Flutamide, Testolactone, and Reduced Hydrocortisone Dose Maintain Normal Growth Velocity and Bone Maturation Despite Elevated Androgen Levels in Children with Congenital Adrenal Hyperplasia

2000 ◽  
Vol 85 (3) ◽  
pp. 1114-1120 ◽  
Author(s):  
Deborah P. Merke ◽  
Margaret F. Keil ◽  
Janet V. Jones ◽  
Jeremy Fields ◽  
Suvimol Hill ◽  
...  
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Growth Velocity ◽  
Normal Growth ◽  
Adrenal Hyperplasia ◽  
Bone Maturation ◽  
Androgen Levels

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.

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.

Congenital adrenal hyperplasia in children – a survey on the current practice in the UK

2015 ◽  
Vol 28 (7-8) ◽  
Author(s):  
Usha Niranjan ◽  
Anuja Natarajan
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Online Survey ◽  
Current Practice ◽  
Normal Growth ◽  
British Society ◽  
Adrenal Crisis ◽  
Optimal Management ◽  
Adrenal Hyperplasia ◽  
Endocrine Society ◽  
The Uk

AbstractCongenital adrenal hyperplasia (CAH) in children varies in presentation and progression with several challenges in optimal management. Effective treatment is to achieve normal growth and development while avoiding adrenal crisis and hyperandrogenisation.Our aim was to ascertain the current practice in the UK on CAH management in children in comparison with the recommendations made by the Endocrine Society.An online survey was emailed to the British Society of Paediatric Endocrinology (BSPED) members requesting a response from each centre regarding CAH management.The survey was completed by 35 out of 92 centres (38% response rate). Tertiary centres constituted 22/35, while 8/35 were district general hospitals providing tertiary services. Treatment varied among centres with 25/35 using 10–15 mg/mOur survey highlights the diversity in managing children with CAH in the UK as compared with the recommendations of the Endocrine Society. It also demonstrates inconsistent involvement of essential specialist services, which are essential for optimal management of this condition.

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.

Patterns of growth from birth to maturity in infants and children with congenital adrenal hyperplasia

1986 ◽  
Vol 113 (4_Suppl) ◽  
pp. S295-S304 ◽  
Author(s):  
George W. Clayton
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Growth Failure ◽  
Normal Growth ◽  
Growth Patterns ◽  
Height Velocity ◽  
Growth Spurt ◽  
First Year ◽  
Adrenal Hyperplasia ◽  
Pubertal Growth Spurt ◽  
First Year Of Life

Abstract Patterns of growth of 30 children with Congenital Adrenal Hyperplasia of the 21-hydroxylase type were studied from infancy to maturity. These children were compliant as to therapy. Intramuscular (I.M.) Cortisone (40mg/M2) given every three days resulted in growth failure after the first year of life. A marked increase in height velocity occurred when oral Cortisone (20-25mg/M2) was given at approximate] 3 years. Growth was relatively normal during childhood in both boys and girls but there was a marked increase in mean weight in both sexes. Pubertal growth spurt occurred normally in males but was delayed in females. Weight decreased in both sexes at puberty but not to normal. Better methods of monitoring this condition should result in therapeutic regimes which allow normal growth patterns as well as normal mature height.

Growth of patients with congenital adrenal hyperplasia due to 21-hydroxylase in infancy, glucocorticoid requirement and the role of mineralocorticoid therapy

2018 ◽  
Vol 31 (9) ◽  
pp. 1019-1022
Author(s):  
Jack Sellick ◽  
Sarah Aldridge ◽  
Matthew Thomas ◽  
Tim Cheetham
Keyword(s):  
Standard Deviation ◽  
Congenital Adrenal Hyperplasia ◽  
Standard Deviation Score ◽  
Normal Growth ◽  
Height Velocity ◽  
Adrenal Hyperplasia ◽  
Growth Data ◽  
Hydroxylase Deficiency ◽  
21 Hydroxylase Deficiency

Abstract Background The dose of hydrocortisone therapy required to maintain normal growth in infants with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency is lower than in later childhood. This reflects the presence of excess non-aromatisable rather than aromatisable androgen but there has been relatively little focus on the role of mineralocorticoid therapy. Methods Growth data of infants with CAH due to 21-hydroxylase deficiency (2008–2016) were reviewed and information regarding hydrocortisone and fludrocortisone regimen was collected. Change in height standard deviation (SD) and height velocity standard deviation score (SDS) were analysed during the first year of life. Results Growth data from 13 children (8 M) were analysed. Height (length) declined from a median of −0.69 SD at 3 months to −1.23 SD at 12 months with a reduction in height velocity SDS from 0.02 between 3 and 6 months to −2.22 between 9 and 12 months (p=0.017) despite a hydrocortisone dose at the lower end of the range as recommended in consensus guidelines. The glucocorticoid activity of hydrocortisone and fludrocortisone was negatively associated with growth velocity (r=−0.55; p=0.049) although renin activity was not suppressed. Conclusions Infants with 21-hydroxylase deficiency can be managed with replacement hydrocortisone. The reasons for this paradigm are now understood although our data confirm that the glucocorticoid activity of fludrocortisone needs to be taken into consideration as well.

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