Central precocious puberty as a prelude to hypogonadism in a patient with Klinefelter syndrome

© 2019 Chinese Medical Association. Pediatric Investigation published by John Wiley & Sons Australia, Ltd on behalf of Futang Research Center of Pediatric Development..

INTRODUCTION: Incomplete pubertal development is a common phenomenon found in patients with Klinefelter syndrome (KFS). KFS combined with central precocious puberty (CPP) rarely occurs. We herein report a rare case of KFS with CPP and review several studies to determine the possible mechanism underlying this condition.

CASE PRESENTATION: An 8-year, 5-month-old male patient was admitted to our hospital because of enlargement of the penis and small testes. Laboratory evaluation revealed high luteinizing hormone and follicle-stimulating hormone levels, a high testosterone level, and the 48,XXYY karyotype. He was treated with triptorelin. One year later, the patient's testosterone level decreased and pubertal arrest occurred.

CONCLUSION: The literature review in this study showed that the occurrence of hypogonadism combined with CPP is not unique to patients with KFS; it has also been reported in individuals with Turner syndrome, adrenal hypoplasia congenita, and other diseases. Such individuals share common features including partial hypogonadism and a normal hypothalamic-pituitary-gonadal axis. CPP is considered a prelude to hypogonadism, and treatment with a gonadotropin-releasing hormone agonist may prevent gonadal failure.

Medienart:

E-Artikel

Erscheinungsjahr:

2019

Erschienen:

2019

Enthalten in:

Zur Gesamtaufnahme - volume:3

Enthalten in:

Pediatric investigation - 3(2019), 2 vom: 01. Juni, Seite 127-130

Sprache:

Englisch

Beteiligte Personen:

Gong, Chunxiu [VerfasserIn]
Li, Lele [VerfasserIn]
Chen, Jiahui [VerfasserIn]
Li, Wenjing [VerfasserIn]

Links:

Volltext

Themen:

48,XXYY
Case Reports
Central precocious puberty
Hypogonadism
Klinefelter syndrome
Turner syndrome

Anmerkungen:

Date Revised 29.03.2024

published: Electronic-eCollection

Citation Status PubMed-not-MEDLINE

doi:

10.1002/ped4.12136

funding:

Förderinstitution / Projekttitel:

PPN (Katalog-ID):

NLM31424591X