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1.
目的观察促性腺激素释放激素类似物(GnRHa)和甲孕酮用于治疗真性性早熟女孩,对其抑制性发育,减慢骨成熟和生长速度,改善成人期预测身高的作用。方法分别使用两种药物治疗两组特发性真性性早熟女孩各9例,时间6~12个月,观察治疗前后的身高、性发育情况、骨龄、成人期预测身高等,并进行综合比较。结果两组患儿经治疗后,性发育情况大多数得到抑制。甲孕酮治疗组骨龄年增长为11岁,身高年增长为76cm,成人期预测身高治疗前后无改变。长效GnRHa组骨龄年增长为02岁,身高年增长为56cm,成人期预测身高治疗6个月时增长31cm,较治疗前明显改善(P<001),治疗1年时增长64cm,较治疗6个月时更为明显(P<005)。结论长效GnRHa与甲孕酮相比较,除可抑制性发育进程外,还可有效减慢骨成熟和生长速度,最终改善成人期预测身高,治疗时间愈长,效果愈明显  相似文献   

2.
Three girls and one boy with idiopathic precocious puberty and one boy with operated suprasellar hamartoma and progressing precocious puberty were treated with Cyproterone acetate for periods ranging from 15 months to 4 years and 6 months. In all children, the signs of puberty were reduced. Height velocity, as related to bone age, was increased, as was the ratio of height age increment to bone age increment. An improvement of adult height prognosis in precocious puberty has been achieved by Cyproterone acetate.  相似文献   

3.
目的探讨真性特发性性早熟女童三种不同类型胰岛素样生长因子1(IGF1)、骨龄(BA)及骨密度(BD)的变化。方法真性特发性性早熟女童196例,分为快速进展型、缓慢变化型及生长相对迟缓型,分别检测不同类型性早熟女童IGF1、BA及BD,并进行三种类型间比较,观察其骨生长及骨代谢的变化。结果快速进展型性早熟女童其IGF1、BA显著高于缓慢变化型及生长相对迟缓型女童(P〈0.05);缓慢变化型女童IGF1及BA高于生长相对迟缓型患儿(P〈0.05),生长相对迟缓型患儿其IGF1明显降低,甚至低于同年龄女童正常值,BA与实际年龄相当,BD在三种类型患儿间差异无统计学意义。结论快速进展型女童存在明显的骨生长加速、成熟提前,生长潜力缩短;缓慢变化型女童尽管也存在骨生长、成熟加速,但生长速度、骨成熟进程相对较缓;生长相对迟缓型女童骨生长迟滞,呈现出性腺轴与生长轴分离现象。  相似文献   

4.
ABSTRACT. Sitting height (SH) and sub-ischial leg length (SLL) were measured in 10 boys and 16 girls with precocious puberty; the patients were aged from 1.5 to 13.4 years at the time. Standard deviation scores (SDS) calculated for chronological age and bone age showed higher scores for SH than for SLL in all but two patients, both girls: the differences between the SDS for SH and SLL were more marked in the boys. The findings indicate that growth of the trunk is usually greater than growth of the legs in precocious puberty, particularly in boys.  相似文献   

5.
Vanderschueren-Lodeweyckx, M., Van den Broeck, J., Wolter, R. and Malvaux, P. (Department of Paediatric and Adolescent Endocrinology, University of Leuven, Department of Paediatrics, University of Brussels and Department of Paediatrics, University of Louvain, Belgium). Early initiation of growth hormone treatment: influence on final height. Acta Paediatr Scand [Suppl] 337:4, 1987.
Retrospective growth data for 34 hGH deficient patients who had been treated for at least 3 years with hGH were analysed in a Belgian multicentre study. Final height was related to target height and was usually below it, hut it was not determined by chronological or bone ages, bone age delay, height velocity before or during therapy, nor by duration of treatment. Total height gain during long-term substitution with hGH is inversely related to chronological and hone ages at the start of therapy, and is positively related to the duration of therapy. Early diagnosis of hGH deficiency is thus important, as it allows catch-up growth to optimal height before puberty, which in turn results in a good pubertal growth spurt.  相似文献   

