Diagnosis and management of pediatric adrenal insufficiency
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Diagnosis and management of pediatric adrenal insufficiency Ahmet Uçar, Firdevs Baş, Nurçin Saka Istanbul, Turkey
Data sources: We reviewed the recent original publications and classical data from the literature, as well as the clinical, diagnostic and management strategies of pediatric AI. Results: Practical points in the diagnosis and management of AI with an emphasis on iatrogenic AI, neonatal AI, and CIRCI are provided. Given the lack of sensitive and practical biochemical tests for diagnosis of subtle AI, GC treatment has to be tailored to highly suggestive clinical symptoms and signs. Treatment of adrenal crisis is well standardized and patients almost invariably respond well to therapy. It is mainly the delay in treatment that is responsible for mortality in adrenal crisis. Conclusion: Education of patients and health care professionals is mandatory for timely interventions for patients with adrenal crisis. World J Pediatr 2016;12(3):261-274 Key words: adrenal insufficiency; critical illness; hypothalamo-pituitary-adrenal axis; iatrogenic; steroids
Author Affiliations: Growth-Development and Pediatric Endocrine Unit, Istanbul School of Medicine, Istanbul University, Turkey (Uçar A, Baş F, Saka N) Corresponding Author: Ahmet Uçar, MD, Growth-Development and Pediatric Endocrine Unit, Istanbul School of Medicine, Istanbul University, Turkey (Tel: +902124142000-31645; Fax: +902125831333; Email: aucar76@ yahoo.com; [email protected]) doi: 10.1007/s12519-016-0018-x Online First April 2016 ©Children's Hospital, Zhejiang University School of Medicine, China and Springer-Verlag Berlin Heidelberg 2016. All rights reserved.
World J Pediatr, Vol 12 No 3 . August 15, 2016 . www.wjpch.com
Introduction
A
drenal insufficiency (AI) encompasses a wide spectrum of life-threatening disorders related to the congenital or acquired pathologies of the adrenal cortex (primary AI), pituitary gland (secondary AI) or hypothalamus (tertiary AI). The clinical findings of primary AI are associated with deficient synthesis or release of glucocorticoids (GCs) and frequent deficiency of mineralocorticoids. [1-3] Secondary and tertiary AI are associated with deficiencies of adrenocorticotropic hormone (ACTH) and corticotropin releasing hormone (CRH), respectively. Consequently, biochemical findings of mineralocorticoid deficiency are not expected in the latter two subtypes of AI.
Physiology of the adrenal functions
The mature adrenal cortex comprises the zona glomerulosa (outermost layer), zona fasciculata (intermediate zone), and zona reticularis (innermost layer), which are associated with aldosterone, cortisol and androgen synthesis, respectively. The adrenal cortex is under control of the hypothalamo-pituitary-adrenal (HPA) axis for regulation of cortisol synthesis, which is stimulated by CRH and arginine vasopressin secretion from the hypothalamus and by ACTH secretion from the pituitary gland. Mineralocorticoid synthesis is regulated by the renin-angiotensin-aldosterone system, sympathetic innervation, blood pressure, i
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