scholarly journals Aromatase Inhibitor as Treatment for Severely Advanced Bone Age in Congenital Adrenal Hyperplasia: A Case Report

2019 ◽  
Vol 92 (3) ◽  
pp. 209-213
Author(s):  
Wesley J. Goedegebuure ◽  
Anita C.S. Hokken-Koelega
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Adult Height ◽  
Corticosteroid Treatment ◽  
Bone Age ◽  
Adrenal Hyperplasia ◽  
Bone Maturation ◽  
Standard Dosage ◽  
Novel Treatment ◽  
Accelerated Bone Maturation ◽  
Androgen Levels

Introduction:Treatment with aromatase inhibitors (AI) is a potential novel treatment in patients with congenital adrenal hyperplasia (CAH) and advanced bone age (BA), to increase near adult height (NAH). Not much is known about the efficacy of AI treatment in CAH and how AI treatment will influence the management of corticosteroid treatment. Case Presentation:At the age of 6 years and 3 months, a boy with salt-losing CAH presented with a BA 7 years in advance. Treatment with an AI (exemestane) was initiated to decelerate bone maturation. We continued the standard dosage of corticosteroid treatment. Precocious puberty was treated with 4 years of gonadotropin-releasing hormone agonist, while AI treatment was continued until attainment of NAH. His NAH 177.7 cm (–0.8 SDS) was considerably higher than his predicted adult height of 151.3 cm (–4.6 SDS) at the start of AI treatment. The higher serum androgen levels during AI treatment did not result in short adult stature. Discussion/Conclusion:This report shows that AI treatment can adequately decelerate bone maturation, causing predicted adult height to increase significantly in patients of CAH with accelerated bone maturation. We suggest continuing the same corticosteroid dosage during AI treatment and accepting higher serum androgen levels.

Growth curves for congenital adrenal hyperplasia from a national retrospective cohort

2016 ◽  
Vol 29 (12) ◽  
Author(s):  
Patricia Bretones ◽  
Benjamin Riche ◽  
Emmanuel Pichot ◽  
Michel David ◽  
Pascal Roy ◽  
...  
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Adult Height ◽  
Bone Age ◽  
Strong Impact ◽  
Adrenal Hyperplasia ◽  
Hydroxylase Deficiency ◽  
Bone Maturation ◽  
The Mean ◽  
Puberty Onset ◽  
21 Hydroxylase Deficiency

Abstract Background: In congenital adrenal hyperplasia (CAH), adjusting hydrocortisone dose during childhood avoids reduced adult height. However, there are currently no CAH-specific charts to monitor growth during treatment. Our objective was to elaborate growth reference charts and bone maturation data for CAH patients. Methods: We conducted a retrospective observational cohort study, in 34 French CAH centers. Patients were 496 children born 1970–1991 with genetically proven 21-hydroxylase deficiency. Their growth and bone maturation data were collected until age 18 together with adult height, puberty onset, parental height, and treatment. The mean (SD) heights were modeled from birth to adulthood. The median±1 SD and ±2 SDs model-generated curves were compared with the French references. A linear model for bone maturation and a logistic regression model for the probability of short adult height were built. Results: Growth charts were built by sex for salt wasting (SW) and simple virilizing (SV) children treated before 1 year of age. In girls and boys, growth was close to that of the general French population up to puberty onset. There was almost no pubertal spurt and the mean adult height was shorter than that of the general population in girls (−1.2 SD, 156.7 cm) and boys (−1.0 SD, 168.8 cm). Advanced bone age at 8 years had a strong impact on the risk of short adult height (OR: 4.5 per year advance). Conclusions: The 8-year bone age is a strong predictor of adult height. It will help monitoring the growth of CAH-affected children.

scholarly journals Histrelin Implantation and Growth Outcomes in Children With Congenital Adrenal Hyperplasia: An Institutional Experience

2019 ◽  
Vol 4 (2) ◽  
Author(s):  
Robert A Swendiman ◽  
Barbara E Coons ◽  
Craig A Alter ◽  
Vaneeta Bamba ◽  
Michael L Nance ◽  
...  
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Precocious Puberty ◽  
Retrospective Cohort ◽  
Adult Height ◽  
Cohort Analysis ◽  
Bone Age ◽  
Adrenal Hyperplasia ◽  
Growth Data ◽  
Hydroxylase Deficiency ◽  
21 Hydroxylase Deficiency

Abstract Background Children with congenital adrenal hyperplasia (CAH) because of 21 hydroxylase deficiency (21OHD) are at risk for early or precocious puberty and a short adult height compared to population means and midparental height. The effect of histrelin in suppressing puberty and improving growth in these children has not been reported. Methods Retrospective cohort analysis of all patients (age ≤ 20) at our institution who underwent histrelin implantation between 2008 and 2017. Treated patients with CAH (classic and nonclassic forms of 21OHD) were identified and their growth data analyzed. Results Fifteen children with CAH were treated with histrelin for a median of 3 years (range 2–5; age at first implantation 7.7 ± 1.5 years). Bone age (BA) to chronologic age (CA) decreased from 1.57 ± 0.4 to 1.25 ± 0.25 (P < .01), while predicted adult height (PAH) increased by 7.1 ± 6.6 cm (P < .01). A subgroup of 10 children reached adult height. Similar changes in BA/CA and PAH were observed with therapy (P = .02). Adult height z improved compared to pretreatment PAH z (–1.42 ± 0.9 vs. –1.96 ± 1.1 respectively, P < .01), but remained lower than midparental height z (P = .01). Conclusion In this retrospective cohort study of children with CAH due to 21OHD and early or precocious puberty, histrelin implantation resulted in a decrease in BA progression compared to CA and an improvement in PAH. In the subgroup who completed growth, adult height remained significantly lower than midparental. These results need to be confirmed with prospective controlled studies.

