Background/Aim: Gonadotropin levels measured by radioimmunoassays are high in girls with Turner syndrome (TS), but overlap significantly with those of normal girls. We hypothesized that gonadotropin levels would be above the normal range in TS when measured by ultrasensitive assays. Methods: Follicle-stimulating hormone (FSH) and luteinizing hormone (LH) levels were measured in 68 TS, and 133 control girls using ultrasensitive immunochemiluminometric assays (ICMA). Results: FSH levels in TS and normal girls were highest in early childhood (56.0 ± 39.7 and 2.3 ± 1.8 IU/l, respectively), declined at 6–10 years of age (11.3 ± 13.1 and 1.8 ± 0.9 IU/l, respectively), and then increased again (104.4 ± 68.9 and 4.9 ± 2.4 IU/l, respectively). FSH was in the normal range on 11 of 27 occasions in TS girls with ages 5–10 years, and on 3 of 44 occasions in >10 years. Although average LH values were higher than those of controls, they often overlapped the normal range. Conclusion: A significant number of TS girls have normal gonadotropins by ICMA. Spontaneous gonadotropin levels are not an adequate screening test for the diagnosis of TS but may prove useful for predicting the gonadal function and determining the appropriate timing of estrogen replacement therapy.

Keywords:
Follicle-stimulating hormone, Luteinizing hormone, Turner syndrome, Gonadal dysgenesis, X chromosome
1.
Davenport ML, Calikoglu AS: Turner syndrome; in Pescovitz OH, Eugster EA (eds): Pediatric Endocrinology: Mechanisms, Manifestations, and Management. Philadelphia, Lippincott Williams & Wilkins, 2004, pp 203–223.
2.
Sybert VP, McCauley E: Turner’s syndrome. N Engl J Med 2004;351:1227–1238.
3.
Elsheikh M, Dunger DB, Conway GS, Wass JA: Turner’s syndrome in adulthood. Endocr Rev 2002;23:120–140.
4.
Simpson JL, Rajkovic A: Ovarian differentiation and gonadal failure. Am J Med Genet 1999;89:186–200.
5.
Zinn AR: The X chromosome and the ovary. J Soc Gynecol Investig 2001;8:S34–S36.
6.
Vialard F, Cocquet J, Christin-Maitre S, Veitia R, Fellous M: The X chromosome and ovarian function. Cytogenet Genome Res 2002;99:218–223.
7.
Pasquino AM, Passeri F, Pucarelli I, Segni M, Municchi G: Spontaneous pubertal development in Turner’s syndrome. Italian Study Group for Turner’s Syndrome. J Clin Endocrinol Metab 1997;82:1810–1813.
8.
Lippe B, Westra SJ, Boechat MI: Ovarian function in Turner syndrome: recognizing the spectrum; in Hibi I, Takano K (eds): Basic and Clinical Approach to Turner Syndrome. Elsevier Science, 1993, pp 117–122.
9.
Ross JL, Loriaux DL, Cutler GB Jr: Developmental changes in neuroendocrine regulation of gonadotropin secretion in gonadal dysgenesis. J Clin Endocrinol Metab 1983;57:288–293.
10.
Hosoda A, Fujieda K, Matsuura N, Okuno A, Yuri K: Age-related change of pulsatile gonadotropin secretion in Turner syndrome. Pediatr Res 1991;29:196–200.
11.
Mauras N, Rogol AD, Veldhuis JD: Specific, time-dependent actions of low-dose ethinyl estradiol administration on the episodic release of growth hormone, follicle-stimulating hormone, and luteinizing hormone in prepubertal girls with Turner’s syndrome. J Clin Endocrinol Metab 1989;69:1053–1058.
12.
Nathwani NC, Hindmarsh PC, Massarano AA, Brook CG: Gonadotrophin pulsatility in girls with the Turner syndrome: modulation by exogenous sex steroids. Clin Endocrinol 1998;49:107–113.
13.
Conte FA, Grumbach MM, Kaplan SL: A diphasic pattern of gonadotropin secretion in patients with the syndrome of gonadal dysgenesis. J Clin Endocrinol Metab 1975;40:670–674.
14.
Penny R, Guyda HJ, Baghdassarian A, Johanson AJ, Blizzard RM: Correlation of serum follicular-stimulating hormone and luteinizing hormone as measured by radioimmunoassay in disorders of sexual development. J Clin Invest 1970;49:1847–1852.
15.
Winter JS, Faiman C: Serum gonadotropin in concentrations in agonadal children and adults. J Clin Endocrinol Metab 1972;35:561–564.
