多内分泌腺腫瘍症第 2A 型家系における小児期スクリーニング検査の臨床的意義

概要

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Eleven members of a family with MEN2A who were 19 years old or younger were evaluated from the viewpoint of appearance of clinical signs or symptoms during infancy, childhood and adolescence and a screening program with measurement of serum calcitonin concentrations with pentagastrin stimulation (O.5μg/kg 5sec. I. V.) was perfomed for the early diagnosis of medullary thyroid carcinoma (MTC). This screening revealed 2 additional patients aged 16 and 19, and total thyroidectomies with regional lymph node dissections were performed. For early detection of MTC in those at high risk for MEN2A, screening programs should be established and performed in their childhood and adolescence on the basis of the precise natural history of this syndrome.
日本小児外科学会の論文
1990-06-20