6.
We examined 22 girls and 11 boys with idiopathic precocious puberty (IPP) treated with a GnRH analogue for a period of about 4 y. The purpose of our study was to evaluate possible differences between the two sexes in bone growth and skeletal maturation during treatment and in the achievement of final height, and also to study the relative contribution of particular hormones–sex steroids, DHEAS, GH and IGF-I–during the pubertal growth spurt. At the beginning of therapy mean chronological age (CA) was 7.61 ± 0.84 y in boys and 7.32 ± 1.06 y in girls. After the first year of treatment, growth velocity and Dbone age/Dchronological age (ΔBA/ΔCA) ratio had declined significantly in both groups. At the end of therapy we observed a statistically relevant increase in predicted adult height in both sexes, with a more appreciable mean gain (expressed as SDS) being achieved by male patients. During the first year following discontinuation of treatment, a significant increase in the ΔBA/ΔCA ratio was observed in both males and females; by contrast, growth velocity increased only in male patients. Adult height SDS was thus greater in boys (0.13 ± 0.91) than in girls (-0.62 ± 0.88,p < 0:05). With regard to endocrinological data, oestradiol and testosterone were significantly reduced during the first year of therapy, while DHEAS levels increased slightly in both sexes throughout the course of treatment. GH peak after clonidine and IGF-I concentrations remained unchanged in both groups. Also, a study of nocturnal GH secretion (10 subjects) showed no noteworthy decrease in any of the patients, whether in terms of mean GH, of the sum of pulse amplitudes, or of pulse frequency. In conclusion, our data indicate that boys achieve more significant results in terms of adult height than girls. With reference to endocrinological data, the effect of sex steroids on bone maturation seems to be more significant than previously thought, and we hypothesize a different role for androgens and oestrogens in regulating height velocity and bone maturation in both male and female subjects during pubertal growth spurt.  相似文献   

7.
Heinrichs C, Craen M, Vanderschueren-Lodeweyckx M, Malvaux P, Fawe L, Bourguignon JP. Variations in pituitary-gonadal suppression during intranasal buserelin and intramuscular depot-triptorelin therapy for central precocious puberty. Acta Pædiatr 1994;83:627–33. Stockholm. ISSN 0803–5253
This study evaluated pubertal development, growth and pituitary—gonadal suppression in 21 patients with central precocious puberty treated with buserelin intranasally and switched after a mean of 2.1 yr to depot-triptorelin given im for 1 year. Arrest or regression of puberty was observed in 12 patients while progression of puberty during therapy was seen in 9 patients (6 on buserelin, 2 on triptorelin and 1 on both therapies). The increment in serum LH and FSH concentrations after sc injection of short-acting triptorelin was greater on buserelin than on triptorelin therapy, particularly in patients with evidence of progression of puberty. Height velocity during therapy showed a reduction which paralleled the decelerating phase of the normal pubertal growth spurt. The rate of bone maturation during therapy was inversely related to pretreatment bone age. Predicted final height showed marked individual variations which were inversely related to predicted adult height before therapy. These data indicate that differences in the nature and route of administration of Gn-RH agonist therapy for central precocious puberty can be of importance for inhibition of pituitary gonadotropin secretion and development of secondary sex characteristics. Height velocity and bone maturation are age-related and the change in predicted adult height depends on pretreatment level.  相似文献   

8.
In order to evaluate the effects of two long-acting luteinizing hormone-releasing hormone agonists on growth, bone maturation and final height in girls with central precocious puberty, we analyzed growth data from 40 girls (15 treated with buserelin intranasal spray (group A), 15 treated with triptorelin depot im every 28 days (group B) and 10 untreated (group C)). Patients in group A started treatment when chronological age (CA) was 7.7 ± 0.9 years, bone age (BA) was 10.2 ± 1.1 years and height was 131.9 ± 5.0 cm. Patients in group B started therapy when CA was 7.6 ± 0.5 years, BA 9.8 ± 1.0 years and height 133.2 ± 7.6 m. The diagnosis of untreated patients (group C) was made when CA was 7.2 ± 0.9 years, BA 9.6 ± 2.2 years and height 130.2 ± 8.6cm. Both luteinizing hormone-releasing hormone agonists appeared to control precocious puberty. Final height in group B (160.6 ± 5.7 cm) was significantly higher than that of group A (153.2 ± 5.0 cm: p < 0.05) and group C (149.6 ± 6.3; p < 0 .01), whereas the difference between groups A and C was not statistically significant. In group B a positive difference was observed between final height (160.6 ± 5.7 cm) and target height (157.6 ± 5.9 cm) (ns); on the contrary, in groups A and C, final height was lower than target height (155.5 ± 5.3 and 156.4 ± 1.3cm, respectively), but only in group C the difference was statistically significant ( p < 0.01). The best results regarding final height obtained by slow-release depot im therapy may be associated with more stable agonist blood levels during treatment.  相似文献   

