Abstract

BackgroundData on adrenarche and pubarche in girls with Turner syndrome (TS) are inconsistent in the literature.MethodsThe cohort consisted of 94 girls and young women with TS born between 1971 and 2001 (age range: 3.1–23.2 yrs.), who were treated with human growth hormone and regularly presented at our outpatient clinic every 4 to 6 months.The longitudinal data of all patients were ascertained retrospectively from patient charts. The data collection ended in January 2016. Adrenarche was assessed by serum DHEAS levels and pubertal status by Tanner stages. Pubarche was defined as the appearance of pubic hair (PH2), whereas spontaneous puberty was defined as Tanner stage B2. The patients were retrospectively subdivided in two groups with regard to pubertal development: group 1 (n = 21) with spontaneous puberty and group 2 (n = 70) with induced puberty. Since blood samples were not taken at every visit, we generated seven groups according to the age of the children at which the blood samples were taken: 3–5, 5–7, 7–9, 9–11, 11–13, 13–15, and 15–17 yrs. Serum DHEAS and follicle-stimulating hormone (FSH) levels were measured by chemiluminescence immunoassay and compared with those of a control group of healthy girls.ResultsAdrenarche started in TS girls between 5 and 7 years. TS girls had higher DHEAS levels than the control group, with statistically significant differences in the age groups 7 to 17 years. No differences were determined between the TS girls with spontaneous puberty and those with POI. TS girls in group 2 reached the Tanner stages PH2 (p < 0.04), PH3 (p < 0.01), PH4 and PH5 (p < 0.001) markedly later than TS girls in group 1.ConclusionsThe onset of adrenarche in girls with TS undergoing GH therapy does not differ from that in healthy girls. However, adrenarche is more pronounced in girls with TS. There is no difference in DHEAS levels between the TS girls with spontaneous puberty and the TS girls with primary ovarian insufficiency (POI), while the tempo of pubarche is markedly slower in the girls with POI.

Highlights

  • Data on adrenarche and pubarche in girls with Turner syndrome (TS) are inconsistent in the literature

  • A case report suggested that growth hormone (GH) excess may result in premature adrenarche [10], whereas no changes in Dehydroepiandrosterone sulfate (DHEAS) levels were found in children with GH deficiency after 6 months of GH therapy [11], or in boys with non-GH deficient short stature [12]

  • DHEAS levels were elevated in all TS girls aged ≥5 years compared with the reference cohort

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Summary

Introduction

Data on adrenarche and pubarche in girls with Turner syndrome (TS) are inconsistent in the literature. Turner syndrome (TS) affects approximately 1 in 2500 live-born females. It is characterized by loss or structural anomalies of an X chromosome. Many factors have been identified in the regulation of adrenarche in the past, such as (2019) 19:9 normal ACTH secretion, increasing adrenal 17,20-lyase activity and serum IGF-1 (Insulin-like Growth Factor 1) concentrations [6,7,8]. A case report suggested that GH excess may result in premature adrenarche [10], whereas no changes in DHEAS levels were found in children with GH deficiency after 6 months of GH therapy [11], or in boys with non-GH deficient short stature [12]. There are still many unknown factors in the regulation of adrenarche

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