Cortisol response to adrenocorticotropin testing in non-classical congenital adrenal hyperplasia (NCCAH)
-
Fotini-Heleni Karachaliou
,
Maria Kafetzi
Abstract
Background:
The adequacy of cortisol response in non-classical congenital adrenal hyperplasia (NCCAH) has not been fully elucidated. The aim was to evaluate cortisol response to adrenocorticotropin (ACTH) stimulation test in children and adolescents with NCCAH and heterozygotes for CYP21A2 gene mutations.
Methods:
One hundred and forty-six children and adolescents, mean age 7.9 (0.7–17.5) years with clinical hyperandrogenism, were evaluated retrospectively. Thirty-one subjects had NCCAH, 30 were heterozygotes for CYP21A2 gene mutations, while 85 showed normal response to ACTH test.
Results:
Baseline cortisol levels did not differ among NCCAH, heterozygotes, and normal responders: 15.75 (5.83–59.6) μg/dL vs. 14.67 (5.43–40.89) μg/dL vs. 14.04 (2.97–34.8) μg/dL, p=0.721. However, NCCAH patients had lower peak cortisol compared to heterozygotes and control group: 28.34 (12.25–84.40) vs. 35.22 (17.47–52.37) μg/dL vs. 34.92 (19.91–46.68) μg/dL, respectively, p=0.000. Peak cortisol was <18 μg/dL in 7/31 NCCAH patients and in one heterozygote.
Conclusions:
A percentage of 21.2% NCCAH patients showed inadequate cortisol response to ACTH stimulation. In these subjects, the discontinuation of treatment on completion of growth deserves consideration.
Acknowledgments
We thank the nursing team of the Endocrinology Department for performing ACTH stimulation tests in our patients and the technicians of Biochemistry-Endocrinology Laboratory for performing the 17-OHP and cortisol measurements.
Author contributions: All the authors have accepted responsibility for the entire content of this submitted article and approved submission.
Research funding: None declared.
Employment or leadership: None declared.
Honorarium: None declared.
Competing interests: The funding organization(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.
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©2016 Walter de Gruyter GmbH, Berlin/Boston
Artikel in diesem Heft
- Frontmatter
- Editorial
- Non-alcoholic fatty liver disease in children and adolescents
- Original Articles
- Serum vascular endothelial cadherin and thrombomodulin are markers of non-alcoholic fatty liver disease in children
- Ferritin level is associated with metabolic syndrome and elevated alanine aminotransferase in children and adolescents
- Dietary fructose intake in obese children and adolescents: relation to procollagen type III N-terminal peptide (P3NP) and non-alcoholic fatty liver disease
- Central diabetes insipidus: clinical profile that suggests organicity in Peruvian children: Lima – Peru 2001–2013
- Salivary flow rate, buffer capacity, and urea concentration in adolescents with type 1 diabetes mellitus
- Cortisol response to adrenocorticotropin testing in non-classical congenital adrenal hyperplasia (NCCAH)
- Efficacy of micellized vs. fat-soluble vitamin D3 supplementation in healthy school children from Northern India
- Growth curves for congenital adrenal hyperplasia from a national retrospective cohort
- The effects of type 1 diabetes mellitus on cardiac functions in children: evaluation by conventional and tissue Doppler echocardiography
- The association between single nucleotide polymorphisms of the Apelin gene and diabetes mellitus in a Chinese population
- Case Reports
- Successful transition to sulfonylurea therapy in two Iraqi siblings with neonatal diabetes mellitus and iDEND syndrome due to ABCC8 mutation
- A case of 46,XX dysgenesis and marked tall stature; the need for caution in interpreting array comparative genomic hybridization (CGH)
- Successful treatment of a child with a prolactin secreting macroadenoma with temozolomide
- Acknowledgment
- Acknowledgment
Artikel in diesem Heft
- Frontmatter
- Editorial
- Non-alcoholic fatty liver disease in children and adolescents
- Original Articles
- Serum vascular endothelial cadherin and thrombomodulin are markers of non-alcoholic fatty liver disease in children
- Ferritin level is associated with metabolic syndrome and elevated alanine aminotransferase in children and adolescents
- Dietary fructose intake in obese children and adolescents: relation to procollagen type III N-terminal peptide (P3NP) and non-alcoholic fatty liver disease
- Central diabetes insipidus: clinical profile that suggests organicity in Peruvian children: Lima – Peru 2001–2013
- Salivary flow rate, buffer capacity, and urea concentration in adolescents with type 1 diabetes mellitus
- Cortisol response to adrenocorticotropin testing in non-classical congenital adrenal hyperplasia (NCCAH)
- Efficacy of micellized vs. fat-soluble vitamin D3 supplementation in healthy school children from Northern India
- Growth curves for congenital adrenal hyperplasia from a national retrospective cohort
- The effects of type 1 diabetes mellitus on cardiac functions in children: evaluation by conventional and tissue Doppler echocardiography
- The association between single nucleotide polymorphisms of the Apelin gene and diabetes mellitus in a Chinese population
- Case Reports
- Successful transition to sulfonylurea therapy in two Iraqi siblings with neonatal diabetes mellitus and iDEND syndrome due to ABCC8 mutation
- A case of 46,XX dysgenesis and marked tall stature; the need for caution in interpreting array comparative genomic hybridization (CGH)
- Successful treatment of a child with a prolactin secreting macroadenoma with temozolomide
- Acknowledgment
- Acknowledgment