9.
An attempt was made to identify the less severe cases of precocious puberty and to describe their natural course. A group of 17 girls with precocious puberty and a bone age advance over chronological age of less than two years (group 1) was compared with a group of 19 patients with severe precocious puberty and bone age advance of two years or more (group 2). Mean (SEM) plasma oestradiol concentrations were 82 (30) pmol/l and 164 (21) pmol/l (p less than 0.05), vaginal maturation indexes were 16 (5) and 41 (4), and plasma somatomedin C concentrations were 1.0 (0.2) U/ml (n = 8) and 2.1 (0.3) U/ml (n = 16) in groups 1 and 2, respectively. The time between onset and diagnosis of secondary sexual characteristics was about one year in both groups. After two years'' follow up the untreated patients in group 1 had maintained their predicted final height. These changes were in contrast to those observed at first examination in patients in group 2 who had a mean (SD) predicted final height of -1.3 (0.2) and a mean bone age advance of 3.0 (0.2) years. These data show that bone age advance to chronological age, and plasma somatomedin C concentrations measured at initial evaluation are helpful in identifying less severe and potentially slow progressing forms of central precocious puberty.  相似文献   

10.
Growth hormone-insulin-like growth factor-I status and response to growth hormone therapy (0.6 IU/kg/week sc, six times a week for 12 months) were evaluated in 12 girls (chronological age 9.4 ± 1.6 years) suffering from central precocious puberty with growth velocity less than 4 cm/year and no substantial increase or decrease in predicted adult height during gonadotropin releasing hormone (Gn-RH) analogue treatment (D-Trp6-LH-RH, 60 μg/kg im/28 days). At baseline, large variations were observed in nocturnal growth hormone (GH) means (pathological values (< 3.6μg/l) 33.3%), stimulated levodopa GH peaks (pathological values (<10.0 μg/I) 28.6%) and serum insulin-like growth factor-I (IGF-I) levels. Neither GH nor IGF-I levels were correlated with growth velocity. During recombinant GH therapy, growth velocity increased significantly (baseline 3.0 ± 0.9 cm/year; 6 months 6.4 ± 1.9cm/year, p < 0.001 versus baseline; 12 months 6.0 ± 1.3cm/year, p < 0.001 versus baseline). There was a significant increase in height SDS for bone age (baseline –1.6 ±0.5 SDS; 12 months -1.04 ± 0.6SDS; p < 0.002) and in predicted adult height (baseline 152.0 ± 3.6cm; 12 months 155.9 ± 3.4cm; p < 0.002). Our results suggest that combined therapy with Gn-RH analogues and recombinant GH can improve growth velocity and predicted adult height in girls with central precocious puberty and impaired height prognosis during Gn-RH analogue treatment.  相似文献   

