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Cited by 42Sexual Differentiation and DisordersAdrenal Hormones and DisordersMetabolism and Genetic Disorders

Growth Analysis in Patients with 21-Hydroxylase Deficiency Influence of Glucocorticoid Dosage, Age at Diagnosis, Phenotype and Genotype on Growth and Height Outcome

Grigorescu-Sido, Anca, Bettendorf, Markus, Schulze, Egbert, Duncea, Ileana, Heinrich, Udo·2003·Hormone Research in Paediatrics
DOI 10.1159/000071876
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Abstract

Objective To evaluate the impact of hydrocortisone dosage, age at diagnosis, compliance, genotype and phenotype on growth and height outcome in 21-hydroxylase-deficient patients. Methods We analyzed 37 patients with 21-hydroxylase deficiency (17 had completed growth, 20 still growing). Final (FH)/predicted final height (pFH) and loss of height potential related to target height (TH) were calculated and the impact of 4 hydrocortisone (HC) dosage regimens on height outcome and growth velocities was evaluated. Mean FH SDS and pFH SDS were analyzed in accordance to age at diagnosis, compliance, genotype and phenotype. Results Mean (FH SDS, pFH SDS) was -1.8+/-1.06 SD, with 35.1% of all 37 patients exhibiting short stature. Doses >20 mg/m2/day during the first year and >15 mg/m2/day during age 1-5 and at puberty resulted in significantly lower FH SDS, pFH SDS and greater height losses. Age at diagnosis, compliance, genotype and phenotype played only a minor role in growth development. Conclusions Hydrocortisone substitution in 21-hydroxylase-deficient patients should be kept at the lowest efficient level, if possible <20 during the first year and <15 mg/m2/day until age 5 and during puberty. Normal growth and not complete androgen suppression should be aimed for.

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Crossref · 10.1159/000071876

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3 of 3 references verified0 citation errors · 0 not checkable

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  • In patients with CAH, glucocorticoid dosages exceeding 20 mg/m2/d in infants and 15 to 17 mg/m2/d in adolescents have been shown to result in loss of height and shorter adult stature.

    “In patients with CAH, glucocorticoid dosages exceeding 20 mg/m 2 /d in infants and 15 to 17 mg/m 2 /d in adolescents have been shown to result in loss of height and shorter adult stature (124, 125).”

    From Diagnosis and Treatment of Primary Adrenal Insufficiency: An Endocrine Society Clinical Practice Guideline (2016) · cites this paper as [125]

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Authors
Grigorescu-Sido, A., Bettendorf, M., Schulze, E., Duncea, I., & Heinrich, U.
Affiliations
  • Iuliu Hațieganu University of Medicine and Pharmacy (RO)
  • Heidelberg University (DE)
ORCID
Bettendorf, Markus
Venue
Hormone Research in Paediatrics
Publisher
S. Karger AG
Published
2003
Volume
60
Issue
2
Pages
84-90
ISSN
  • 1663-2818 (ISSN-L)
  • 1663-2826
DOI
10.1159/000071876
OpenAlex
W2089639144
Cited by
42
References
3
Citations by year
2026: 2 · 2025: 1 · 2024: 2 · 2023: 3 · 2022: 3 · 2021: 3 · 2019: 3 · 2018: 2 · 2017: 2 · 2016: 3 · 2015: 2 · 2014: 1 · 2013: 2 · 2012: 2
FWCI
0.57
Type
journal article
Language
en
Open access
closed
Licence
Locations
  • Hormone Research in Paediatrics (publishedVersion)
  • PubMed (publishedVersion)
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Sexual Differentiation and Disorders
  • Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Adrenal Hormones and Disorders
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
Keywords
21-hydroxylase deficiency, congenital adrenal hyperplasia, glucocorticoid therapy, adult height, short stature, age at diagnosis, medication adherence, genotypes, phenotypes, growth rate, puberty, androgen deprivation therapy
MeSH terms
Adolescent, Adult, Body Height, Child, Child, Preschool, Dose-Response Relationship, Drug, Female, Follow-Up Studies, Genotype, Humans, Hydrocortisone, Infant, Male, Metabolism, Inborn Errors, Phenotype, Retrospective Studies, Steroid 21-Hydroxylase

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