CLINICAL NUTRITION. Body mass index in adult patients with diet-treated phenylketonuria

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1 CLINICAL NUTRITION Body mass index in adult patients with diet-treated phenylketonuria L. V. Robertson,* N. McStravick, S. Ripley, E. Weetch, S. Donald, S. Adam,** A. Micciche, S. Boocock,* & A. MacDonald *University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK The Royal Hospitals Metabolic Clinics, The Royal Victoria, The Royal Hospitals, BHSCT, Belfast, UK Salford Royal NHS Foundation Trust, Salford, UK Northern General Hospital, Sheffield, UK Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK **Royal Hospital for Sick Children, Glasgow, UK Guy s and St Thomas NHS Foundation Trust, London, UK Birmingham Children s Hospital NHS Foundation Trust, Birmingham, UK Journal of Human Nutrition and Dietetics Keywords adult, body mass index, obesity, phenylalanine, phenylketonuria. Correspondence L. V. Robertson, University Hospitals Birmingham NHS Foundation Trust, IMD Office, Room 27, Ground Floor, North block, Old Queen Elizabeth Hospital, Birmingham B15 2TH, UK. Tel.: +44 () Fax: +44 () louise.robertson@uhb.nhs.uk How to cite this article Robertson L.V., McStravick N., Ripley S., Weetch E., Donald S., Adam S., Micciche A., Boocock S. & MacDonald A. (213) Body mass index in adult patients with diet-treated phenylketonuria. J Hum Nutr Diet. 26 (Suppl. 1), 1 6 doi:1.1111/jhn.1254 Abstract Background: There is an increasing number of adults with phenylketonuria (PKU) on a low phenylalanine diet. In the general population, an increasing body mass index (BMI) in the UK is a major problem with associated co-morbidities. The present study aimed to identify whether patients with diet-treated PKU have obesity rates comparable to those in the general population. Methods: Two hundred and thirty-six PKU subjects (49% male, 51% female), aged >16 years, who were diagnosed by newborn screening and were receiving a low phenylalanine diet, were identified from seven metabolic centres in the UK. Retrospective data were collated on age, sex, BMI and mean phenylalanine concentration over the previous 12 months. Results: Mean (SD) phenylalanine concentration for all 236 subjects was 789 (311) lm; mean (SD) BMI was 26 (5.4) kg m 2 [males 25 (4.3) kg m 2, females 27 (6.2) kg m 2 ]; mean (SD) age was 26 (7) years; and 55% had a BMI > 25 kg m 2. The percentage of subjects with a BMI >25 kg m 2 and >3 kg m 2, as well as increasing obesity with age, was similar to the UK population. A correlation was observed between increasing BMI and a higher phenylalanine concentration (r =.243, P =.1). Conclusions: The number of overweight and obese patients with diet-treated PKU in the UK is a concern. This could lead to other obesity-related complications increasing the complexity of diet and the cost of their care. There is a need to educate patients with respect to adopting a healthy, low phenylalanine diet and lifestyle to prevent further rises in BMI. Introduction Phenylketonuria (PKU; OMIM 2616) is an inborn error of metabolism caused by a deficiency of the enzyme phenylalanine hydroxylase, which converts the amino acid phenylalanine into tyrosine. High levels of phenylalanine are neurotoxic to the brain, which, if left untreated, may cause irreversible brain damage (Blau et al., 21). National screening for PKU in the UK was introduced in Before this time, most affected children sustained irreversible brain damage before diagnosis (The National Society for Phenylketonuria, 24). Patients treated with diet from birth post-screening are now reaching their early 4s, well into adulthood. As a result of growing evidence, the medical research council s working party on PKU (Medical Research Council Working Party on Journal of Human Nutrition and Dietetics ª 213 The British Dietetic Association Ltd. 1

