Growth-promoting agents for nongrowth hormone-deficient short children

2011 ◽  
pp. 1083-1095
Author(s):  
Pierre Chatelain
Keyword(s):  
Growth Plate ◽  
Growth Phase ◽  
Growth Velocity ◽  
Adult Height ◽  
Final Height ◽  
Normal Range ◽  
Growth Plate Chondrocytes ◽  
Accelerate Growth ◽  
Short Children ◽  
Growth Promoting

Growth-promoting agents refer to compounds, existing naturally or otherwise, which, when given to a short child as a medication, will accelerate growth velocity (GV), bringing the child’s height closer to or within normal range. The first objective, normalizing height during the growth phase, may not normalize adult height, which is the second objective. The ideal growth-promoting agent should normalize both. The mechanism behind these two objectives is not fully understood. It seems that when the growth plate cartilage chondrocytes multiply, they also differentiate then stop multiplying. The balance between multiplication and differentiation of growth plate chondrocytes is key to normalizing final height.

Aromatase excess syndrome in a Chinese boy due to a novel duplication at 15q21.2

2019 ◽  
Vol 32 (1) ◽  
pp. 85-88 ◽  
Author(s):  
Xinrui Tan ◽  
Xiaochuan Wu ◽  
Jie Chen ◽  
Yan Wu ◽  
Shijun Li ◽  
...  
Keyword(s):  
Adult Height ◽  
Linear Growth ◽  
De Novo ◽  
Final Height ◽  
Clinical Manifestations ◽  
Bone Age ◽  
Autosomal Dominant Disorder ◽  
Case Presentation ◽  
Growth Promoting ◽  
Pubertal Gynecomastia

Abstract Background Aromatase excess syndrome (AEXS) is a rare autosomal dominant disorder caused by CYP19A1 overexpression. Clinical manifestations of AEXS include pre- or peri-pubertal gynecomastia, advanced bone age and compromised adult height. Case presentation Here we report an 8-year-old boy diagnosed with AEXS by chromosomal array that revealed a 1.1 Mb novel de novo duplication at 15q21.2, with a predicted final height of 157.4 cm. We prescribed letrozole and growth hormone (GH) to maximize his linear growth. Without further bone age advancement, his height increased from 137.7 cm to 144 cm after an 8-month treatment period. Conclusions We identified a novel duplication at 15q21.2 in AEXS, and found that aromatase inhibitor (AI) plus GH might provide a better growth-promoting approach for AEXS patients.

Growth velocity and biological variables during puberty in achondroplasia

2018 ◽  
Vol 31 (4) ◽  
pp. 421-428 ◽  
Author(s):  
Mariana del Pino ◽  
Virginia Fano ◽  
Paula Adamo
Keyword(s):  
Growth Velocity ◽  
Adult Height ◽  
Peak Velocity ◽  
Final Height ◽  
Pubertal Development ◽  
Individual Growth ◽  
Growth Spurt ◽  
Adult Size ◽  
Biological Variables ◽  
The Mean

AbstractBackground:Achondroplasia is the most common form of inherited disproportionate short stature. Cross-sectional design studies of height show that, during childhood, height standard deviation scores (SDS) declines steadily and reaches a mean adult height at −6.42 and −6.72 SDS. However, there is a lack of knowledge about longitudinal growth and biological variables during puberty for children with achondroplasia. Here we report the growth velocity and biological parameters during puberty in children with achondroplasia.Methods:The study was an observational, cohort study. A total of 23 patients, 15 girls and eight boys with achondroplasia, who reached adult size were included. Growth data was collected from mid-childhood until final height by the same trained observer. Individual growth curves were estimated by fitting the Preece-Baines model 1 (PB1) to each individual’s height for age data. Pubertal development was scored on Tanner scale on each visit.Results:In boys with achondroplasia the mean adult height was 129.18 cm. Age and velocity at peak velocity in puberty were 13.89 years and 4.86 cm/year, respectively. The adolescent gain was 20.40 cm. Mean age at genital development 2 and 5 were 12.16 (0.60) and 14.97 (0.88), respectively. In girls the mean adult height was 118.67 cm. Age and velocity at peak velocity in puberty were 11.45 years and 4.40 cm/year, respectively. The adolescent gain was 19.35 cm. Mean age at breast 2 and 4 were 10.20 (1.24) and 12.49 (1.07), respectively.Conclusions:Children with achondroplasia experienced an adolescent growth spurt, which was similar in shape and half the magnitude of the non-achondroplasia population.

