Disorder of Adrenal Function:
The disorders of adrenal function may lead to acute and chronic .adrenocortical insufficiency. Let us briefly describe each one as follows.
Acute Adrenocortical Insufficiency:
This is a rare disorder and may result from variety of causes such as haemorrhage in glands from trauma due to difficult labour, meningococcal infections and or abrupt withdrawal of exogenous sources of cortisone or failure to increase exogenous supplies during stress or congenital adreno genital hyperplasia. Management includes replacement of cortisol, body fluids to correct dehydration and hypovolemia administration of glucose to correct hypoglycemia and specific antibiotic therapy. Blood transfusion is given if haemorrhage is severe.
Chronic Adrenocortical Insufficiency (Addison's Disease):
This disorder rarely occurs in children. It is caused by destructive lesion of the adrenal glands or a neoplasm, or it is idiopathic. The child may present with neurologic symptoms such as muscular weakness, mental fatigue, irritability pigmentry changes, palinar creases, hyperpigmentation over pressure points, dehydration, anorexia weight loss, headache, hunger, sweating and weakness etc. Therapeutic treatment includes replacement of cotisol and aldosterone.
Cushings syndrome is uncommon in children. It is a characteristic group of manifestations caused by excessive circulating free cortisol. (Fig. 5.2). It may occur due to one or more of the following causes:
Pituitary with adrenal hyperplasia, usually attributed to an excess of ACTH
Adrenal with hypersecretion of glucocorticoids, generally the result of adrenocortical neoplasms
Ectopic with autonomous secretion of ACTH, most often caused by extrapituitary neoplasms Iatrogenic, frequently the result of administration of large amounts of exogenous coricosterioids Food dependent, inappropriate sensitivity of adrenal glands to normal postprandial increases in secretion of gastric inhibitory polypeptide Therapeutic management involves bilateral adrenalectomy and post operative replacement of the cortical hormgnes.