Ivanovskaia T E, Leonova L V
Arkh Patol. 1981;43(6):3-11.
The main changes occuring in the theory of thesaurismoses are presented briefly. Main groups of thesaurismoses characterized by disorders of one or another kind of metabolism are dealt with. Most frequently occuring forms of thesaurismoses are described and by their examples it is shown how new nosological entities are distinguished by the nature of metabolic disorders, by the defect of the enzyme, and by the degree of severity of the enzymatic block. The main criteria for morphological diagnosis of thesaurismoses are presented including the presence of accumulation cell, their localization in different organs, histochemical analysis of accumulation products and electron microscopic findings. Biochemical examinations are considered to be essential for diagnosis.
本文简要介绍了贮积病理论中发生的主要变化。探讨了以一种或另一种代谢紊乱为特征的贮积病主要类型。描述了最常见的贮积病形式,并通过实例展示了如何根据代谢紊乱的性质、酶的缺陷以及酶阻断的严重程度来区分新的病种。提出了贮积病形态学诊断的主要标准,包括蓄积细胞的存在、它们在不同器官中的定位、蓄积产物的组织化学分析以及电子显微镜检查结果。生化检查被认为对诊断至关重要。