11.
Twenty-one boys with a height of 135 cm or less at onset of puberty were treated with a combination of GnRH analog and anabolic steroid hormone, and their pubertal height gain and adult height were compared with those of untreated 29 boys who enter puberty below 135 cm. The mean age at the start of treatment with a GnRH analog, leuprorelin acetate depot (Leuplin®) was 12.3 yr, a mean of 1.3 yr after the onset of puberty, and GnRH analog was administered every 3 to 5 wk thereafter for a mean duration of 4.1 yr. The anabolic steroid hormone was started approximately 1 yr after initiation of treatment with the GnRH analog. The mean pubertal height gain from onset of puberty till adult height was significantly greater in the combination treatment group (33.9 cm) than in the untreated group (26.4 cm) (p<0.0001). The mean adult height was significantly greater in the combination treatment group (164.3 cm) than in the untreated group (156.9 cm) (p<0.0001). The percentage of subjects with an adult height of 160 cm or taller was 90.5% (19/21) in the combination treatment group, and it was 13.8% (4/29) in the untreated group (p<0.0001). Since growth of the penis and pubic hair is promoted by the anabolic steroid hormone, no psychosocial problems arose because of delayed puberty. No clinically significant adverse events appeared. Combined treatment with GnRH analog and anabolic steroid hormone significantly increased height gain during puberty and adult height in boys who entered puberty with a short stature, since the period until epiphyseal closure was extended due to deceleration of the bone age maturation by administration of the GnRH analog and the growth rate at this time was maintained by the anabolic steroid hormone.  相似文献   

12.
Sitting height (SH) and sub-ischial leg length (SLL) were measured in 10 boys and 16 girls with precocious puberty; the patients were aged from 1.5 to 13.4 years at the time. Standard deviation scores (SDS) calculated for chronological age and bone age showed higher scores for SH than for SLL in all but two patients, both girls: the differences between the SDS for SH and SLL were more marked in the boys. The findings indicate that growth of the trunk is usually greater than growth of the legs in precocious puberty, particularly in boys.  相似文献   

13.
We evaluated height prognosis and therapeutic efficacy of long-term, combination therapy with gonadotropin releasing-hormone agonist and growth hormone (GH) in five children (three girls) with coexistent precocious puberty and GH deficiency. Their clinical characteristics and growth response were compared with those of 12 girls with idiopathic true precocious puberty and eight prepubertal GH-deficient children (one girl). Precocious GH-deficient subjects were older than the precocious GH-sufficient children (9.5 +/- 1.8 years vs 6.5 +/- 1.3 years; mean +/- SD), but bone ages were comparable (12 +/- 3.7 years vs 10 +/- 0.9 years); their chronologic age was similar to that of the prepubertal GH-deficient children (9.6 +/- 2.1 years), but bone age was significantly more advanced (6.9 +/- 2.3 years). The mean height velocity of the prepubertal GH-deficient children (3.8 +/- 1.5 cm/yr) was lower than that of the precocious GH-deficient subjects (6.7 +/- 1.6 cm/yr) and the precocious GH-sufficient children (9.5 +/- 2.9 cm/yr). Baseline adult height prediction z scores were significantly lower in the precocious GH-deficient children (-3.7 +/- 1.0) than in either the precocious GH-sufficient children (-2.2 +/- 1.0) or the prepubertal GH-deficient subjects (-1.5 +/- 0.8). During therapy with gonadotropin releasing-hormone agonist, growth rates slowed to an average of 3.7 cm/yr in the precocious GH-deficient children but increased after the addition of GH to 7.4 cm during the first year of combination therapy. After 2 to 3 years of combination therapy, height predictions increased an average of 10 cm, compared with an increase of 2.8 cm in the precocious GH-sufficient group treated with gonadotropin releasing-hormone agonist alone. We conclude that combination treatment with gonadotropin releasing-hormone agonist and GH improves the height prognosis of children with coexistent true precocious puberty and GH deficiency, but falls short of achieving normal adult height potential.  相似文献   

14.
ABSTRACT. Growth and skeletal maturation was assessed in 83 girls with central precocious puberty (CPP) during pituitary—gonadal suppression induced by treatment with a gonadotrophin-releasing hormone agonist (GnRHa). The mean pretreatment chronological age (CA) was 6.3 years and the mean bone age (BA) was 10.6 years. During the suppression of gonadal sex steroid secretion, mean height velocity (HV) decreased from a pretreatment value of 10.8 cm/year to 5.9 (year 1, n = 83), 4.9 (year 2, n = 72), 4.2 (year 3, n = 49, and 4.4 (year 4, n = 23) cm/year. During each interval, there was a negative correlation between HV and the pretreatment BA. In addition, the rate of skeletal maturation was reduced during GnRHa treatment (ΔBA/ΔCA = 0.6 ± 0.1 over 3 years, n = 45). The rate of skeletal maturation during therapy was also negatively correlated with pretreatment BA. Predicted adult stature, based upon z -scores of height for BA, increased significantly and progressively during therapy but the changes in height SDS for BA varied significantly. Since HV, ΔBA/ΔCA, and the change in height SDS for BA (ΔHT SDS for BA) during pituitary—gonadal suppression all correlated with the initial degree of skeletal maturation, the effect of GnRHa therapy on Final adult height in children with CPP will be best understood if growth data are assessed within a developmental framework.  相似文献   

