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Human Physiology - a question related to the endocrine system.

A 14 day old child has a very low blood cortisol level and very high salt Na+ concentration in their urine and will require lifelong medication for survival.

Define the disorder that leads to these clinical results. Also explain what is happening within the adrenal gland itself and what medication/s would be prescribed, and how they work to save the life of the child. (10 marks worth)

Posted Aug 26 2019

Answered on Aug 28 2019
A 14 day old child has a very low blood cortisol level and very high salt Na+ concentration in their urine and will require lifelong medication for survival. Yes, life-long medication is needed for survival. Girls with this disorder require surgery to restore their genital appearance to normal. Boys do not. This is a condition comparises with Congenital adrenal hyperplasia (CAH) . It's an inherited (genetic) disorder. It causes the adrenal glands to swell. This disorder compares with a decrease in the blood level of a hormone called cortisol and an increase in the level of male sex hormones (androgens) in both sexes. This is a condition compared to Congenital adrenal hyperplasia (CAH). It is an inherited (genetic) disorder, Adrenal gland make the hormones cortisol and aldosterone. The cortisol amount is controlled by another hormone called adrenocorticotrophic hormone (ACTH). ACTH is made of the pituitary gland. ACTH passes into the bloodstream, is transported to the adrenal glands and stimulates the adrenal glands to make cortisol. Adrenal cortex cell also produces small amounts of sex hormones such as testosterone and oestrogen, although most of these are made in the testicles (tests) and ovaries. In this disorder, the cortisol level is low, and the lower cortisol level results in increased ACTH. Thishigher level of ACTH stimulation induces overgrowth (hyperplasia) and overactivity of the steroid-producing cells of the adrenal cortex. The defects causing adrenal hyperplasia are congenital (i.e. present at birth). Medicatation would be prescribed There are several medications are substitutes for the natural hormone, cortisol. They include hydrocortisone, cortisone, prednisolone and dexamethasone. It helps to maintain cortisol levels in the blood. They differ in power (strength) and duration of action. Fludrocortisones are used as a hormone for salt retention. It reduces the loss of salt in urine. Dexamethasone is generally not advise to infant because its high potency and very long action (24-48 hours) interferes with growth. Blood tests are essential every three months throughout childhood. It is used to monitor, replace medication therapy. Cortisone (hydrocortisone) injection may be required if a person with CAH becomes very ill or needs surgery. In girls, male features would appear to be so aviod that surgery has to be done. Due to surgery, it reduces the size of the clitoris to normal, separates the fused labia and enlarges the vaginal entrance. The technical name for this operation is Ëœclitoral recession or reduction and vaginoplasty'.It's done in the first few months of life. Thus, the cortisol substitute mentioned above can maintain the leve of cortisol and helps to save the life of the child. And fludrocortisones are used as a retaining hormone for salt. It reduces the loss of salt in urine.

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