Abstract
A healthy 5 mo. old male had six apneic spells with cyanosis and loss of tone, lasting up to 60 seconds, within two days. Although monitored and free of further apneic episodes, hyperlactatemia (46 mg%) was present two and four days later, pyruvate 2.2 mg%, arterial and CSF lactate 43 and 20 mg%; uric acid 11 mg%, plasma alanine increased, urine screen by GC for organic acids normal. Multiple FBS determinations, glucose tolerance and alanine loads were normal and not associated with lactate elevation. Conversion of I-14C pyruvate to 14CO2, measured in WBC and cultured fibroblasts was normal. BMR was normal. On anticonvulaants and thiamine there were 7 more episodes in 6 weeks. Multiple EEC's were normal except for one episode of seizure discharges following 10 second apnea. Spontaneous panting episodes occurred prior to and throughout observation. Following a ketogenic diet with medium chain triglycerides, serum lactate declined gradually to 8.5 mg% and lactate pyruvate ratio normalized: 11. The patient who excretes large amounts of medium chain dlcarboxylic acids, has been asymptomatic for 5 months. A causal relationship with the apparent recovery is possible. The above tests do not suggest defective gluconeogenesis; defective pyruvate dehydrogenase complex in brain is unlikely in view of normal pyruvate metabolism in WBC and fibroblasts, and lower lactate levels in CSF than blood. Lactate, which may be elevated due to dysregulation of respiratory drive, should be monitored in infants at risk for SIDS.
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Nigro, M., Krieger, I. “NEAR-MISS” SUDDEN INFANT DEATH SYNDROME (SIDS) WITH I-ACTIC ACIDOSIS. Pediatr Res 11, 538 (1977). https://doi.org/10.1203/00006450-197704000-01012
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DOI: https://doi.org/10.1203/00006450-197704000-01012