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Cited by 30Adrenal Hormones and DisordersSexual Differentiation and DisordersMetabolism and Genetic Disorders

Hypoglycemia during acute illness in children with classic congenital adrenal hyperplasia

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Keil, Margaret F., Bosmans, Charlotte, Van Ryzin, Carol, Merke, Deborah P.·2010·Journal of Pediatric Nursing
DOI 10.1016/j.pedn.2008.06.003PMC PMC2819226
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Abstract

Congenital adrenal hyperplasia (CAH) describes a group of genetic, autosomal recessive conditions, where there is a block in cortisol biosynthesis. Approximately 95 percent of cases are due to 21-hydroxylase deficiency, which is discussed in this article. Patients with the severe or classic form of CAH have epinephrine deficiency in addition to cortisol deficiency. Both epinephrine and cortisol are important counterregulatory hormones and help prevent hypoglycemia during physical stress. This is the first prospective study to evaluate the incidence of hypoglycemia during acute illness in children with classic CAH. Our objective was to examine blood glucose levels and symptoms of these children during the physical stressor of a typical acute illness managed at home. Twenty patients, ages 3 to 10 years with classic CAH participated. Parents were instructed regarding management of illnesses, home blood glucose monitoring and questionnaire completion. Over 29 months, 20 patients completed questionnaires and 6 patients performed home blood glucose monitoring. A blood glucose of <60 mg/dL was documented in 3 out of 8 monitored acute illness episodes, and in 2 out of 6 of monitored children. The acute illness episodes with documented blood glucose <60 mg/dL were not associated with vomiting. Our data suggest that children with classic CAH may experience lowering of blood glucose during illnesses, and patient education regarding the management of common childhood illness should include glucose supplementation.

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  • When stressed, infants and toddlers with CAH are at high risk for morbidity and mortality from adrenal crises and severe hypoglycemia, with epinephrine deficiency possibly contributing to further risk.

    “When stressed, infants and toddlers with CAH are at high risk for morbidity and mortality from adrenal crises and severe hypoglycemia, with epinephrine deficiency possibly contributing to further risk (19).”

    From Decreased adrenomedullary function in infants with classical congenital adrenal hyperplasia. (2014) · cites this paper as [19]

    The Introduction and Discussion say that young children with classic CAH are at risk of hypoglycemia during illness, that stress-dose hydrocortisone helps prevent adrenal crisis, and that epinephrine deficiency may contribute. But the study enrolled children aged 3–10, not infants and toddlers; it measured significant glucose lowering rather than specifically severe hypoglycemia and did not establish a high risk of mortality. (LLM, conf=0.86)

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Authors
Keil, M. F., Bosmans, C., Van Ryzin, C., & Merke, D. P.
Affiliations
National Institutes of Health (US)
Corresponding author
Keil, Margaret F.
ORCID
Venue
Journal of Pediatric Nursing
Publisher
Elsevier BV
Published
2010-02
Volume
25
Issue
1
Pages
18-24
ISSN
0882-5963 (ISSN-L)
DOI
10.1016/j.pedn.2008.06.003
PMC
PMC2819226
OpenAlex
W2089955349
Cited by
30
References
17
Citations by year
2024: 5 · 2023: 3 · 2022: 3 · 2021: 2 · 2020: 2 · 2019: 1 · 2018: 5 · 2017: 3 · 2016: 1 · 2014: 2 · 2013: 1 · 2012: 1
FWCI
0.24
Type
journal article
Language
en
Open access
Licence
Locations
  • Journal of Pediatric Nursing (publishedVersion)
  • PubMed (publishedVersion)
  • PubMed Central (acceptedVersion)
Funders
  • CARES Foundation
  • LifeScan
  • National Institutes of Health (Z99HD999999)
Topics
  • Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Adrenal Hormones and Disorders
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Sexual Differentiation and Disorders
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
Keywords
congenital adrenal hyperplasia, 21-hydroxylase deficiency, hypoglycemia, acute illness, children, cortisol deficiency, counterregulatory hormones, home blood glucose monitoring, glucose supplementation
MeSH terms
Acute Disease, Adrenal Hyperplasia, Congenital, Blood Glucose, Child, Child, Preschool, Epinephrine, Female, Fever, Genotype, Home Nursing, Humans, Hydrocortisone, Hypoglycemia, Male, Patient Education as Topic, Prospective Studies, Surveys and Questionnaires, Severity of Illness Index, Blood Glucose Self-Monitoring, Incidence

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