scholarly journals Adult height of prepubertal short children born small for gestational age treated with GH

2005 ◽  
Vol 152 (6) ◽  
pp. 835-843 ◽  
Author(s):  
Myriam Rosilio ◽  
Jean-Claude Carel ◽  
Emmanuel Ecosse ◽  
Jean-Louis Chaussainon ◽  
_ _
Keyword(s):  
Treatment Period ◽  
Gestational Age ◽  
Small For Gestational Age ◽  
Adult Height ◽  
Growth Potential ◽  
Phase Iii ◽  
Efficacy And Safety ◽  
Normal Range ◽  
Short Children ◽  
Height Gain

Objective: Human GH (hGH) treatment leads to catch-up growth in children with short stature born small for gestational age (SGA). However, long-term efficacy and safety results in this patient group remain scarce. The present study assessed the efficacy and safety of late childhood treatment with biosynthetic hGH (Humatrope) in a group of short children born SGA (height <−2 standard deviation scores (SDS)). Design: Patients in this open-label, Phase III, multicenter study received a daily hGH dose of 0.067 mg/kg for 2 years, and then received no treatment for the following 2 years. After the fourth year on study, patients whose height had decreased more than 0.5 SDS but who still showed growth potential based on bone age were allowed to resume treatment until they reached adult height. Methods: Height gain SDS was assessed for 11 girls and 24 boys (mean age±s.d. 9.6±0.9 years) at the end of the 2 years of hGH treatment, during the subsequent 2-year off-treatment period, and upon reaching adult height. Results: At the end of the initial 2-year treatment period, 83% of patients had reached a height within the normal range, with a mean increase in height SDS vs baseline of 1.3±0.3 (P <0.001). Adult heights (n = 20) were within the normal range for 50% of patients, and mean height gain from baseline was statistically significant (0.7±0.8 SDS, P <0.001). Fasting glucose and glycosylated hemoglobin levels were not significantly modified during treatment. Conclusions: High-dose hGH treatment for a minimum of 2 years in short children born SGA was well tolerated and resulted in a significant increase in adolescent and adult height.

scholarly journals Systemic and Local Regulation of the Growth Plate

2003 ◽  
Vol 24 (6) ◽  
pp. 782-801 ◽  
Author(s):  
B. C. J. van der Eerden ◽  
M. Karperien ◽  
J. M. Wit
Keyword(s):  
Growth Factors ◽  
Growth Plate ◽  
Final Height ◽  
Age At Onset ◽  
Longitudinal Growth ◽  
Growth Spurt ◽  
Growth Disorders ◽  
Growth Plate Chondrocytes ◽  
Chondrocyte Proliferation ◽  
Proliferation And Differentiation

Abstract The growth plate is the final target organ for longitudinal growth and results from chondrocyte proliferation and differentiation. During the first year of life, longitudinal growth rates are high, followed by a decade of modest longitudinal growth. The age at onset of puberty and the growth rate during the pubertal growth spurt (which occurs under the influence of estrogens and GH) contribute to sex difference in final height between boys and girls. At the end of puberty, growth plates fuse, thereby ceasing longitudinal growth. It has been recognized that receptors for many hormones such as estrogen, GH, and glucocorticoids are present in or on growth plate chondrocytes, suggesting that these hormones may influence processes in the growth plate directly. Moreover, many growth factors, i.e., IGF-I, Indian hedgehog, PTHrP, fibroblast growth factors, bone morphogenetic proteins, and vascular endothelial growth factor, are now considered as crucial regulators of chondrocyte proliferation and differentiation. In this review, we present an update on the present perception of growth plate function and the regulation of chondrocyte proliferation and differentiation by systemic and local regulators of which most are now related to human growth disorders.

