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[Turner syndrome: spontaneous growth of stature, weight increase and accelerated bone maturation].

作者信息

Cabrol S, Saab C, Gourmelen M, Raux-Demay M C, Le Bouc Y

机构信息

Laboratoires d'explorations fonctionnelles endocriniennes, hôpital Armand-Trousseau, Paris, France.

出版信息

Arch Pediatr. 1996 Apr;3(4):313-8. doi: 10.1016/0929-693x(96)84683-5.

DOI:10.1016/0929-693x(96)84683-5
PMID:8762950
Abstract

BACKGROUND

Since growth hormone is effective in increasing the height of girls with Turner's syndrome, it is important to dispose of growth and bone maturation curves in a large number of untreated patients.

POPULATION AND METHODS

Data on growth and bone maturation were collected from 160 patients with Turner's syndrome (50 have reached final height), born 1965-1991, untreated with growth hormone or anabolic steroids. X monosomy was found in half of the patients, mosaicism or X abnormality was present in the other half. Spontaneous puberty occurred in 25% (n = 25) of patients older than 13 years, 38 patients received estrogen after 13 years. Final heights were compared to predicted height according to Lyon's method.

RESULTS

Forty-five percent of patients were small for date. Height velocity decreased from 2 years of age and decreased faster during adolescence, when gonadal dysgenesis occurred. Bone maturation velocity decreased also during adolescence. Excessive weight appeared after the age of 5 years. Patients with partial deletion of the long arm of X (n = 6) were taller than the other girls (n = 44) (mean +/- DS) 152.5 +/- 3.1 cm, range 150-158 cm versus 142.5 +/- 4.9 cm, 130-150 cm (P < 0.0001). Final height was not modified by spontaneous puberty. Final height was correlated with birth weight (r = 0.7), maternal height (r = 0.5) and mid parental height (r = 0.5). Finally, the Lyon's method for predicted final height seemed to be suitable for this population, (r = 0.8, P < 0.001).

CONCLUSION

Appropriate growth curve is an essential clinical tool in evaluating treatment aimed at increasing final stature in patients with Turner's syndrome.

摘要

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Arch Pediatr. 1996 Apr;3(4):313-8. doi: 10.1016/0929-693x(96)84683-5.
2
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Noonan syndrome and Turner syndrome patients respond similarly to 4 years' growth-hormone therapy: longitudinal analysis of growth-hormone-naïve patients enrolled in the NordiNet® International Outcome Study and the ANSWER Program.努南综合征和特纳综合征患者对4年生长激素治疗的反应相似:对参加NordiNet®国际结果研究和ANSWER项目的未接受过生长激素治疗的患者的纵向分析。
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