15.
The aim of our study was to determine the pattern of female reproductive organ growth in Indian girls from birth to 18 years of age and to correlate the uterine length, mean ovarian volume (MOV) and Fundo Cervical Ratio (FCR) with chronological age, bone age and pubertal breast staging. A cross sectional study was performed on 218 girls from birth to 18 years of age. Height, weight, stage of puberty, X-ray for bone age and transabdominal ultrasounds were performed on all girls. Higher chronological age, bone age and increase in breast stage significantly predicted higher MOV (P < 0.001) and higher uterine length (P < 0.001). The MOV, uterine length and FCR are positively correlated with chronological age, bone age, height, weight and breast staging. Data from present study may be useful in screening cases of precocious puberty and other disorders that may need further evaluation.  相似文献   

16.
ABSTRACT. Statural growth and skeletal development were assessed in 87 girls with idiopathic central precocious puberty (CPP) during gonadotrophin-releasing hormone analogue (GnRHa)-induced suppression of gonadarche. Before the start of therapy, mean chronological age (CA) was 6.3 years and mean bone age (BA) was 10.6 years. During up to 6 consecutive years of complete suppression of gonadal sex steroid secretion, the mean height velocity decreased from 10.8 cm/year to prepubertal rates. At each interval height velocity was found to be inversely and negatively correlated with BA such that girls with advanced BAs grew at rates well below prepubertal norms but appropriately for their degree of skeletal maturation. Skeletal maturation similarly slowed during prolonged GnRHa administration (ABA/ACA = 0.6 ± 0.1 over 3 years, mean ± SD, n = 66) and was also negatively correlated with the BA before the start of therapy. Predicted adult height increased progressively during therapy; however, when analysed as changes in height SDS(BA), the impact of treatment was variable and correlated positively with the initial degree of skeletal maturation. The effect of GnRHa therapy on growth in children with CPP requires long-term study and is best analysed by employing a developmental perspective.  相似文献   

17.
Between 1979 and 1983, 129 children (95 girls) with precocious puberty were referred to the National Institutes of Health and received treatment for at least 6 months with the long-acting LHRH analogue D-Trp6-Pro9-NEt-LHRH. The majority (107 of 129) of the children had central precocious puberty mediated by activation of the hypothalamic-pituitary-gonadal axis in association with hypothalamic hamartomas (24 of 107) or other central nervous system lesions (21 of 107), or idiopathic precocious puberty (62 of 107). Hypothalamic hamartomas or other central nervous system lesions were a frequent cause of central precocious puberty in girls (27 of 87), but idiopathic precocious puberty was still the most frequent diagnosis (63%). Idiopathic precocious puberty was uncommon in boys (6%). The patients with peripheral precocious puberty included six girls with McCune-Albright syndrome and six boys with familial male precocious puberty. These children had peripheral sex steroid secretion in the absence of hypothalamic-pituitary-gonadal axis maturation. The children with combined peripheral and central precocious puberty included nine children with congenital adrenal hyperplasia and one girl with a virilizing adrenal tumor. In the patients with central precocious puberty or combined peripheral and central precocious puberty, LHRHa therapy caused suppression of gonadotropin and sex steroid levels (P less than 0.001), stabilization or regression of secondary sexual characteristics, and decreases in growth rate and in the rate of bone age maturation (P less than 0.005). Patients with peripheral precocious puberty, however, had no significant change in gonadotropin or sex steroid levels, growth rate, or the rate of bone age maturation, and no improvement in secondary sexual characteristics. Thus, LHRHa is an effective treatment of central precocious puberty and combined peripheral and central precocious puberty, but is ineffective in the therapy of peripheral precocious puberty.  相似文献   