16.
Neely EK, Wilson DM, Lee PA, Stene M, Hintz RL: Spontaneous serum gonadotropin concentrations in the evaluation of precocious puberty. J Pediatr 1995;127:47–52.
17.
Neely EK, Hintz RL, Wilson DM, Lee PA, Gautier T, Argente J, Stene M: Normal ranges for immunochemiluminometric gonadotropin assays. J Pediatr 1995;127:40–46.
18.
Brown DC, Stirling HF, Butler GE, Kelnar CJ, Wu FC: Differentiation of normal male prepuberty and hypogonadotrophic hypogonadism using an ultrasensitive luteinizing hormone assay. Horm Res 1996;46:83–87.
19.
Eckert KL, Wilson DM, Bachrach LK, Anhalt H, Habiby RL, Olney RC, Hintz RL, Neely EK: A single-sample, subcutaneous gonadotropin-releasing hormone test for central precocious puberty. Pediatrics 1996;97:517–519.
20.
Sequera AM, Fideleff HL, Boquete HR, Pujol AB, Suarez MG, Ruibal GF: Basal ultrasensitive LH assay: a useful tool in the early diagnosis of male pubertal delay? J Pediatr Endocrinol Metab 2002;15:589–596.
21.
Ropelato MG, Escobar ME, Gottlieb S, Bergada C: Gonadotropin secretion in prepubertal normal and agonadal children evaluated by ultrasensitive time-resolved immunofluorometric assays. Horm Res 1997;48:164–172.
22.
Savendahl L, Davenport ML: Delayed diagnoses of Turner’s syndrome: proposed guidelines for change. J Pediatr 2000;137:455–459.
23.
Chernausek SD, Attie KM, Cara JF, Rosenfeld RG, Frane J: Growth hormone therapy of Turner syndrome: the impact of age of estrogen replacement on final height. Genentech Inc. Collaborative Study Group. J Clin Endocrinol Metab 2000;85:2439–2445.
24.
Hogler W, Briody J, Moore B, Garnett S, Lu PW, Cowell CT: Importance of estrogen on bone health in Turner syndrome: a cross-sectional and longitudinal study using dual-energy X-ray absorptiometry. J Clin Endocrinol Metab 2004;89:193–199.
25.
Khastgir G, Studd JW, Fox SW, Jones J, Alaghband-Zadeh J, Chow JW: A longitudinal study of the effect of subcutaneous estrogen replacement on bone in young women with Turner’s syndrome. J Bone Miner Res 2003;18:925–932.
26.
Paterson WF, Hollman AS, Donaldson MD: Poor uterine development in Turner syndrome with oral oestrogen therapy. Clin Endocrinol (Oxf) 2002;56:359–365.
27.
Piippo KS, Lenko H, Kainulainen P, Sipila I: Use of percutaneous estrogen gel for induction of puberty in girls with turner syndrome. Obstet Gynecol Surv 2005;60:102–103.
28.
Elsheikh M, Bird R, Casadei B, Conway GS, Wass JA: The effect of hormone replacement therapy on cardiovascular hemodynamics in women with Turner’s syndrome. J Clin Endocrinol Metab 2000;85:614–618.
29.
Gravholt CH: Turner syndrome and the heart: cardiovascular complications and treatment strategies. Am J Cardiovasc Drugs 2002;2:401–413.
30.
Kalantaridou SN, Naka KK, Papanikolaou E, Kazakos N, Kravariti M, Calis KA, Paraskevaidis EA, Sideris DA, Tsatsoulis A, Chrousos GP, Michalis LK: Impaired endothelial function in young women with premature ovarian failure: normalization with hormone therapy. J Clin Endocrinol Metab 2004;89:3907–3913.
31.
Rosenfield RL, Devine N, Hunold JJ, Mauras N, Moshang T Jr, Root AW: Salutary effects of combining early very low-dose systemic estradiol with growth hormone therapy in girls with Turner syndrome. J Clin Endocrinol Metab 2005;90:6424–6430.
32.
Ankarberg-Lindgren C, Elfving M, Wikland KA, Norjavaara E: Nocturnal application of transdermal estradiol patches produces levels of estradiol that mimic those seen at the onset of spontaneous puberty in girls. J Clin Endocrinol Metab 2001;86:3039–3044.
33.
Abir R, Fisch B, Nahum R, Orvieto R, Nitke S, Ben Rafael Z: Turner’s syndrome and fertility: current status and possible putative prospects. Hum Reprod Update 2001;7:603–610.
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2006
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