Knowledge, compliance and serum phenylalanine concentrations in adolescents and adults with phenylketonuria and the effect of a patient-focused educational resource

Journal of Human Nutrition and Dietetics

Volumn 21, Issue 5, 2008, Pages 474-485

  Durham Shearer, S J ^a   Judd, P A ^b   Whelan, K ^c   Thomas, J E ^c  

^a ST GEORGE'S UNIVERSITY HOSPITALS NHS FOUNDATION TRUST   (United Kingdom)

^b UNIVERSITY OF CENTRAL LANCASHIRE   (United Kingdom)

^c KING'S COLLEGE LONDON   (United Kingdom)

Author keywords

Dietary compliance; Education resource; Phenylketonuria

Indexed keywords

PHENYLALANINE;

ADOLESCENT; ADULT; AMINO ACID BLOOD LEVEL; ARTICLE; CLINICAL ARTICLE; CLINICAL TRIAL; CONTROLLED CLINICAL TRIAL; CONTROLLED STUDY; DIETARY INTAKE; DIETITIAN; FEEDING BEHAVIOR; FEMALE; HUMAN; KNOWLEDGE; MALE; NUTRITION EDUCATION; NUTRITIONAL REQUIREMENT; PATIENT ATTITUDE; PATIENT CARE; PHENYLKETONURIA; QUESTIONNAIRE; RANDOMIZED CONTROLLED TRIAL;

ADOLESCENT; ADULT; DIETARY PROTEINS; FEMALE; HEALTH KNOWLEDGE, ATTITUDES, PRACTICE; HUMANS; MALE; PATIENT COMPLIANCE; PATIENT EDUCATION AS TOPIC; PHENYLALANINE; PHENYLKETONURIAS; PREGNANCY; QUESTIONNAIRES;

EID: 51649115844     PISSN: 09523871     EISSN: 1365277X     Source Type: Journal    
DOI: 10.1111/j.1365-277X.2008.00879.x     Document Type: Article

References (38)

