Abstract
A retrospective study of 53 patients with phenylketonuria (PKU), whose disease was managed with a low-phenylalanine diet, revealed a high incidence of iron depletion (as reflected by subnormal serum ferritin concentrations). Serum ferritin concentrations under 10 μg/l were found in one out of six infants aged 5–12 months. Concentrations under 16 μg/l were found in 16 of 22 children aged 1–3 years and in 11 of 25 children aged 4–12 years. Dietary iron, estimated from prescribed intakes of medical foods, exceeded the Canadian recommended nutrient intake, suggesting that low stores of iron may be secondary to reduced bioavailability and absorption of iron. These findings suggest that the current dietary management of PKU is associated with an increased risk for low iron stores. Investigators have reported an association in young children between iron-deficiency anaemia and both cognitive and motor disturbances. Children with PKU, already at risk of neurological damage because of phenylalanine neurotoxicity, may be at increased risk as a result of iron depletion. Serum ferritin as well as haemoglobin concentration should be monitored, along with plasma phenylalanine and tyrosine, to ensure optimum treatment of affected children.
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Abbreviations
- MCV:
-
mean corpuscular volume
- PKU:
-
phenylketonuria
References
Böhles H, Ullrich K Endres W, Behbehani AW, Wendel U (1991) Inadequate iron availability as a possible cause of low serum carnitine concentrations in patients with phenylketonuria. Eur J Pediatr 150:425–428
Brise H, Hallberg L (1962) Effect of ascorbic acid on iron absorption. Acta Med Scand 171 [Suppl 376]:51–58
Chandra RK (1988) Immune responses and nutritional status. Mod Med Can 43:906–912
Dallman PR (1987) Iron deficiency and the immune response. Am J Clin Nutr 46:329–334
Entman SS, Richardson LD, Killam AP (1982) Elevated serum ferritin in the altered ferrokinetics of toxemia of pregnancy. Am J Obstet Gynecol 144:418–422
Fairweather-Tait SJ (1989) Iron in food and its availability. Acta Paediatr Scand [Suppl] 361:12–20
Fomon SJ, Ziegler EE, Rogers RR, Nelson SE, Edwards BB, Guy DG, Erve JC, Janghorbani M (1989) Iron absorption from infant foods. Pediatr Res 26:250–254
Gropper SS, Acosta PB, Clarke-Sheehan N, Wenz E, Cheng M, Koch R (1988) Trace element status of children with PKU and normal children. J Am Diet Assoc 88:459–465
Holtzman NA, Kronmal RA, Doorninck W van, Azen C, Koch R (1986) Effect of age at loss of dietary control on intellectual performance and behavior of children with phenylketonuria. N Engl J Med 314:593–598
Koch R, Azen CG, Hurst N, Friedman EG, Fishler K (1987) The effects of diet discontinuation in children with phenylketonuria. Eur J Pediatr 146, [Suppl 1]:A12-A16
Lozoff B (1988) Behavioral alterations in iron deficieny. Adv Pediatr 35:331–360
Lozoff B, Brittenham GM, Wolf AW, McClish DK, Kuhnert PM, Jimenez E, Jimenez R, Mora LA, Gomez I, Krauskoph D (1987) Iron deficiency anemia and iron therapy effects on infant developmental test performance. Pediatrics 79:981–995
MacCready RA (1974) Admissions of phenylketonuric patients to residential institutions before and after screening programs of the newborn infant. J Pediatr 85:383–385
McCabe ERB, McCabe L (1986) Issues in the dietary management of phenylketonuria: breast-feeding and trace-metal nutriture. Ann NY Acad Sci 477:215–222
Michals K, Azen C, Acosta P, Koch R, Matalon R (1988) Blood phenylalanine levels and intelligence of 10-year-old children with PKU in the National Collaborative Study. J Am Diet Assoc 88:1226–1229
Oski FA, Honig AS, Helu B, Howanitz P (1983) Effect of iron therapy on behavior performance in nonanemic, iron-deficient infants. Pediatrics 71:877–880
Reilly C, Barrett JE, Patterson CM, Tinggi U, Latham SL, Marrinan A (1990) Trace element nutrition status and dietary intake in children with phenylketonuria. Am J Clin Nutr 52: 159–165
Saudubray JM, Rey F, Ogier H, Abadie V, Farriaux JP, Ghisolfi J, Guibaud P, Rey J, Vidailhet M (1987) Intellectual and school performances in early-treated classical PKU patients. The French Collaborative Study. Eur J Pediatr 146 [Suppl 1]: A20-A22
Scaglioni S, Zuccotti G, Vedovello M, Rottoli A, Paccanelli S, Longhi R, Riva E, Giovanni M (1985) Study of serum ferritin in 58 children with classic phenylketonuria and persistent hyperphenylalaninaemia. J Inherited Metab Dis 8:160
Smith I, Beasely M (1989) Intelligence and behaviour in children with early treated phenylketonuria. A report from the MRC/DHSS phenylketonuria register. Eur J Clin Nutr 43 [Suppl 1]:1–5
Smith I, Beasley MG, Wolff OH, Ades AE (1988) Behavior disturbance in 8-year-old children with early treated phenylketonuria. Report from the MRC/DHSS phenylketonuria register. J Pediatr 112:403–408
Smith I, Beasley MG, Ades AE (1990) Intelligence and quality of dietary treatment in phenylketonuria. Arch Dis Child 65: 472–478
SI Steering Committee (1990) Reference values and SI unit information. The Hospital for Sick Children, Toronto (Form # 35112)
The Scientific Review Committee, Health and Welfare Canada (1990) Summary of recommended nutrient intakes. In: Nutrition recommendations. Canadian Government Publishing Centre, Ottawa, pp 204
Veale AMO (1980) Screening for phenylketonuria. In: Bickel H, Guthrie R, Hammersen G (eds) Neonatal screening for inborn errors of metabolism. Springer, Berlin Heidelberg New York, pp 7–18
Youdim MBH (1985) Brain iron metabolism: biochemical and behavioral aspects in relation to dopaminergic neurotransmission. In: Lajtha A (ed) Handbook of neurochemistry, vol 10, 2nd edn. Plenum Press, New York, pp 731–755
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Bodley, J.L., Austin, V.J., Hanley, W.B. et al. Low iron stores in infants and children with treated phenylketonuria: A population at risk for iron-deficiency anaemia and associated cognitive deficits. Eur J Pediatr 152, 140–143 (1993). https://doi.org/10.1007/BF02072491
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DOI: https://doi.org/10.1007/BF02072491