Growth and development in girls with Turner's syndrome during early therapy with low doses of estradiol

1986 ◽  
Vol 113 (4_Suppl) ◽  
pp. S157-S163 ◽  
Author(s):  
K.W. KASTRUP ◽  
_ _
Keyword(s):  
Growth Velocity ◽  
Final Height ◽  
Bone Age ◽  
Growth Spurt ◽  
First Year ◽  
Turner's Syndrome ◽  
Turner’S Syndrome ◽  
Early Therapy ◽  
Group I ◽  
Group Ii

Abstract Early therapy with a low dose of estrogen (estradiol-17β) was given to 33 girls with Turner's syndrome (T.s.) for a period of 4 years. The dose (0.25-2 mg/day) was adjusted every 3 months to maintain plasma estradiol in the normal concentration range for bone age. Growth velocity was compared with that of untreated girls with T.s. All girls were above age 10 years. Bone age was below 10 years in 11 girls (group I) and above 10 years in 22 girls (group II). Growth velocity in the first year of treatment in group I 7.5 ± 1.3 cm (SD) with mean SD score (SDS) of +4.3 and in group II 4.9 ± 1.3 with mean SDS of +3.5. Growth velocity decreased in the following years to 1.6 ± 1.0 cm, SDS -1.44 in group I and 0.9 ± 0.6cm, SDS -2.34 in group II during the fourth year. Withdrawal bleeding occurred in 16 girls of group II after the mean of 23 (range 15-33) months and in 3 girls of group I after 15 to 51 months of treatment. The treatment did not cause an inappropriate acceleration of pubertal development. Breast development appeared in most girls by 3 months of treatment. Pubic hair appeared by 12 months of treatment in group I; it was present in most girls in group II at start of treatment. Final height is known for 12 girls of group II; it was 144.2 ± 4.5 cm. The final height as predicted at the start of therapy was 142.2 ± 5.3 cm. Bone age advanced in the first year of treatment by 2 years. Early treatment with small doses of estrogens induces a growth spurt and normalizes the events of puberty. This will presumably decrease the psychological risks associated with abnormally delayed development.

Pubertal development and adult height in patients with congenital hypothyroidism detected by neonatal screening in southern Brazil

2020 ◽  
Vol 33 (11) ◽  
pp. 1449-1455
Author(s):  
Suzana Nesi-França ◽  
Rodrigo B. Silveira ◽  
Juliana Cristina R. Rojas Ramos ◽  
Adriane A. Cardoso-Demartini ◽  
Monica N. Lima Cat ◽  
...  
Keyword(s):  
Congenital Hypothyroidism ◽  
Neonatal Screening ◽  
Adult Height ◽  
Screening Program ◽  
Pubertal Development ◽  
Z Score ◽  
Normal Range ◽  
Adequate Treatment ◽  
Increased Risk ◽  
Puberty Onset

AbstractObjectivesAdequate treatment of congenital hypothyroidism (CH) is required for normal growth and sexual development. To evaluate pubertal development in patients with permanent CH detected by a statewide Neonatal Screening Program of Paraná and, secondly, to evaluate adult height (AH) in a subgroup of patients.MethodsClinical, laboratory, and auxological data obtained from medical records of 174 patients (123 girls).ResultsMedian chronological age (CA) at treatment initiation was 24 days, and mean initial levothyroxine dose was 11.7 ± 1.9 μg/kg/day; mean CA at puberty onset was 11.5 ± 1.3 years (boys) and 9.7 ± 1.2 years (girls); mean CA in girls who underwent menarche (n=81) was 12.1 ± 1.1 years. Thyroid-stimulating hormone (TSH) values above the normal range were observed in 36.4% of the boys and 32.7% of the girls on puberty onset, and in 44.6% around menarche. Among 15 boys and 66 girls who had reached the AH, the median height z-score value was significantly greater than the target height (TH) z-score value in boys (p=0.01) and in girls (p<0.001). Boys with normal TSH values at puberty onset had greater mean AH z-score compared with boys with TSH values above the normal range (p=0.04).ConclusionsIn this group, pubertal development in girls with CH was not different from that reported in healthy girls in the general Brazilian population. Boys with higher TSH at puberty onset may have an increased risk of not reaching their potential height compared with those with normal TSH during this period. In a subgroup who attained AH, the median AH z-score was greater than the median TH z-score.

