Individuals with MEN 2B show a variety of additional conditions: a characteristic facial appearance with swollen lips; tumors of the mucous membranes of the eye, mouth, tongue, and nasal cavity; enlarged colon; and skeletal abnormalities.
Symptoms develop early in life (often before five years of age) in cases of MEN 2B and the medullary thyroid cancer is much more aggressive and may develop in patients who are one year old.
Patients with both MEN 2A and MEN 2B experience two main symptoms, medullary thyroid cancer (MTC) and a tumor of the adrenal gland medulla known as pheochromocytoma.
Diagnosed early through genetic testing, the prognosis for the MEN diseases is reasonably good, even for MEN 2B, the most dangerous of the three forms.
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There are three types of MEN: MEN 1 (Wermer's syndrome), MEN 2A (Sipple syndrome), and MEN 2B (previously known as MEN 3).
Patients with type 2B VWD should not be treated with this medication, since DDAVP can induce dangerous platelet clumping.
A number of different mutations can lead to MEN 2A, but only one specific genetic alteration causes MEN 2B.
In the case of MEN 2A and MEN 2B, children would undergo frequent calcitonin testing.
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