Abstract
A 2½ year old girl on long term HPN for microvillous atrophy presented with distal limb myalgia with normal power, reflexes & tone, & progressive inability to bear weight. Normal full blood count, electrolytes, creatine kinase and transaminases. Serum concentrations of Vitamins A, E & B12 folate zinc and copper were normal. Plasma selenium was very low at < 0.05 μmol/l (reference range (RR) 0.8-2.0 μmol/1); RBC glutathione peroxidase was undetectable at < 1μ/g Hb) and plasma glutathione peroxidase was 6μ/l (RR 90-35μ/l). RBC enzymes showed normal vitamins B1, B2, B6 status. Electromyogram was mildly abnormal. Muscle biopsy demonstrated atrophy of type II fibres & muscle selenium was 0.23μg/g dry weight (adult RR: 0.6-0.97 μg/g). Full cardiological evaluation was normal. Sodium selenite 15 μg/day was added to IV nutrition. Within 3 days muscle pain had improved; she began to crawl again & by 6 weeks she was walking normally. At 3 months, plasma selenium was 0.53 μmol/1. RBC glutathione peroxidase was 15 μ/g Hb. Sodium selenite supplements were reduced. Although biochemical evidence of selenium depletion is common in adults & children on HPN, associated clinical symptoms have rarely been reported. This is the youngest reported. Relationship between biochemical depletion & clinical symptoms is poorly understood, the dramatic response to selenium suggests that deficiency was the cause.
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Kelly, D., Shenkin, A., Coe, A. et al. 85. Symptomatic Selenium Deficiency in a child on home parenteral nutrition (HPN). Pediatr Res 22, 110 (1987). https://doi.org/10.1203/00006450-198707000-00106
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DOI: https://doi.org/10.1203/00006450-198707000-00106