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Salpietro V, Polizzi A, Di Rosa G, et al. Adrenal disorders and the paediatric brain: pathophysiological considerations and clinical implications. Int J Endocrinol. 2014;2014:282489doi: 10.1155/2014/282489.
Salpietro, V., Polizzi, A., Di Rosa, G., Romeo, A. C., Dipasquale, V., Morabito, P., Chirico, V., Arrigo, T., & Ruggieri, M. (2014). Adrenal disorders and the paediatric brain: pathophysiological considerations and clinical implications. International journal of endocrinology, 2014282489. https://doi.org/10.1155/2014/282489
Salpietro, Vincenzo, et al. "Adrenal disorders and the paediatric brain: pathophysiological considerations and clinical implications." International journal of endocrinology vol. 2014 (2014): 282489. doi: https://doi.org/10.1155/2014/282489
Salpietro V, Polizzi A, Di Rosa G, Romeo AC, Dipasquale V, Morabito P, Chirico V, Arrigo T, Ruggieri M. Adrenal disorders and the paediatric brain: pathophysiological considerations and clinical implications. Int J Endocrinol. 2014;2014:282489. doi: 10.1155/2014/282489. Epub 2014 Sep 03. PMID: 25276129; PMCID: PMC4167812.
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Int J Endocrinol. 2014;2014:282489. doi: 10.1155/2014/282489. Epub 2014 Sep 03.

Adrenal disorders and the paediatric brain: pathophysiological considerations and clinical implications.

International journal of endocrinology

Vincenzo Salpietro, Agata Polizzi, Gabriella Di Rosa, Anna Claudia Romeo, Valeria Dipasquale, Paolo Morabito, Valeria Chirico, Teresa Arrigo, Martino Ruggieri

Affiliations

  1. Department of Pediatric Neurology, Chelsea and Westminster Hospital NHS Foundation Trust, 369 Fulham Road, London SW10 9NH, UK ; Unit of Genetics and Paediatric Immunology, Department of Pediatrics, University of Messina, Italy.
  2. National Center for Rare Diseases, Istituto Superiore di Sanità, Rome, Italy ; Institute of Neurological Sciences, National Research Council, Catania, Italy.
  3. Infantile Neuropsychiatry Unit, Department of Pediatrics, University of Messina, Italy.
  4. Unit of Genetics and Paediatric Immunology, Department of Pediatrics, University of Messina, Italy.
  5. Department of Clinical and Experimental Medicine and Pharmacology, University of Messina, Italy.
  6. Chair of Pediatrics, Department of Educational Sciences, University of Catania, Italy.

PMID: 25276129 PMCID: PMC4167812 DOI: 10.1155/2014/282489

Abstract

Various neurological and psychiatric manifestations have been recorded in children with adrenal disorders. Based on literature review and on personal case-studies and case-series we focused on the pathophysiological and clinical implications of glucocorticoid-related, mineralcorticoid-related, and catecholamine-related paediatric nervous system involvement. Childhood Cushing syndrome can be associated with long-lasting cognitive deficits and abnormal behaviour, even after resolution of the hypercortisolism. Exposure to excessive replacement of exogenous glucocorticoids in the paediatric age group (e.g., during treatments for adrenal insufficiency) has been reported with neurological and magnetic resonance imaging (MRI) abnormalities (e.g., delayed myelination and brain atrophy) due to potential corticosteroid-related myelin damage in the developing brain and the possible impairment of limbic system ontogenesis. Idiopathic intracranial hypertension (IIH), a disorder of unclear pathophysiology characterised by increased cerebrospinal fluid (CSF) pressure, has been described in children with hypercortisolism, adrenal insufficiency, and hyperaldosteronism, reflecting the potential underlying involvement of the adrenal-brain axis in the regulation of CSF pressure homeostasis. Arterial hypertension caused by paediatric adenomas or tumours of the adrenal cortex or medulla has been associated with various hypertension-related neurological manifestations. The development and maturation of the central nervous system (CNS) through childhood is tightly regulated by intrinsic, paracrine, endocrine, and external modulators, and perturbations in any of these factors, including those related to adrenal hormone imbalance, could result in consequences that affect the structure and function of the paediatric brain. Animal experiments and clinical studies demonstrated that the developing (i.e., paediatric) CNS seems to be particularly vulnerable to alterations induced by adrenal disorders and/or supraphysiological doses of corticosteroids. Physicians should be aware of potential neurological manifestations in children with adrenal dysfunction to achieve better prevention and timely diagnosis and treatment of these disorders. Further studies are needed to explore the potential neurological, cognitive, and psychiatric long-term consequences of high doses of prolonged corticosteroid administration in childhood.

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