Symptomatic selenium deficiency in a child on home parenteral nutrition.

A 27-month-old girl with microvillous atrophy who was maintained on long-term home parenteral nutrition presented with regression of walking skills. She was found to have a tender skeletal myopathy affecting both legs only. Plasma selenium level was less than 0.05 mumol/L (0.4 microgram/dl) (normal 0.8-1.6 mumol/L; 6.3-12.6 micrograms/dl), red blood cell glutathione peroxidase was undetectable, and plasma glutathione peroxidase was 6 U/L (normal 90-350 U/L). Electromyography was abnormal, muscle biopsy showed a nonspecific myopathy, and muscle selenium was 0.23 micrograms/g dry weight (normal 0.45-1.05 micrograms/g dry weight). Following replacement with intravenous sodium selenite, there was a rapid improvement with complete disappearance of muscle pain and tenderness within 1 week. Crawling and walking skills were regained within 6 weeks, indicating that selenium deficiency was the cause of her symptoms. It is suggested that selenium be monitored and supplemented in children on long-term total parenteral nutrition particularly if enteral absorption is deficient.