Cushing's Syndrome is a condition where children produce too much cortisol, which is made in the adrenal gland.
Cushing's Syndrome can be ACTH dependent or ACTH independent.
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Pathophysiology of Cushing's Syndrome
Normally, the hypothalamus sends the hormone CRH to the anterior pituitary gland, which stimulates ACTH production. ACTH then stimulates the adrenal gland to secrete cortisol. Cortisol in the blood regulates the hypothalamus and anterior pituitary production of CRH and ACTH. Elevated levels of cortisol start a negative feedback, the hypothalamus decreases the amount of CRH, which decreases the amount of ACTH.
In ACTH dependent Cushing's Syndrome, a small tumour causes increased production of ACTH. This makes up for 70% of cases. This causes blood ACTH levels to be elevated, along with cortisol levels. ACTH levels remain elevated as the tumour is unresponsive to negative feedback from high cortisol levels.
In ACTH independent Cushing's Syndrome (Cushing's Disease), the adrenal gland produces too much cortisol, and negative feedback cause ACTH levels to be very low. This can be caused by corticosteroids such as asthma inhalers and eczema creams.
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Symptoms of Cushing's Syndrome
'Moon Face' and Weight Gain - Due to the rise of cortisol levels in the blood, this causes rapid weight gain o the face, chest and abdomen.
Stretch Marks - Caused by rapid weight gain.
Muscle Weakness - Lack of ACTH and rise of cortisol in the blood causes weakness in the muscles of the arms and legs.
Mood Disturbance - High levels of cortisol in the blood can alter mood.
Growth Failure in Children - High cortisol levels can have a negative impact on the release of growth hormone, leading to a lack of growth.
Excess Androgen Production - Overproduction of androgen hormones such as testosterone can lead to exaggerated male sex characteristics such as excessive hair growth and acne.
Hypertension - High levels of cortisol in the blood enhance the vasoconstrictive effects of adrenaline, raising blood pressure.
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Cushing's Syndrome Treatment
Stopping use of corticosteroids - In those with ACTH independent Cushing's Syndrome, this can be treated by stopping use of the medication which causes it.
Removal of Tumours - In ACTH dependent Cushing's Syndrome, removal of the tumour will improve symptoms.
Medications - Glucocorticoid receptor antagonists can be helpful in managing symptoms.
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