Hypoglycaemia in adrenal insufficiency
Adrenal insufficiency encompasses a group of congenital and acquired disorders that lead to inadequate steroid production by the adrenal glands, mainly glucocorticoids, mineralocorticoids and androgens. These may be associated with other hormone deficiencies. Adrenal insufficiency may be primary, af...
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| Format: | Article |
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Frontiers Media S.A.
2023-11-01
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| Series: | Frontiers in Endocrinology |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fendo.2023.1198519/full |
| _version_ | 1797564380412379136 |
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| author | Shien Chen Lee Elizabeth S. Baranowski Rajesh Sakremath Vrinda Saraff Vrinda Saraff Zainaba Mohamed Zainaba Mohamed |
| author_facet | Shien Chen Lee Elizabeth S. Baranowski Rajesh Sakremath Vrinda Saraff Vrinda Saraff Zainaba Mohamed Zainaba Mohamed |
| author_sort | Shien Chen Lee |
| collection | DOAJ |
| description | Adrenal insufficiency encompasses a group of congenital and acquired disorders that lead to inadequate steroid production by the adrenal glands, mainly glucocorticoids, mineralocorticoids and androgens. These may be associated with other hormone deficiencies. Adrenal insufficiency may be primary, affecting the adrenal gland’s ability to produce cortisol directly; secondary, affecting the pituitary gland’s ability to produce adrenocorticotrophic hormone (ACTH); or tertiary, affecting corticotrophin-releasing hormone (CRH) production at the level of the hypothalamus. Congenital causes of adrenal insufficiency include the subtypes of Congenital Adrenal Hyperplasia, Adrenal Hypoplasia, genetic causes of Isolated ACTH deficiency or Combined Pituitary Hormone Deficiencies, usually caused by mutations in essential transcription factors. The most commonly inherited primary cause of adrenal insufficiency is Congenital Adrenal Hyperplasia due to 21-hydroxylase deficiency; with the classical form affecting 1 in 10,000 to 15,000 cases per year. Acquired causes of adrenal insufficiency can be subtyped into autoimmune (Addison’s Disease), traumatic (including haemorrhage or infarction), infective (e.g. Tuberculosis), infiltrative (e.g. neuroblastoma) and iatrogenic. Iatrogenic acquired causes include the use of prolonged exogenous steroids and post-surgical causes, such as the excision of a hypothalamic-pituitary tumour or adrenalectomy. Clinical features of adrenal insufficiency vary with age and with aetiology. They are often non-specific and may sometimes become apparent only in times of illness. Features range from those related to hypoglycaemia such as drowsiness, collapse, jitteriness, hypothermia and seizures. Features may also include signs of hypotension such as significant electrolyte imbalances and shock. Recognition of hypoglycaemia as a symptom of adrenal insufficiency is important to prevent treatable causes of sudden deaths. Cortisol has a key role in glucose homeostasis, particularly in the counter-regulatory mechanisms to prevent hypoglycaemia in times of biological stress. Affected neonates particularly appear susceptible to the compromise of these counter-regulatory mechanisms but it is recognised that affected older children and adults remain at risk of hypoglycaemia. In this review, we summarise the pathogenesis of hypoglycaemia in the context of adrenal insufficiency. We further explore the clinical features of hypoglycaemia based on different age groups and the burden of the disease, focusing on hypoglycaemic-related events in the various aetiologies of adrenal insufficiency. Finally, we sum up strategies from published literature for improved recognition and early prevention of hypoglycaemia in adrenal insufficiency, such as the use of continuous glucose monitoring or modifying glucocorticoid replacement. |
| first_indexed | 2024-03-10T18:56:31Z |
| format | Article |
| id | doaj.art-99e591fbe498437aa4ef5679726a3553 |
| institution | Directory Open Access Journal |
| issn | 1664-2392 |
| language | English |
| last_indexed | 2024-03-10T18:56:31Z |
| publishDate | 2023-11-01 |
| publisher | Frontiers Media S.A. |
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| series | Frontiers in Endocrinology |
