Natural history, treatment, and long-term follow up of patients with multiple endocrine neoplasia type 2B: An international, multicentre, retrospective study
The Lancet Diabetes & Endocrinology Feb 25, 2019
Castinetti F, et al. - In this retrospective, multicenter, international study, researchers sought to raise awareness and improve the recognition of the phenotype and natural history of multiple endocrine neoplasia type 2B. There were 345 patients, 338 of whom had a thyroidectomy. A high likelihood of cure was linked with thyroidectomy performed up to 1 year of age, however it’s after this recommended age that most children will be diagnosed. In multiple endocrine neoplasia type 2B, adrenal-sparing surgery is possible and offers a good chance of regular adrenal function. All healthcare providers must be aware of the extra-endocrine signs and natural history of this rare syndrome in order to improve prognosis of these patients.
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