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未经激素治疗的重型地中海贫血患者的成人身高

Adult height in thalassaemia major without hormonal treatment.

作者信息

De Luca F, Simone E, Corona G, Pandullo E, Siracusano M F, Arrigo T

机构信息

Istituto di Clinica Pediatrica, Università di Messina, Italy.

出版信息

Eur J Pediatr. 1987 Sep;146(5):494-6. doi: 10.1007/BF00441601.

Abstract

Height and sitting height were measured in all the 28 patients (19 females and 9 males) of our Thalassaemic Centre who exhibited evidence of cessation of growth. Their average final stature, which had been spontaneously achieved at an age ranging from 15.0-21.5 years (mean 17.9), was slightly lower with respect to the mean standards for women (159.1 +/- 8.4 vs 160.0 +/- 6.0 cm) and men (167.8 +/- 6.4 vs 172.5 +/- 6.0 cm). Height deficiency exceeded 2 SD scores in only 3/28 patients. If compared to the familial target height, patients' stature fell within the familial range in 19/24 cases and in only one case was it below the lower limit. In the whole sample the patients' height was positively related to their familial target height (r = 0.72, P less than 0.0002) and also to mid-parental stature (r = 0.45, P less than 0.05). Heights of both females and males were superimposable on those of their own mothers and fathers respectively. Average values of either SH (76.4 +/- 3.8 vs 79.9 +/- 3.4 cm, 2 P less than 0.02) or SH/H ratio (48.9 +/- 2.5 vs 51.5 +/- 1.0, 2 P less than 0.005) were significantly lower in the female thalassaemics than in the sex-matched controls. Three females and one male showed subnormal values of both SH and SH/H ratio. Two out of these patients with eunochoid body proportions and another one with a decreased SH/H ratio have hypogonadotropic hypogonadism and are undergoing a chronic substitutive treatment with sexual hormones, which was instituted after the achievement of final stature.

摘要

我们地中海贫血中心的28例(19例女性和9例男性)出现生长停滞迹象的患者均测量了身高和坐高。他们的平均最终身高在15.0至21.5岁(平均17.9岁)时自然达到,与女性(159.1±8.4 vs 160.0±6.0 cm)和男性(167.8±6.4 vs 172.5±6.0 cm)的平均标准相比略低。身高不足超过2个标准差分数的仅3/28例患者。与家族目标身高相比,患者身高在19/24例中处于家族范围内,仅1例低于下限。在整个样本中,患者身高与家族目标身高呈正相关(r = 0.72,P < 0.0002),也与父母平均身高呈正相关(r = 0.45,P < 0.05)。女性和男性的身高分别与他们自己的母亲和父亲的身高重叠。女性地中海贫血患者的坐高(76.4±3.8 vs 79.9±3.4 cm,P < 0.02)或坐高/身高比(48.9±2.5 vs 51.5±1.0,P < 0.005)的平均值均显著低于性别匹配的对照组。3例女性和1例男性的坐高和坐高/身高比均低于正常水平。这些患者中有2例具有类无睾体型,另1例坐高/身高比降低,患有低促性腺激素性性腺功能减退,在达到最终身高后开始接受性激素的慢性替代治疗。

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