POMC
There is a rapid onset of weight gain shortly after birth. Children can weigh more than 33 lbs at 1 year old and more than 55 lbs by 3 years old. There have only been 6 cases of obesity from POMC mutations reported so far. Proopiomelanocortin is also converted into the hormone ACTH. Loss of ACTH, which stimulates the adrenal glands, causes a decrease of the adrenal gland's function. Low blood pressure, fatigue, and other symptoms arise. Symptoms are apparent early in life, and lifelong replacement of glucocorticoids are needed to prevent adrenal crises. Babies can have prolonged jaundice and are more vulnerable to infection. Additionally, a-MSH also signals the pigment cells in the skin. People with this genetic defect have pale skin and most have red hair. The adrenal glands make certain types of steroids. Replacing them early helps with many of these problems. However, the hunger remains and children with proopiomelanocortin mutations ultimately become obese.
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It is important you
discuss any weight loss or exercise plan with your doctor. Only you and
your physician can decide what is best for you. Some people have
certain conditions that prevent them from doing all exercises, and goal
body weights may be different for different people. You need to discuss
all these things with your physician before starting any weight loss or
exercise program. This article was written by John Vickery, MD. References Fauci et al., Harrison's Principles of Internal Medicine 17th ed., ch 74 Farooqi and O'Rhilly. Endocrine Reviews 27(7):710-718 Endocrinol Metab Clin N Am 2008;37:733-751
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