Effect of complete suppression of androstenedione on auxological development in prepubertal patients with classical congenital adrenal hyperplasia
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Belana Debor
,
Susanne Bechtold-Dalla Pozza
Abstract
Objectives
Children with classical congenital adrenal hyperplasia (CAH) require glucocorticoid (GC) substitution due to impaired cortisol synthesis. To avoid over- or undertreatment, one has to consider auxology as well as biochemical parameters for adrenal derived steroids like androstenedione (A4) and 17-hydroxyprogesterone (17-OHP). There are no established reference values for A4 and 17-OHP in CAH.
Methods
We performed a retrospective study in 53 prepubertal patients with CAH. Datasets of patients were included if the plasma A4 values of the respective clinical visit were under the limit of quantification. Related 17-OHP values were extracted as well as height/length, weight, dose of hydrocortisone, HC regimen, bone age and stages of pubertal development.
Results
Median hydrocortisone doses were in most observations within the recommended reference ranges. Hydrocortisone has a significant negative influence on 17-OHP values and HSDS. Age has a positive significant influence on 17-OHP, BMI-SDS, and HSDS. Median height standard-deviation-score (HSDS) was beneath 0 at all times, but showed an increasing trend in both sexes. Median body mass index standard-deviation-score (BMI-SDS) was above 0 at all times and showed an increasing trend as well.
Conclusions
With guideline-compliant doses of hydrocortisone, suppression of A4 at the respective time of day is possible in prepubertal children. Although HC has a significant negative influence on HSDS, increasing values for HSDS and HC are observed with increasing age. Thus, A4 suppression at the respective time point does not hinder regular growth. An increase in body mass index can already be observed as early as in prepuberty.
Acknowledgments
We would like to thank Amalie Busch and Simone Kroke-Büsgen for the excellent work in our Paediatric Endocrinology Laboratory and Department at the Dr. von Hauner Children’s Hospital.
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Research ethics: The study and the study protocol were advised, reviewed and approved by the local Ethics Committee at LMU Munich, ethical approval number no. 22-0485.
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Informed consent: Not applicable.
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Author contributions: Belana Debor analyzed the data and wrote the manuscript. Ilja Dubinski, Nicole Reisch, Heinrich Schmidt and Susanne Bechtold provided the patients’ clinical data. Ilja Dubinski, Belana Debor and Heinrich Schmidt contributed to study concept and data analysis. Ilja Dubinski, Belana Debor, Heinrich Schmidt, Susanne Bechtold and Nicole Reisch have significantly contributed to this study providing intellectual input to the manuscript and have accepted responsibility for the entire content of this submitted manuscript and approved submission.
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Competing interests: Authors state no conflict of interest.
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Research funding: None declared.
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Articles in the same Issue
- Frontmatter
- Reviews
- Artificial Intelligence (AI) in pediatric endocrinology
- Relationship between vitamin D deficiency and neonatal hypocalcemia: a meta-analysis
- Original Articles
- Associated autoimmune thyroid diseases in children and adolescents with type one diabetes in Jordan
- The therapeutic effect of oral desmopressin lyophilisate formulation in children with central diabetes insipidus
- Effect of complete suppression of androstenedione on auxological development in prepubertal patients with classical congenital adrenal hyperplasia
- Safety and efficacy of the structured onboarding steps and initiation protocol for MiniMed™ 780G system among an Egyptian cohort of young people living with type 1 diabetes
- The effect of problem-solving skills on blood glucose regulation and disease management in children with type 1 diabetes mellitus
- Depression, anxiety, eating problems, and diabulimia risk in adolescents with type 1 diabetes: a case–control study
- Genotyping in patients with congenital adrenal hyperplasia by sequencing of newborn bloodspot samples
- Case Reports
- A pediatric case of autosomal dominant hypocalcemia type 2
- Very severe hypertriglyceridemia complicating pediatric acute lymphoblastic leukemia treatment: a call for management guidelines
- Management of acute metabolic crisis in TANGO2 deficiency: a case report
- Novel PAX4 variant in a child and family with diabetes mellitus – case report and review of the literature
- Graves’ disease after COVID mRNA vaccination for the first time diagnosed in adolescence-case report. Cause and effect relationship or simple coincidence?
