Differences in Infantile Growth Patterns in Turner Syndrome Girls with and without Spontaneous Puberty

Abstract

Objective: The role of prepubertal estrogen in child growth was modeled using Turner’s syndrome, comparing growth patterns of girls who later did or did not enter puberty spontaneously. The hypothesis was that TS patients with normal prepubertal estrogen levels would have a different growth pattern from those with subnormal estrogen levels. Study Design: Growth data from 78 full-term patients with Turner’s syndrome were collected retrospectively. 24/78 later developed spontaneous puberty, (+ Pub), and their growth data were compared to TS patients without spontaneous puberty (- Pub). A nonlinear mixed model was fitted using the bi-exponential model. Results: The growth velocity difference between the - Pub and + Pub groups suggests an early infantile growth advantage in the - Pub group, which disappears before the end of the first year of life; growth velocity remains similar (± 1 cm/y) for the next 6 years and declines at age 7 - 8 years in the + Pub group faster than it does in the - Pub group. Bi-exponential analysis showed that both the 1^st (restrictive) and 2^nd exponent (forward) were different (p = 0.0003). Conclusions: Comparison of girls with or without spontaneous puberty suggests a role for estrogen in child growth. Estrogens restrict infantile growth, as well as growth during the mid-childhood spurt.

Turner’s syndrome - Child growth · Female · Male · Sex steroids

References

• 1 Klein K O, Baron J, Colli M J, McDonnell D P, Cutler G B Jr. Estrogen levels in childhood determined by an ultrasensitive recombinant cell bioassay.  J Clin Invest. 1994;  33 2475-2480
• 2 Lampit M, Golander A, Guttmann H, Hochberg Z. Estrogen mini-dose replacement during GnRH agonist therapy in central precocious puberty: a pilot study.  J Clin Endocrinol Metab. 2002;  87 687-690
• 3 Ranke M B, Pfluger H, Rosendahl W, Stubbe P, Enders H, Bierich J R, Majewski F. Turner’s syndrome: spontaneous growth in 150 cases and review of the literature.  Euro J Pediatr. 1983;  141 81-88
• 4 Lyon A J, Preece M A, Grant D B. Growth curve for girls with Turner’s syndrome.  Arch Dis Child. 1985;  60 932-935
• 5 Even L, Cohen A, Marbach N, Brand M, Kauli R, Sippell W, Hochberg Z. Longitudinal analysis of growth over the first three years of life in Turner’s syndrome.  J Pediatr. 2000;  137 460-4
• 6 Rao E, Weiss B, Fukami M, Rump A, Niesler B, Mertz A. et al . Pseudoautosomal deletions encompassing a novel homeobox gene cause growth failure in idiopathic short stature and Turner’s syndrome.  Nature Gen. 1997;  16 54-63
• 7 Ellison J W, Wardak Z, Young M F, Gehron-Robey P, Laig-Webster M. Chiong W. PHOG, a candidate gene for involvement in the short stature of Turner’s syndrome.  Hum Mol Gen. 1997;  6 1341-1347
• 8 Pasquino A M, Passeri F, Pucarelli I, Segni M, Municchi G. Spontaneous pubertal development in Turner’s syndrome.  J Clin Endocrinol Metab. 1997;  82 1810-1813
• 9 Hreinsson J G, Otala M, Fridstrom M, Borgstrom B, Rasmussen C, Lundqvist M, Tuuri T, Simberg N, Mikkola M, Dunkel L, Hovatta O. Follicles are found in the ovaries of adolescent girls with Turner’s syndrome.  J Clin Endocrinol Metab. 2002;  87 3618-3623
• 10 Wilson C A, Heinrichs C, Larmore K A, Craen M, Brown-Dawson J, Shaywitz S. et al . Estradiol levels in girls with Turner’s syndrome compared to normal prepubertal girls as determined by an ultrasensitive assay.  J Pediatr Endocrinol Metab. 2003;  16 91-96
• 11 Conte F A, Grumbach M M, Kaplan S L, Reiter E O. Correlation of luteinizing hormone-releasing factor-induced luteinizing hormone and follicle-stimulating hormone release from infancy to 19 years with the changing pattern of gonadotropin secretion in agonadal patients: relation to the restraint of puberty.  J Clin Endocrinol Metab. 1980;  50 163-168
• 12 Grumbach M M, Hughes I A, Conte F A. Disorders of sex differentiation. In: Larsen PR, DW, Kronenberg HM, Melmed S, Polonsky KS, (eds.) Williams Textbook of Endocrinlogy. 10^th ed. Philadelphia; WB Saunders 2003: 842-1002
• 13 Heinrichs C, Bourdoux P, Saussez C, Vis H L, Bourguignon J P. Blood spot follicle-stimulating hormone during early postnatal life in normal girls and Turner’s syndrome.  J Clin Endocrinol Metab. 1994;  78 978-981
• 14 Karlberg J. A biologically oriented mathematical model (ICP) for human growth.  Acta Paediat Scand (Suppl). 1987;  350 70-94
• 15 Barnard R, Haynes K M, Werther G A, Waters M J. The ontogeny of growth hormone receptors in the rabbit tibia.  Endocrinology. 1998;  122 2562-2569
• 16 Werther G A, Haynes K, Waters M J. Growth hormone (GH) receptors are expressed on human fetal mesenchymal tissues identification of messenger ribonucleic acid and GH-binding protein.  J Clin Endocrinol Metab. 1993;  76 1638-1646
• 17 Davidian M, Giltinan D M. Nonlinear Models for Repeated Measures Data. London; Chapman & Hall/CRC 1995
• 18 Silverman B W. Some aspects of the spline smoothing approach to nonparametric regression curve fitting.  J R Soc Stat B. 1985;  47 1-52
• 19 Amara J F, Dannies P S. 17 beta-Estradiol has a biphasic effect on GH cell growth.  Endocrinology. 1983;  112 1141-1143
• 20 Ho K K, O'Sullivan A J, Weissberger A J, Kelly J J. Sex steroid regulation of growth hormone secretion and action.  Horm Res. 1996;  45 67-73
• 21 Fishman A, Hertz P, Hochberg Z. Ontogenesis of the sexual dimorphism of growth hormone secretion by perifused rat hemipituitaries.  Neuroendocrinology. 1993;  57 782-788
• 22 Fukami M, Matsuo N, Hasegawa T, Sato S, Ogata T. Longitudinal auxological study in a female with SHOX (short stature homeobox containing gene) haploinsufficiency and normal ovarian function.  Eur J Endocrinol. 2003;  149 337-341