18.
Linear growth and sexual maturation were assessed in 48 children during dialysis treatment and in 68 children following renal transplantation. Height at the onset of haemodialysis treatment was more than 2 SD below the mean in 33% of prepubertal children. During dialysis treatment most children showed a progressive deterioration in SD score. The onset of puberty and sexual maturation was delayed but was in accordance with bone age. After transplantation 59% of prepubertal children had a normal height increment. Onset of puberty was recorded at a chronological age of 14.6 ± 1.9 years in boys and 13.3 ± 1.9 years in girls. The peak of the pubertal growth spurt was 6.6 ± 1.6 cm/year in boys and 6.5 ± 2.9 cm/year in girls. The duration of pubertal development in transplanted children was within normal limits. In transplanted girls menarche was achieved at a mean chronological age of 15.9 years and bone age of 12.9 years. Adult height was achieved at a mean age of 20.3 years in men and 18.7 years in women. Overall, one third of the children attained an adult height more than 2 SD below the mean. These data indicate that poor growth is achieved in most children on dialysis treatment; following transplantation normal growth may be restored. However, poor growth before kidney transplantation and the loss of growth potential during pubertal development have a great influence on adult height.  相似文献   

19.
Liang Y  Wei H  Zhang JL  Hou L  Luo XP 《中华儿科杂志》2004,42(11):845-849
目的 观察延长促性腺激素释放激素类似物 (GnRHa)缓释剂注射间期对特发性中枢性性早熟女孩下丘脑 垂体 性腺轴抑制情况和临床症状改善方面的效果。方法  4 6例特发性中枢性性早熟女孩随机分为两组 ,A组 :2 6例 ,年龄 (8 3± 1 4 )岁 (6 1~ 11 2 )岁 ,乳房开始发育年龄 (6 6±1 4 )岁 (2~ 8)岁 ,骨龄 (8 9± 1 5 )岁 (6 8~ 11 5 )岁 ;每 6周腹部皮下注射曲普瑞林 3 75mg ,平均剂量为 2 8± 0 6 (1 8~ 4 1) μg/ (kg·d) ;B组 :2 0例 ,年龄 (8 1± 1 3)岁 (5 1~ 10 3)岁 ,乳房开始发育年龄 (7 0± 1 2 )岁 (4~ 8)岁 ,骨龄 (8 9± 1 4 )岁 (6~ 11 5 )岁 ;每 4周肌肉注射曲普瑞林 3 75mg,平均剂量为 4 8± 1 1(3 2~ 7 4 ) μg / (kg·d)。两组均连续用药 1年以上。治疗过程中注意监测以下指标 :性发育情况、身高、体重、生长速率、血性激素的水平、骨龄等。结果 每 6周皮下注射曲普瑞林3 75mg的治疗方案与每 4周肌肉注射普瑞林 3 75mg的治疗方案均能使患儿的第二性征减退、停止发育 ,乳房发育明显受到抑制 ,乳腺回缩或乳腺组织变松软 ;卵巢体积缩小 ,直径大于 0 4cm的卵泡消失 ;促性腺激素分泌减少 ,性激素下降至青春前期水平 ;A、B两组的年生长速率 (cm/年 )分别由治疗前的 6 3±  相似文献   

20.
Girls adopted from developing countries often have early or precocious puberty, requiring treatment with gonadotropin-releasing hormone (GnRH) analogues. During such treatment decreased growth velocity is frequent. The aim of this investigation was to study whether the addition of growth hormone (GH) to GnRH analogue treatment improves height velocity and final height in girls with early or precocious puberty. Forty-six girls with early or precocious puberty adopted from developing countries were randomized for treatment with GnRH analogue or a combination of GH and GnRH analogue. After 2 y of treatment the mean growth in the GH/GnRH analogue group was significantly higher, 14.6 cm, compared to 10.9 cm in the control group. The increase in bone age did not differ, while the difference in predicted adult height increased by 2.7 cm in favour of the combination group. Although data on final height are not yet available, combined GH/GnRH analogue treatment for 2 y resulted in a higher growth velocity and predicted final height compared to GnRH analogue treatment alone.  相似文献   

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