caa a22 4500 469045248 CHVBK 20180323132822.0 cr unu---uuuuu 170328e19920301xx s 000 0 eng 10.1007/BF01799640 doi (NATIONALLICENCE)springer-10.1007/BF01799640 Decreased selenium intake and low plasma selenium concentrations leading to clinical symptoms in a child with propionic acidaemia [Elektronische Daten] [S. Yannicelli, K. Hambidge, M. Picciano] Summary: A child with biotin-non-responsive propionic acidaemia treated with a propiogenic amino acid-restricted diet presented with an elevated blood mean corpuscular volume (MCV) of 93.1 fl, indicative of macrocytosis, and unusual hair texture with hypopigmentation. Plasma selenium concentration at this time was subnormal (45.9 µg/L), and calculated dietary selenium intake was 4.7 µg/day. After 4 months of selenium supplementation (50 µg/day) plasma selenium concentration normalized (97.7 µg/L) in conjunction with a reduced MCV (84.0 fl) and a dramatic improvement in hair growth, colour and length. Two additional periods off and on selenium supplementation, of varying time intervals, resulted in similar clinical changes. We conclude that these clinical changes were due to a deficient intake of dietary selenium. SSIEM and Kluwer Academic Publishers, 1992 Yannicelli S. Department of Pediatrics, Division of Genetics and Inherited Metabolic Diseases Clinic, The Children's Hospital, 1056 East 19th Avenue, Box B-153, Denver, Colorado, USA aut Hambidge K. Department of Pediatrics, The Children's Hospital, Denver, Colorado, USA aut Picciano M. Nutrition Department, Pennsylvania State University, University Park, Pennsylvania, USA aut Journal of Inherited Metabolic Disease Kluwer Academic Publishers 15/2(1992-03-01), 261-268 0141-8955 15:2<261 1992 15 10545 https://doi.org/10.1007/BF01799640 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/BF01799640 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Yannicelli S. Department of Pediatrics, Division of Genetics and Inherited Metabolic Diseases Clinic, The Children's Hospital, 1056 East 19th Avenue, Box B-153, Denver, Colorado, USA aut NATIONALLICENCE 700 1- Hambidge K. Department of Pediatrics, The Children's Hospital, Denver, Colorado, USA aut NATIONALLICENCE 700 1- Picciano M. Nutrition Department, Pennsylvania State University, University Park, Pennsylvania, USA aut NATIONALLICENCE 773 0- Journal of Inherited Metabolic Disease Kluwer Academic Publishers 15/2(1992-03-01), 261-268 0141-8955 15:2<261 1992 15 10545 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer