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[Type II multiple endocrine neoplasms (MEN IIB): an unusual cause of constipation. A plea for early thyroidectomy]
E D Post1, E A Wauters, J Huber
1Wilhelmina Kinderziekenhuis, Universiteitskliniek voor kinderen en jeugdigen, afd. alg. Pediatrie, Utrecht.
Tijdschrift Voor Kindergeneeskunde
|April 1, 1993
Abstract:
We present a 5 year old girl who was diagnosed to have multiple endocrine neoplasia syndrome type IIB (MEN IIB). She presented with serious constipation and had the distinct features of MEN IIB. 100% of the patients with MEN IIB develop medullary thyreoïd carcinoma (MTC). MTC in MEN IIB is known to behave in a very aggressive way. Microscopically MTC was already present in this patient despite of normal calcitonin stimulation tests. Accordingly we plead for a more radical management relating to thyreoïdectomy in MEN IIB.