Hoffmann-Werdnig syndrome

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 Children'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 immmediate cause of the children's death was acute respiratory insufficiency associated with polysegmental pneumonia, spinal amyotrophy.