Lactic acidosis with necrotizing encephalopathy in two sibs.

作者：H. E. Worsley, Ronald Winston Brookfield, J. Stanley Elwood, ROBERT L. NOBLE, W.H. Taylor · 发表于：Archives of Disease in Childhood · 年份：1965 · DOI：10.1136/adc.40.213.492 · 被引用次数：83 · 研究领域：Infectious Encephalopathies and Encephalitis、Metabolism and Genetic Disorders、Infant Health and Development

The plasma lactate concentration is raised physiologically, during muscular exertion, and sometimes in diseases associated with demonstrable hypoxaemia, such as peripheral circulatory failure, severe shock, acute bronchial asthma, and obstructive emphysema.The plasma lactate level is also significantly raised in respiratory alkalosis arising from primary hyperventilation, and often in metabolic alkalosis.When there is alkalosis, there is usually no hypoxaemia, and the plasma concentration of pyruvate, the immediate precursor of lactate, is also proportionally raised.Gevers and Dowdle (1963) have shown that alkaline conditions stimulate the glycolytic rate in vitro of rat liver and kidney slices and that the effect is exerted at a stage or stages proximal to the dehydrogenation of triosephosphate.A distinction has thus been drawn (Huckabee, 1961) between the alkalotic and the hypoxaemic group, for in the latter 'excess' lactate, relative to pyruvate, is found.'Excess' lactate is defined as, (Ln-Lo)-(Pn-Po) (Lo/Po), where Ln and Pn are the actual values of lactate and pyruvate, respectively, and Lo and Po, the expected respective normal values.Recently, a syndrome of lactic acidosis has been described (Huckabee, 1961; Daughaday, Lipicky, and Rasinski, 1962) in patients who have 'excess' lactate but apparently no hypoxaemia.The patients show hyperpnoea, dyspnoea, and metabolic acidosis, and have a poor prognosis.They present with a variety of diseases including poliomyelitis, ga...