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The occurrence of multiple endocrine neoplasia Type II b , in two children of an affected mother
Author(s) -
Sciubba J.J.,
D'Amico E.,
Attie J.N.
Publication year - 1987
Publication title -
journal of oral pathology and medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.887
H-Index - 83
eISSN - 1600-0714
pISSN - 0904-2512
DOI - 10.1111/j.1600-0714.1987.tb00700.x
Subject(s) - medicine , multiple endocrine neoplasia , stigmata , medullary cavity , medullary carcinoma , thyroidectomy , thyroid , endocrine system , multiple endocrine neoplasia type 2 , thyroid carcinoma , daughter , pathology , pediatrics , surgery , hormone , biochemistry , chemistry , archaeology , gene , history , germline mutation , evolutionary biology , biology , mutation
Two children with multiple endocrine neoplasia syndrome, Type II b , (MEN II b ), whose natural mother died from complications of general anesthesia, due to undiagnosed bilateral pheochromocytomas, arc described. Of particular interest is the fact that the daughter presented with many stigmata of the syndrome, including the typical facies, while her brother had no obvious clinical manifestations of the syndrome. Both children to date have undergone total thyroidectomy, and pathology reports have confirmed the presence of medullary thyroid carcinoma.