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 |
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Erscheinungsjahr: |
2019 |
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Erschienen: |
2019 |
Enthalten in: |
Zur Gesamtaufnahme - volume:3 |
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Enthalten in: |
Pediatric investigation - 3(2019), 2 vom: 01. Juni, Seite 127-130 |
Sprache: |
Englisch |
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Beteiligte Personen: |
Gong, Chunxiu [VerfasserIn] |
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Links: |
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Themen: |
48,XXYY |
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Anmerkungen: |
Date Revised 29.03.2024 published: Electronic-eCollection Citation Status PubMed-not-MEDLINE |
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doi: |
10.1002/ped4.12136 |
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funding: |
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Förderinstitution / Projekttitel: |
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PPN (Katalog-ID): |
NLM31424591X |
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520 | |a © 2019 Chinese Medical Association. Pediatric Investigation published by John Wiley & Sons Australia, Ltd on behalf of Futang Research Center of Pediatric Development. | ||
520 | |a 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 | ||
520 | |a 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 | ||
520 | |a 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 | ||
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