Kutafin Iu F, Osipov S M, Osipov E S
Zh Nevropatol Psikhiatr Im S S Korsakova. 1991;91(3):15-8.
The paper is concerned with an analysis of the anamnestic clinical and histological data on 8 children (6 girls and 2 boys) who died at an age of 3 to 7 months. They had been treated at the Child's Clinical Hospital of Moscow for Hoffmann-Werdnig syndrome, bilateral polysegmental pneumonia, respiratory insufficiency, stage III, hypotrophy, stages II and III, anemia of infectious alimentary etiology. Ground for hospitalization was superaddition of intercurrent infection, the growth of respiratory insufficiency. In spite of intensive care, the children died on days I-III of the stay at the hospital. The clinical analysis of the disease and the autopsy findings allowed a conclusion about the predominance of the early form of Hoffmann-Werdnig syndrome running a rapid progressive course. The immediate cause of the children's death was acute respiratory insufficiency associated with polysegmental pneumonia, spinal amyotrophy.
本文涉及对8名儿童（6名女孩和2名男孩）的既往临床和组织学数据的分析，这些儿童在3至7个月龄时死亡。他们曾在莫斯科儿童医院接受治疗，患有霍夫曼 - 韦尔迪希综合征、双侧多节段性肺炎、呼吸功能不全（III期）、营养不良（II期和III期）、感染性营养性病因贫血。住院的原因是并发感染的叠加以及呼吸功能不全的加重。尽管进行了重症监护，但这些儿童在住院的第1 - 3天死亡。对疾病的临床分析和尸检结果得出结论，霍夫曼 - 韦尔迪希综合征的早期形式占主导，病程进展迅速。儿童死亡的直接原因是与多节段性肺炎、脊髓性肌萎缩相关的急性呼吸功能不全。
