Abstract
The Journal is the primary organ of Continuing Paediatric Medical Education in Sri Lanka. The journal also has a website. Free full text access is available for all readers.The Sri Lanka Journal of Child Health is now indexed in SciVerse Scopus (Source Record ID 19900193609), Index Medicus for South-East Asia Region (IMSEAR), CABI (Centre for Agriculture and Bioscience International Global Health Database), DOAJ and is available in Google, as well as Google Scholar.The policies of the journal are modelled on the Committee on Publication Ethics (COPE) Guidelines on Principles of Transparency and Best Practice in Scholarly Publishing. Sri Lanka Journal of Child Health is recognised by the International Committee of Medical Journal Editors (ICMJE) as a publication following the ICMJE Recommendations.
Highlights
Primary adrenal insufficiencyThe adrenal gland is made up of two distinct embryological cell lines, the cortex derived from mesenchymal cells and the medulla from neuroectodermal cells
Symptoms of adrenal insufficiency appear after symptoms of white matter disease but 20% of the patients had adrenal insufficiency before neurological signs
Marked elevation of adrenocorticotrophic hormone (ACTH) seen in this condition causes hyperpigmentation which does not resolve with cortisol replacement therapy[4].Tall stature is seen in patients with type 2 disease and they present later
Summary
The adrenal gland is made up of two distinct embryological cell lines, the cortex derived from mesenchymal cells and the medulla from neuroectodermal cells. The adrenal cortex consists of three concentric zones, the outer zona glomerulosa secreting the mineralocorticoid aldosterone, the intermediate zona fasciculata secreting cortisol and the inner zona reticularis secreting androgens. Chromaffin cells in the adrenal medulla produce catecholamines. Primary adrenal insufficiency results when the adrenal gland itself becomes dysfunctional. The commonest cause of primary adrenal insufficiency is autoimmune destruction of the adrenal cortex[1]. Autoimmune destruction of the adrenal gland could occur as an isolated event or as part of an autoimmune polyglandular syndrome. The aetiology of primary adrenal insufficiency is given in table 1
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