Growth of patients with congenital adrenal hyperplasia due to 21-hydroxylase in infancy, glucocorticoid requirement and the role of mineralocorticoid therapy
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Jack Sellick
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.
Author contributions: Tim Cheetham had the original idea for the manuscript and took the lead in writing, data analysis and its interpretation. Matthew Thomas was involved in data analysis. Jack Sellick was involved in data collection, analysis and writing. Sarah Aldridge was involved in data collection, analysis and manuscript preparation.
Research funding: None declared.
Employment or leadership: None declared.
Honorarium: None declared.
Competing interests: The funding organisation(s) played no role in the study design; in the collection, analysis, and interpretation of data; in the writing of the report; or in the decision to submit the report for publication.
References
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©2018 Walter de Gruyter GmbH, Berlin/Boston
Artikel in diesem Heft
- Frontmatter
- Original Articles
- Intracranial pathologies associated with central diabetes insipidus in infants
- Omentin-1 and NAMPT serum concentrations are higher and CK-18 levels are lower in children and adolescents with type 1 diabetes when compared to healthy age, sex and BMI matched controls
- Evaluation of anthropometric parameters of central obesity in Pakistani children aged 5–12 years, using receiver operating characteristic (ROC) analysis
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- Untreated congenital hypothyroidism due to loss to follow-up: developing preventive strategies through quality improvement
- The effect of 17 years of increased salt iodization on the prevalence and nature of goiter in Croatian schoolchildren
- Is there an association between thyrotropin levels within the normal range and birth growth parameters in full-term newborns?
- Etiology of short stature in Indian children and an assessment of the growth hormone-insulin-like growth factor axis in children with idiopathic short stature
- Growth of patients with congenital adrenal hyperplasia due to 21-hydroxylase in infancy, glucocorticoid requirement and the role of mineralocorticoid therapy
- A personal series of 100 children operated for Cushing’s disease (CD): optimizing minimally invasive diagnosis and transnasal surgery to achieve nearly 100% remission including reoperations
- 12-Week aerobic exercise and nutritional program minimized the presence of the 64Arg allele on insulin resistance
- Letter to the Editor
- Successful treatment of life-threatening severe metabolic acidosis by continuous veno-venous hemodialysis in a child with diabetic ketoacidosis
- Case Reports
- Two siblings with metachromatic leukodystrophy caused by a novel identified homozygous mutation in the ARSA gene
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- Van Wyk-Grumbach syndrome with hemangioma in an infant
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Artikel in diesem Heft
- Frontmatter
- Original Articles
- Intracranial pathologies associated with central diabetes insipidus in infants
- Omentin-1 and NAMPT serum concentrations are higher and CK-18 levels are lower in children and adolescents with type 1 diabetes when compared to healthy age, sex and BMI matched controls
- Evaluation of anthropometric parameters of central obesity in Pakistani children aged 5–12 years, using receiver operating characteristic (ROC) analysis
- A lower energetic, protein and uncooked cornstarch intake is associated with a more severe outcome in glycogen storage disease type III: an observational study of 50 patients
- Untreated congenital hypothyroidism due to loss to follow-up: developing preventive strategies through quality improvement
- The effect of 17 years of increased salt iodization on the prevalence and nature of goiter in Croatian schoolchildren
- Is there an association between thyrotropin levels within the normal range and birth growth parameters in full-term newborns?
- Etiology of short stature in Indian children and an assessment of the growth hormone-insulin-like growth factor axis in children with idiopathic short stature
- Growth of patients with congenital adrenal hyperplasia due to 21-hydroxylase in infancy, glucocorticoid requirement and the role of mineralocorticoid therapy
- A personal series of 100 children operated for Cushing’s disease (CD): optimizing minimally invasive diagnosis and transnasal surgery to achieve nearly 100% remission including reoperations
- 12-Week aerobic exercise and nutritional program minimized the presence of the 64Arg allele on insulin resistance
- Letter to the Editor
- Successful treatment of life-threatening severe metabolic acidosis by continuous veno-venous hemodialysis in a child with diabetic ketoacidosis
- Case Reports
- Two siblings with metachromatic leukodystrophy caused by a novel identified homozygous mutation in the ARSA gene
- To diet or not to diet in neonatal diabetes responding to sulfonylurea treatment
- Van Wyk-Grumbach syndrome with hemangioma in an infant
- Maternal iodine excess: an uncommon cause of acquired neonatal hypothyroidism