scholarly journals Characteristics of growth in children with classic congenital adrenal hyperplasia due to 21-hydroxylase deficiency during adrenarche and beyond

2021 ◽  
Author(s):  
Tobias Troger ◽  
Grit Sommer ◽  
Mariarosaria Lang-Muritano ◽  
Daniel Konrad ◽  
Beatrice Kuhlmann ◽  
...  
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Adult Height ◽  
Final Height ◽  
Bone Age ◽  
Growth Patterns ◽  
Adrenal Hyperplasia ◽  
Classic Congenital Adrenal Hyperplasia ◽  
Classic Cah ◽  
21 Hydroxylase Deficiency ◽  
The Impact

Abstract Context Patients with classic congenital adrenal hyperplasia (CAH) often fail to achieve their full growth potential. Adrenarche may accelerate bone maturation and thereby result in decreased growth in CAH. Objective To analyze the impact of growth during adrenarche on final height of adequately treated classic CAH patients. Design Retrospective, multi-center study. Setting Four academic pediatric endocrinology centers. Participants Fourty-one patients with classical CAH, born between 1990 and 2012. Main outcome measures We assessed skeletal maturation (bone age), growth velocity and (projected) adult height outcomes, and analyzed potential influencing factors, such as sex, genotype, and glucocorticoid therapy. Results Patients with classic CAH were shorter than peers (-0.4SDS±0.8SD) and their parents (corrected final height -0.6SDS±1.0SD). Analysis of growth during adrenarche revealed two different growth patterns: patients with accelerating bone age (49%), and patients with non-accelerating bone age compared to chronological age (BA-CA). Patients with accelerating BA-CA were taller than the normal population during adrenarche years (p=0.001) and were predicted to achieve a lower adult height SDS (-0.9SDS, 95%CI -1.3;-0.5) than non-accelerating patients when assessed during adrenarche (0.2SDS, 95%CI -0.3;0.8). Final adult height was similarly reduced in both accelerating and non-accelerating BA-CA groups (-0.4SDS, 95%CI -0.9;0.1 vs -0.3SDS, 95%CI -0.8;0.1). Conclusions Patients with and without significant bone age advancement, and thus differing height prediction during adrenarche, showed similar (predicted) final height when reassessed during pubertal years. Bone age alone should not be used during adrenarche as clinical marker for metabolic control in CAH treatment.

scholarly journals Final Adult Height in Children with Congenital Adrenal Hyperplasia Treated with Growth Hormone

2011 ◽  
Vol 96 (6) ◽  
pp. 1710-1717 ◽  
Author(s):  
Karen Lin-Su ◽  
Madeleine D. Harbison ◽  
Oksana Lekarev ◽  
Maria G. Vogiatzi ◽  
Maria I. New
Keyword(s):  
Growth Hormone ◽  
Congenital Adrenal Hyperplasia ◽  
Adult Height ◽  
Adrenal Hyperplasia ◽  
Final Adult Height

Final Adult Height in Children with Congenital Adrenal Hyperplasia Treated with Growth Hormone

2011 ◽  
pp. P1-756-P1-756
Author(s):  
Karen Lin-Su ◽  
Madeleine D Harbison ◽  
Oksana Lekarev ◽  
Maria G Vogiatzi ◽  
Maria I New
Keyword(s):  
Growth Hormone ◽  
Congenital Adrenal Hyperplasia ◽  
Adult Height ◽  
Adrenal Hyperplasia ◽  
Final Adult Height

LONG-TERM TREATMENT OF CONGENITAL ADRENAL HYPERPLASIA WITH CORTISOL AND PREDNISOLONE ACETATE IN AQUEOUS SUSPENSION

1962 ◽  
Vol 39 (3) ◽  
pp. 323-327 ◽  
Author(s):  
Bernardo Léo Wajchenberg ◽  
J. Schnaider ◽  
R. D. Federico ◽  
A. Gelman ◽  
Virgílio G. Pereira ◽  
...  
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Linear Growth ◽  
Aqueous Suspension ◽  
Term Treatment ◽  
Prednisolone Acetate ◽  
Adrenal Hyperplasia ◽  
Bone Maturation ◽  
Intramuscular Injections ◽  
Long Term Treatment

ABSTRACT Treatment of congenital adrenal hyperplasia with cortisol or prednisolone acetate, by infrequent intramuscular injections, is quite adequate as shown by arrest of precocious bone maturation and excessive linear growth, despite the intermittent adrenal depression as shown by the 17-ketosteroid levels.

Validation of automatic bone age determination in children with congenital adrenal hyperplasia

2013 ◽  
Vol 43 (12) ◽  
pp. 1615-1621 ◽  
Author(s):  
David D. Martin ◽  
Katharina Heil ◽  
Conrad Heckmann ◽  
Angelika Zierl ◽  
Jürgen Schaefer ◽  
...  
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Age Determination ◽  
Bone Age ◽  
Adrenal Hyperplasia
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