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Title: Pheochromocytoma-induced hypertensive encephalopathy revealing MEN-IIa syndrome in a 13-year old boy. Implications for screening procedures and surgery. Author: Jadoul M, Leo JR, Berends MJ, Ooms EC, Buurke EJ, Vasen HF, Seelen PJ, Lips CJ. Journal: Horm Metab Res Suppl; 1989; 21():46-9. PubMed ID: 2572528. Abstract: In the multiple endocrine neoplasia (MEN) type II syndrome, pheochromocytomas become manifest at a later age than medullary thyroid carcinomas (MTC) do. The present report concerns a 13-year-old boy, belonging to a MEN-IIA kindred, who was admitted because of convulsive seizures related to hypertensive encephalopathy. A pheochromocytoma was suspected immediately and appropriate medical therapy was initiated. A right adrenal pheochromocytoma was removed, as well as a pheochromocytoma of an accessory right adrenal gland. Today, three years later our patient is still asymptomatic and the results of the thyroid C-cell provocative tests remain normal. This case clearly justifies the conclusion that periodic investigation of MEN-II family members to detect both medullary thyroid carcinoma and pheochromocytoma should begin early in life, as the latter may be the initial life-threatening expression of the disease. Long-term follow-up of patients treated with unilateral adrenalectomy will permit better definition of the risk of contralateral recurrence in such cases.[Abstract] [Full Text] [Related] [New Search]