Onset and progression of puberty in Klinefelter syndrome.
Klinefelter syndrome
androgens
puberty
sex chromosome disorder
spermatogenesis
testis
testosterone
Journal
Clinical endocrinology
ISSN: 1365-2265
Titre abrégé: Clin Endocrinol (Oxf)
Pays: England
ID NLM: 0346653
Informations de publication
Date de publication:
03 2022
03 2022
Historique:
revised:
29
07
2021
received:
30
03
2021
accepted:
26
08
2021
pubmed:
16
9
2021
medline:
21
4
2022
entrez:
15
9
2021
Statut:
ppublish
Résumé
Klinefelter syndrome (KS) (47,XXY and variants, KS) is the most common sex chromosome disorder in humans. However, little is known about the onset and progression of puberty in patients with KS. In this study, we describe the onset and progression of puberty in a large series of boys with KS in a single tertiary centre. Retrospective data (Tanner stages, testicular length, testosterone supplementation, levels of luteinizing hormone [LH] and testosterone) before possible testosterone treatment on 72 KS patients with 47,XXY karyotype were reviewed, and G (n = 59 patients) and P (n = 56 patients) stages were plotted on puberty nomograms. One boy had a delayed onset of puberty, as he was at the G1 stage at the age of 13.8 years (-2.2 SDs). No observations of delay were made of boys at Stage G2. The progression of G stages was within normal limits in the majority of patients; only few boys were late at G3 (4.1%; 1 out of 24) and G4 (7.4%; 2 out of 27). Testosterone supplementation was started at the average age of 15.5 years to 35 boys (47%), 2 of whom were over 18 years old. LH level was on average 18.2 IU/L (SD: 6.3 IU/L) and testosterone 9.1 nmol/L (SD: 3.1 nmol/L) when testosterone supplementation was started. Our results suggest that puberty starts within the normal age limits in boys with KS, and testosterone supplementation is not needed for the initial pubertal progression in the majority of patients.
Substances chimiques
Testosterone
3XMK78S47O
Luteinizing Hormone
9002-67-9
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
363-370Informations de copyright
© 2021 The Authors. Clinical Endocrinology published by John Wiley & Sons Ltd.
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