Zaĭrat'iants O V, Berestova A V, Proskurneva E P
Arkh Patol. 1985;47(10):70-8.
48 autopsy cases of thymomegaly in children up to 14 years and in adults from 18 to 55 years are studied. The patients died either from the acute or chronic infectious diseases resistant to the therapy or suddenly during or several hours after the diagnostic manipulations, small surgical interventions. Analysis of clinical data and morphological immunohistochemical and ultrastructural studies of the thymus and organs of the immune and endocrine system showed the thymomegaly in the majority of cases to be a local manifestation of the endocrine-immune deficiency syndrome. Primary (congenital) and secondary (acquired) thymomegaly are to be distinguished. A primary thymomegaly occurs in children and adults having congenital pluriglandular endocrinopathy with hypocortisism as well as an adrenal hypo- or aplasia with an antigenic stimulation. A secondary thymomegaly occurs in children of the first year of life and reflects the first phase of the thymic response in the activation of the immune T-system predominantly prolonged due to the immature state of the hypothalamico-hypophyseal-adrenal system. In the adults secondary thymomegaly occurs in cases of the acquired endocrinopathy with hypocortisism.
对48例年龄在14岁以下儿童及18至55岁成人的胸腺肿大尸检病例进行了研究。这些患者死于对治疗有抵抗性的急性或慢性传染病，或者在诊断操作、小型外科手术后或数小时内突然死亡。对临床数据以及胸腺和免疫与内分泌系统器官的形态学、免疫组织化学和超微结构研究表明，大多数情况下胸腺肿大是内分泌 - 免疫缺陷综合征的局部表现。需区分原发性（先天性）和继发性（后天性）胸腺肿大。原发性胸腺肿大发生于患有先天性多腺体内分泌病伴皮质功能减退以及肾上腺发育不全或发育不良且有抗原刺激的儿童和成人。继发性胸腺肿大发生于1岁儿童，反映了免疫T系统激活时胸腺反应的第一阶段，主要是由于下丘脑 - 垂体 - 肾上腺系统不成熟状态而延长。在成人中，继发性胸腺肿大发生于获得性内分泌病伴皮质功能减退的病例。
