[II型多发性内分泌肿瘤（MEN IIB）：便秘的罕见病因。呼吁早期甲状腺切除术]
[Type II multiple endocrine neoplasms (MEN IIB): an unusual cause of constipation. A plea for early thyroidectomy].
作者信息
Post E D, Wauters E A, Huber J, Williams E D, Lips C J
机构信息
Wilhelmina Kinderziekenhuis, Universiteitskliniek voor kinderen en jeugdigen, afd. alg. Pediatrie, Utrecht.
出版信息
Tijdschr Kindergeneeskd. 1993 Apr;61(2):63-8.
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.
我们报告一名5岁女孩，她被诊断患有IIB型多发性内分泌腺瘤综合征（MEN IIB）。她表现出严重便秘，具有MEN IIB的典型特征。100%的MEN IIB患者会发生甲状腺髓样癌（MTC）。已知MEN IIB中的MTC具有非常侵袭性的行为。尽管降钙素刺激试验正常，但显微镜检查发现该患者已存在MTC。因此，我们主张对MEN IIB的甲状腺切除术采取更激进的治疗方法。
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