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Onset and progression of puberty in Klinefelter syndrome
- Source :
- Clinical Endocrinology. 96:363-370
- Publication Year :
- 2021
- Publisher :
- Wiley, 2021.
-
Abstract
- Objective: 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. Design and Patients: 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. Measurements and Results: 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. Conclusions: 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.
- Subjects :
- Male
Delayed puberty
puberty
medicine.medical_specialty
Adolescent
Endocrinology, Diabetes and Metabolism
LUTEINIZING-HORMONE
030209 endocrinology & metabolism
testis
DIAGNOSIS
SERUM
03 medical and health sciences
Follicle-stimulating hormone
0302 clinical medicine
Endocrinology
INHIBIN B
TESTOSTERONE
Internal medicine
medicine
Humans
Klinefelter syndrome
Retrospective Studies
ADOLESCENT BOYS
030219 obstetrics & reproductive medicine
TESTICULAR FUNCTION
business.industry
Delayed onset
androgens
Testosterone (patch)
DELAYED PUBERTY
Luteinizing Hormone
Nomogram
FOLLICLE-STIMULATING-HORMONE
medicine.disease
spermatogenesis
3121 General medicine, internal medicine and other clinical medicine
Female
sex chromosome disorder
HYPOGONADISM
medicine.symptom
Luteinizing hormone
business
Spermatogenesis
Subjects
Details
- ISSN :
- 13652265 and 03000664
- Volume :
- 96
- Database :
- OpenAIRE
- Journal :
- Clinical Endocrinology
- Accession number :
- edsair.doi.dedup.....671c79f41bdb7262fb8ee6153698cfc6