1. Azen, C.G., Koch, R., Friedman, E., Wenz, E. & Fishler, K. (1996) Summary of findings from the United States collaborative study of children treated for phenylketonuria. Eur. J. Pediatr. 155 (Suppl. 1), 29-32.
2. Behbehani, A.W., Vollrath, M., Matschke, I. & Langenbeck, U. (1986) Termination of strict diet in PKU: Neuropsychological, psychological and biochemical studies. J. Inherit. Metab. Dis. 9 (Suppl. 2), 218-222.
3. Bekhof, J., van Spronsen, F.J., Crone, M.R., van Rijn, M., Oudshoorn, C.G.M. & Verkerk, P.H. (2003) Influence of knowledge of the disease on metabolic control in phenylketonuria. Eur. J. Pediatr. 162, 440-442.
4. Bick, U., Ulirch, K., Stöber, U., Moller, U., Schuierer, G., Ludolph, A.C., Oberwittler, C., Weglage, J. & Wendel, U. (1993) White matter abnormalities in patients with treated hyperphenylalaninaemia: Magnetic resonance relaxometery and protein spectroscopy findings. Eur. J. Pediatr. 152, 1012-1020.
5. Bickel, H., Gerrand, J. & Hickmans, E.M. (1954) The influence of phenylalanine intake on the chemistry and behaviour of a phenylketonuric child. Acta Paediatr. 43, 64-77.
6. Birmingham, B., Shultz, J.A. & Edlefsen, M. (2004) Evaluation of a five-a-day recipe booklet for enhancing the use of fruit and vegetables in low-income households. J. Comm. Health. 29, 45-62.
7. British Inherited Metabolic Disease Group. (2004) Inherited Metabolic Disorders. A Service Vision for Setting Standards of Care and their Provision. Available at http://bimdg.org.uk/BIMDG_vision.asp (accessed on 10 September 2007).
8. Burton, H., Sanderson, S., Dixon, M., Hallam, P. & White, F. (2007) Review of specialist dietitian services in patients with inherited metabolic disease in the United Kingdom. J. Hum. Nutr. Diet. 20, 84-92.
9. Cameron, C. (1996) Patient compliance: Recognition of factors involved and suggestion for promoting compliance with therapeutic regimens. J. Adv. Nurs. 24, 244-250.
10. Cleary, M.A., Walter, J.H., Wraith, J.E. & Jenkins, J.P.R. (1995) Magnetic resonance imaging in phenylketonuria: Reversal of cerebral white matter change. J. Pediatr. 127, 251-255.
11. Contento, I. (1995) The effectiveness of nutrition education and implications for nutrition education policy programs and research: A review of research. J. Nutr. Educ. 27, 279-283.
12. Dyer, K., Fearon, K., Buckner, K. & Richardson, R.A. (2004) Diet and colorectal cancer risk. Impact of a nutrition education leaflet. J. Hum. Nutr. Diet. 17, -576.
13. Gassió, R., Campistol, J., Vilaseca, M.A., Lambrushchini, N., Cambra, F.J. & Fusté, E. (2003) Do adult patients with phenylketonuria improve their quality of life after introduction/ resumption of a phenylalanine-restricted diet? Acta Paediatr. 92, 1474-1478.
14. Gleason, L.A., Michals, K., Matalon, R., Langenberg, P. & Kamath, S. (1992) A treatment program for adolescents with phenylketonuria. Clin. Pediatr. 31, 331-335.
15. Hanley, W.B., Feigenbaum, A.S.J., Clarke, J.T.R., Schoonheyt, W.E. & Austin, V.J. (1996) Vitamin B12 deficiency in adolescents and young adults with phenylketonuria. Eur. J. Pediatr. 155 (Suppl. 1), 145-147.
16. Jackson, J.A., Kinn, S. & Dalgarno, P. (2005) Patient-centred outcomes in dietary research. J. Hum. Nutr. Dietet. 18, 83-92.
17. Koch, R., Burton, B., Hoganson, G., Peterson, R., Rhead, W., Rouse, B., Scott, R., Wolff, J., Stern, A.M., Guttler, F., Nelson, M., de al Cruz, F., Coldwell, J., Erbe, R., Geraghty, M.T., Shear, C., Thomas, J. & Azen, C. (2002) Phenylketonuria in adulthood: A collaborative study. J. Inherit. Metab. Dis. 25, 333-346.
18. Lake, A.A., Speed, C., Brookes, A., Heaven, B., Adamson, A.J., Moynihan, P., Corbett, S. & McColl, E. (2007) Development of a series of patient information leaflets for constipation using a range of cognitive interview techniques: LIFELAX. BMC Health Serv. Res. 7, 3.
19. Levy, H.L. & Waisbren, S.E. (1994) PKU in adolescents: Rationale and psychosocial factors in diet continuation. Acta Paediatr. 407 (Suppl.), 92-97.
20. MacDonald, A. (2000) Diet and compliance in phenylketonuria. Eur. J. Pediatr. 159 (Suppl. 2), 136-141.
21. MacDonald, A., Manji, N., Evans, S., Davies, P., Daly, A., Hendriksz, C. & Chakrapani, A. (2006) Home delivery of dietary products in inherited metabolic disorders reduces prescription and dispensing errors. J. Hum. Nutr. Diet. 19, 375-381.
22. Medical Research Council Working Party on Phenylketonuria. (1993) Recommendations on the dietary management of phenylketonuria. Arch. Dis. Child. 68, 426-427.
23. Perkins, L. (2000) Developing a tool for health professionals involved in producing and evaluation nutrition education leaflets. J. Hum. Nutr. Diet. 13, 41-49.
24. Poustie, V.J. & Rutherford, P. (2001) Dietary interventions for phenylketonuria. Cochrane Database Syst. Rev. 2, CD001304.
25. Robinson, M., White, F.J., Cleary, M.A., Wraith, E., Lam, W.K. & Walter, J.H. (2000) Increased risk of vitamin B12 deficiency in patients with phenylketonuria on an unrestricted or relaxed diet. J. Pediatr. 136, 545-547.
26. Roter, D.L., Hall, J.A., Rolande, M., Nordstrom, B., Cretin, D. & Svarstad, B. (1998) Effectiveness of interventions to improve patient compliance: A meta-analysis. Med. Care 36, 1138-1161.
27. Schulz, B. & Bremer, H.J. (1995) Nutrient intake and food consumption of adolescents and young adults with phenylketonuria. Acta Paediatr. 84, 743-748.
28. Shiloh, S., St James, P. & Waisbren, S. (1990) The development of a patient knowledge test on maternal phenylketonuria. Patient Educ. Couns. 16, 139-146.
29. Singh, R.H., Kable, J.A., Guerrero, N.V., Sullivan, K.M. & Elsas, L.J. (2000) Impact of a camp experience on phenylalanine levels, knowledge, attitudes, and health beliefs relevant to nutrition management of phenylketonuria in adolescent girls. J. Am. Diet. Assoc. 100, 797-803.
30. Smith, I., Lobascher, M.E., Stevenson, J.E., Wolff, O.H., Schmidt, H., Grubel-Kaiser, S. & Bickel, H. (1978) Effect of stopping low phenylalanine diet on intellectual progress of children with PKU. Brit. Med. J. 2, 723-726.
31. Smith, I., Beasley, M.G. & Ades, A.E. (1990) Effect on intelligence of relaxing the low phenylalanine diet in phenylketonuria. Arch. Dis. Child. 65, 311-316.
32. Solomons, G., Keleske, L. & Opitz, E. (1996) Evaluation of the effects of terminating the diet in phenylketonuria. J. Pediatr. 69, 596-602.
33. Thompson, A.J., Smith, I., Brenton, D., Youl, B.D., Rylance, G., Davidson, D.C., Kendall, B. & Lees, A.J. (1990) Neurological deterioration in young adults with phenylketonuria. Lancet 336, 602-605.
34. Ullrich, K., Möller, H., Weglage, J., Schuierer, G., Bick, U., Ludolph, A., Hahn-Ullrich, H., Fünders, B. & Koch, H.G. (1994) White matter abnormalities in phenylketonuria: Results of magnetic resonance measurements. Acta Paediatr. 407 (Suppl.), 78-82.
35. Villasana, D., Butler, I.J., Williams, J.C. & Roongta, S.M. (1989) Neurological deterioration in adult phenylketonuria. J. Inherit. Metab. Dis. 12, 451-457.
36. Walter, J.H. & White, F.J. (2004) Blood phenylalanine control in adolescents with phenylketonuria. Int. J. Adolesc. Med. Health 16, 41-45.
37. Walter, J.H., White, F., Wraith, J.E., Jenkins, J.P. & Wilson, B.P.M. (1997) Complete reversal of moderate/severe brain MRI abnormalities in a patient with classical phenylketonuria. J. Inherit. Metab. Dis. 20, 367-369.
38. Weglage, J., Funders, B., Ullrick, K., Rupp, A. & Scmidt, E. (1996) Psychosocial aspects in phenylketonuria. Eur. J. Pediatr. 151, 522-525.