Abstract
Study Objective
This study identified the prevalence of menarche and coincident sexual characteristics
in female adolescents with Hutchinson-Gilford Progeria Syndrome (HGPS).
Design
Data were examined to determine the prevalence of menarche in female adolescents older
than 12 years; all were participants in clinical trials between 2007 and 2016.
Setting
Pediatric hospital in Boston, Massachusetts.
Participants
Fifteen female adolescents, median age 15 (range, 12.0-20.3) years with a confirmed
diagnosis of HGPS.
Interventions and Main Outcome Measures
Report of menarche, anthropometric and serum hormonal measures, Tanner pubertal staging,
and body composition using dual-energy x-ray absorptiometry.
Results
Nine of 15 (60%) participants reported spontaneous menarche at a median age of 14.4 years
(range, 12.0-16.5 years). In those experiencing menarche vs not, median age was older
(16.5 vs 13.6 years; P = .02), whereas body mass index did not differ (10.5 vs 10.4; P = .53) nor percentage body fat (19.4% vs. 19.3%; P = .98) or serum leptin levels (0.40 vs 0.40 ng/mL; P = .23). Among those who achieved menarche, 2 of 9 (22%) had Tanner II breast development
and 2 of 9 (22%) exhibited Tanner II Pubic hair, all reflecting minimal pubertal development.
Only early signs of puberty were similarly seen in the non-menstruating group, including
1 of 6 (17%) with Tanner II breasts and 2 of 6 (33%) with Tanner II pubic hair, and
Tanner staging did not differ between those who reported menarche vs those who did
not (each P = 1.0). None of the participants achieved Tanner IV or V thelarche over the course
of the study.
Conclusion
Menarche was achieved in more than half of adolescent girls with HGPS, in the setting
of little to no physical signs of pubertal development and minimal body fat.
Key Words
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Article info
Publication history
Published online: December 16, 2017
Footnotes
The authors indicate no conflicts of interest.
Identification
Copyright
© 2017 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc.
