Differences in follicle-stimulating hormone secretion between 45,X monosomy Turner syndrome and 45,X/46,XX mosaicism are evident at an early age.
J Clin Endocrinol Metab
; 91(12): 4896-902, 2006 Dec.
Article
em En
| MEDLINE
| ID: mdl-16968797
ABSTRACT
CONTEXT Little information exists regarding FSH values in very young girls with Turner syndrome (TS). OBJECTIVES:
The objective of the study was to evaluate the pattern, natural progression, and karyotype-related differences in FSH secretion in young, prepubertal girls with TS. STUDYDESIGN:
FSH was measured at study entry and annually for 2 yr.SETTING:
The Toddler Turner study was conducted at 11 U.S. pediatric endocrine centers. STUDYPARTICIPANTS:
Eighty-eight girls with karyotype-proven TS aged 9 months to 4 yr participated in the study. MAIN OUTCOMEMEASURES:
By-karyotype differences in FSH concentration and age-related changes in FSH were measured.RESULTS:
Mean (+/- SD) FSH was markedly elevated in the 45,X (n = 56 68.3 +/- 36.0 IU/liter) and Other groups [n = 15 (excluding three subjects with Y-containing karyotypes) 52.7 +/- 50.8 IU/liter] but was minimally elevated in girls with 45,X/46,XX mosaicism (n = 14 10.1 +/- 13.5 IU/liter, P < 0.005 both comparisons). Over the 2-yr period, FSH declined in the 45,X group (-13.4 IU/liter.yr, P < 0.0001). Nonetheless, only three of 159 FSH values fell within normal range for age at any time during the 2-yr study. FSH decline was similar in the Other group (-14.3 IU/liter.yr, P = 0.0032). In contrast, no significant decrease in FSH with age was observed in the 45,X/46,XX group.CONCLUSIONS:
In contrast to the original report of FSH concentrations in individuals with TS, this study demonstrates distinct differences in patterns of FSH secretion between young girls with monosomy TS, who have persistent elevation of FSH to age 6 yr, and those with 45,X/46,XX mosaicism, whose FSH values suggest retained ovarian function in the majority. These findings have implications for patient management and family counseling.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Síndrome de Turner
/
Hormônio Foliculoestimulante
/
Mosaicismo
Tipo de estudo:
Clinical_trials
Limite:
Child
/
Child, preschool
/
Female
/
Humans
/
Infant
Idioma:
En
Revista:
J Clin Endocrinol Metab
Ano de publicação:
2006
Tipo de documento:
Article
País de afiliação:
Estados Unidos