scholarly journals Safe and persistent growth-promoting effects of vosoritide in children with achondroplasia: 2-year results from an open-label, phase 3 extension study

2021 ◽  
Author(s):  
Ravi Savarirayan ◽  
Louise Tofts ◽  
Melita Irving ◽  
William R. Wilcox ◽  
Carlos A. Bacino ◽  
...  
Keyword(s):  
Growth Velocity ◽  
Bone Growth ◽  
Growth Factor Receptor ◽  
Extension Study ◽  
Controlled Study ◽  
Open Label ◽  
Phase 3 ◽  
Double Blind ◽  
Pathogenic Variants ◽  
Growth Promoting

Abstract Purpose Achondroplasia is caused by pathogenic variants in the fibroblast growth factor receptor 3 gene that lead to impaired endochondral ossification. Vosoritide, an analog of C-type natriuretic peptide, stimulates endochondral bone growth and is in development for the treatment of achondroplasia. This phase 3 extension study was conducted to document the efficacy and safety of continuous, daily vosoritide treatment in children with achondroplasia, and the two-year results are reported. Methods After completing at least six months of a baseline observational growth study, and 52 weeks in a double-blind, placebo-controlled study, participants were eligible to continue treatment in an open-label extension study, where all participants received vosoritide at a dose of 15.0 μg/kg/day. Results In children randomized to vosoritide, annualized growth velocity increased from 4.26 cm/year at baseline to 5.39 cm/year at 52 weeks and 5.52 cm/year at week 104. In children who crossed over from placebo to vosoritide in the extension study, annualized growth velocity increased from 3.81 cm/year at week 52 to 5.43 cm/year at week 104. No new adverse effects of vosoritide were detected. Conclusion Vosoritide treatment has safe and persistent growth-promoting effects in children with achondroplasia treated daily for two years.

scholarly journals Growth in achondroplasia, from birth to adulthood, analysed by the JPA-2 model

2020 ◽  
Vol 33 (12) ◽  
pp. 1589-1595
Author(s):  
Mariana del Pino ◽  
Virginia Fano ◽  
Paula Adamo
Keyword(s):  
General Population ◽  
Growth Curve ◽  
Growth Velocity ◽  
Adult Height ◽  
Growth Curves ◽  
Peak Height ◽  
Height Growth ◽  
Individual Growth ◽  
Height Velocity ◽  
Peak Height Velocity

AbstractObjectivesIn general population, there are three phases in the human growth curve: infancy, childhood and puberty, with different main factors involved in their regulation and mathematical models to fit them. Achondroplasia children experience a fast decreasing growth during infancy and an “adolescent growth spurt”; however, there are no longitudinal studies that cover the analysis of the whole post-natal growth. Here we analyse the whole growth curve from infancy to adulthood applying the JPA-2 mathematical model.MethodsTwenty-seven patients, 17 girls and 10 boys with achondroplasia, who reached adult size, were included. Height growth data was collected from birth until adulthood. Individual growth curves were estimated by fitting the JPA-2 model to each individual’s height for age data.ResultsHeight growth velocity curves show that after a period of fast decreasing growth velocity since birth, with a mean of 9.7 cm/year at 1 year old, the growth velocity is stable in late preschool years, with a mean of 4.2 cm/year. In boys, age and peak height velocity in puberty were 13.75 years and 5.08 cm/year and reach a mean adult height of 130.52 cm. In girls, the age and peak height velocity in puberty were 11.1 years and 4.32 cm/year and reach a mean adult height of 119.2 cm.ConclusionsThe study of individual growth curves in achondroplasia children by the JPA-2 model shows the three periods, infancy, childhood and puberty, with a similar shape but lesser in magnitude than general population.

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