilişkilidir.…”

“…GnRH analog tedavisi ile sürekli pituiter uyarı, yüksek GnRH seviyeleri, persistan resetör sensitizasyonu, hipotalamo pitüiter gonadal aksı baskılayarak pubertal bulguları durdurmakta, büyüme hızını normale çekip iskelet olgunlaşmasını yavaşlatarak erişkin final boyun korunmasını sağlamaktadır (7). Literatürde HH vaka seri-lerinde GnRH ile etkin medikal tedaviyi bildirilmiştir (4,10). Kotwal ve ark.…”