| spelling | doaj.art-99e591fbe498437aa4ef5679726a35532023-11-20T04:42:45ZengFrontiers Media S.A.Frontiers in Endocrinology1664-23922023-11-011410.3389/fendo.2023.11985191198519Hypoglycaemia in adrenal insufficiencyShien Chen Lee0Elizabeth S. Baranowski1Rajesh Sakremath2Vrinda Saraff3Vrinda Saraff4Zainaba Mohamed5Zainaba Mohamed6Department of Paediatrics, Princess Royal Hospital, Telford, United KingdomDepartment of Paediatric Endocrinology, Birmingham Women’s and Children’s Hospital, Birmingham, United KingdomDepartment of Paediatrics, Princess Royal Hospital, Telford, United KingdomDepartment of Paediatric Endocrinology, Birmingham Women’s and Children’s Hospital, Birmingham, United KingdomCentre for Endocrinology, Diabetes and Metabolism, University of Birmingham, Birmingham, United KingdomDepartment of Paediatric Endocrinology, Birmingham Women’s and Children’s Hospital, Birmingham, United KingdomCentre for Endocrinology, Diabetes and Metabolism, University of Birmingham, Birmingham, United KingdomAdrenal insufficiency encompasses a group of congenital and acquired disorders that lead to inadequate steroid production by the adrenal glands, mainly glucocorticoids, mineralocorticoids and androgens. These may be associated with other hormone deficiencies. Adrenal insufficiency may be primary, affecting the adrenal gland’s ability to produce cortisol directly; secondary, affecting the pituitary gland’s ability to produce adrenocorticotrophic hormone (ACTH); or tertiary, affecting corticotrophin-releasing hormone (CRH) production at the level of the hypothalamus. Congenital causes of adrenal insufficiency include the subtypes of Congenital Adrenal Hyperplasia, Adrenal Hypoplasia, genetic causes of Isolated ACTH deficiency or Combined Pituitary Hormone Deficiencies, usually caused by mutations in essential transcription factors. The most commonly inherited primary cause of adrenal insufficiency is Congenital Adrenal Hyperplasia due to 21-hydroxylase deficiency; with the classical form affecting 1 in 10,000 to 15,000 cases per year. Acquired causes of adrenal insufficiency can be subtyped into autoimmune (Addison’s Disease), traumatic (including haemorrhage or infarction), infective (e.g. Tuberculosis), infiltrative (e.g. neuroblastoma) and iatrogenic. Iatrogenic acquired causes include the use of prolonged exogenous steroids and post-surgical causes, such as the excision of a hypothalamic-pituitary tumour or adrenalectomy. Clinical features of adrenal insufficiency vary with age and with aetiology. They are often non-specific and may sometimes become apparent only in times of illness. Features range from those related to hypoglycaemia such as drowsiness, collapse, jitteriness, hypothermia and seizures. Features may also include signs of hypotension such as significant electrolyte imbalances and shock. Recognition of hypoglycaemia as a symptom of adrenal insufficiency is important to prevent treatable causes of sudden deaths. Cortisol has a key role in glucose homeostasis, particularly in the counter-regulatory mechanisms to prevent hypoglycaemia in times of biological stress. Affected neonates particularly appear susceptible to the compromise of these counter-regulatory mechanisms but it is recognised that affected older children and adults remain at risk of hypoglycaemia. In this review, we summarise the pathogenesis of hypoglycaemia in the context of adrenal insufficiency. We further explore the clinical features of hypoglycaemia based on different age groups and the burden of the disease, focusing on hypoglycaemic-related events in the various aetiologies of adrenal insufficiency. Finally, we sum up strategies from published literature for improved recognition and early prevention of hypoglycaemia in adrenal insufficiency, such as the use of continuous glucose monitoring or modifying glucocorticoid replacement.https://www.frontiersin.org/articles/10.3389/fendo.2023.1198519/fullhypoglycaemiaadrenal insufficiencyhypoadrenalismcortisolglucocorticoid |
| spellingShingle | Shien Chen Lee Elizabeth S. Baranowski Rajesh Sakremath Vrinda Saraff Vrinda Saraff Zainaba Mohamed Zainaba Mohamed Hypoglycaemia in adrenal insufficiency Frontiers in Endocrinology hypoglycaemia adrenal insufficiency hypoadrenalism cortisol glucocorticoid |
| title | Hypoglycaemia in adrenal insufficiency |
| title_full | Hypoglycaemia in adrenal insufficiency |
| title_fullStr | Hypoglycaemia in adrenal insufficiency |
| title_full_unstemmed | Hypoglycaemia in adrenal insufficiency |
| title_short | Hypoglycaemia in adrenal insufficiency |
| title_sort | hypoglycaemia in adrenal insufficiency |
| topic | hypoglycaemia adrenal insufficiency hypoadrenalism cortisol glucocorticoid |
| url | https://www.frontiersin.org/articles/10.3389/fendo.2023.1198519/full |
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