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多发性内分泌腺肿瘤综合征(Multiple endocrine neoplasia syndrome-MEN)在50年代称多发性内分泌腺腺瘤(Multiple endocrine adenoma-MEA)综合征,由于许多肿瘤是恶性的而改为MEN综合征。近来认为内分泌腺的病变有良、恶性肿瘤及腺体增生,又有人提出多发性内分泌腺病变(Multiple endocrine adenopathy-MEA)综合征,习惯上仍用MEN综合征。MEN综合征有散发性和遗传性两类,后者有明显的家族史,家族里约有半数患病。MEN综合征是由于常染色体多基因缺陷所致的显性遗传性疾病,无一代遗漏。Pearse提出很多内分泌细胞包括神经内分泌细胞起源于外胚层,从外胚层的神经嵴演化而来,这些细胞具有特异的细胞化学特性和超微结构,电镜证实胞浆内有特殊的分泌颗粒,其内有胺前体和脱羧酶,产生具有特殊内分泌功能的多肽。这些细胞分布于全身很多的内分泌器官内,称
Multiple endocrine neoplasia syndrome (MEN) is called multiple endocrine adenoma-MEA syndrome in the 1950s and changed to MEN syndrome because many tumors are malignant. Recently, it has been suggested that there are benign and malignant tumors and glandular hyperplasia in endocrine glands, and multiple endocrine adenopathy (MEA) syndrome has been proposed. It is customary to use MEN syndrome. There are two types of MEN syndromes, sporadic and hereditary. The latter has a distinct family history. About half of the family members are affected. The MEN syndrome is a dominant hereditary disease caused by an autosomal polygenic defect. There is no one omission. Pearse proposed that many endocrine cells, including neuroendocrine cells, originate from the ectoderm and evolved from the neural crest of the ectoderm. These cells have specific cytochemical properties and ultrastructure. Electron microscopy has demonstrated that there are special secretory granules in the cytoplasm. There are amine precursors and decarboxylases that produce peptides with specific endocrine functions. These cells are distributed throughout the body’s many endocrine organs.