2 BMI in adult patients with diet-treated PKU L. V. Robertson et al. Phenylketonuria, 1993) recommend that adults with PKU should stay on the low phenylalanine diet for life. Treatment consists of a low phenylalanine diet restricting natural phenylalanine intake and requires supplementation with special medical foods that supply sufficient amounts of amino acids and energy (Harding & Blau, 21). Obesity is a major public health problem in England. In 28, 24% of males and 25% of females were classed as obese [body mass index (BMI >3 kg m 2 )] (The Health & Social Care Information Centre, 21). It is estimated that, by 25, 6% of men and 5% of women could be clinically obese and, without action, this could cost society 49.9 billion per year (Butland et al., 27). Obesity is associated with serious chronic conditions, such as type 2 diabetes, hypertension and hyperlipidaemia. Adults who are overweight or obese are more likely to have high blood pressure than those of a healthy weight (The Health and Social Care Information Centre, 21). There is little information available in the literature regarding adults with PKU and their BMI or obesity, and little is known about the long-term health consequences of adopting a low phenylalanine diet. If adult PKU patients follow the same patterns of obesity as the general population, there is a risk that they may develop the same complications. Dietary manipulation forms the basis of treatment for obesity, diabetes, hypertension and hyperlipidaemia. Combining these dietary changes with a low phenylalanine diet could make the diet even more complex for people with PKU. The present study aimed to determine whether BMI trends in adult PKU patients were comparable to non- PKU peers. In addition, the study aimed to determine whether a relationship exists between phenylalanine control and BMI. Materials and methods Subjects Two hundred and thirty-six patients were audited from seven adult PKU treatment centres in the UK. Inclusion criteria included patients with PKU who were treated from birth post-screening (1969), were currently on a low phenylalanine diet under the care of a metabolic dietitian, and who were aged 16 years of age. A low phenylalanine diet was defined as occurring in those patients taking their full dose of protein substitute daily; however, we do not have data on their compliance. Exclusion criteria included patients on a preconception diet or who were pregnant, patients with learning difficulties, and patients who did not have full available data on weight, BMI and phenylalanine concentrations for the last 12 months. Study design The study was a multi-centre retrospective audit. An audit template was sent out to each of the centres and information was gathered on the age, sex, BMI (kg m 2 ) and mean phenylalanine concentration (lm) over the last 12 months for each subject. The information was obtained from the subjects medical and dietetic records. Statistical analysis Data were analysed using PASW STATISTICS, version 18 (SPSS Inc, Chicago, IL, USA). Unpaired t-tests were used to compare continuous variables (phenylalanine concentration, age and BMI) for males and females. One-way analysis of variance was used for the comparison of phenylalanine concentrations in three age groups. The proportions of males and females who were overweight or obese were compared using Fisher s exact test and the 95% confidence intervals for these proportions were calculated for comparison with known population values. Kendall s tau-b was used to assess association between the two ordinal variables, BMI category and age category. Pearson correlation was used to assess the association between phenylalanine concentration and BMI. Results Subjects Table 1 shows the results of the seven individual PKU centres with respect to the number of subjects, males and females, their mean age, BMI and phenylalanine concentrations. Approximately 236 PKU adults who were aged >16 years, born after newborn screening (1969), and who were currently receiving a low phenylalanine diet (including phenylalanine free protein substitutes) were identified. Forty-nine percent were male and 51% were female. The mean (SD) phenylalanine concentration of the cohort was 789 (311) lm. Males had a significantly higher mean phenylalanine concentration compared to females: 832 (296) lm versus 747 (32) lm, respectively (P =.36). The mean phenylalanine concentration from each centre ranged from 648 to 13 lm. The mean (SD) age was 26 (7) (median 25, range 16 41) years with no significant difference between males [26 (6.7) years] and females [27 (7.3) years] (P =.244) and the mean (SD) BMI was 26 (5.4) kg m 2.Nosignificant difference was seen in BMI between males [25 (4.3) kg m 2 ] and females [27 (6.2) kg m 2 ](P =.54). The subjects were split into age ranges: 16 24, and years. These ranges were chosen because they correspond to ranges used by the English, Scottish and Northern Irish health studies. Forty-one years was the upper limit because subjects aged older than this would 2 Journal of Human Nutrition and Dietetics ª 213 The British Dietetic Association Ltd.

3 L. V. Robertson et al. BMI in adult patients with diet-treated PKU Table 1 Individual centres and total results for subject numbers, males and females, age, phenylalanine concentrations and body mass index Centre All centres Subjects, n Males, n (%) 5 (51) 15 (39) 1 (56) 2 (67) 8 (67) 17 (44) 13 (48) 115 (49) Females, n (%) 49 (49) 23 (61) 8 (44) 1 (33) 4 (33) 22 (56) 14 (52) 121 (51) Age (years), mean (SD) 27 (6.5) 27 (7.3) 26 (9.1) 25 (4.6) 26 (5.9) 25 (6.8) 24 (7.4) 26 (7) Phenylalanine concentration (lm), mean (SD) 89 (349) 651 (265) 13 (48) 648 (268) 942 (36) 754 (236) 743 (282) 789 (311) Body mass index (kg m 2 ), mean (SD) 26 (5.1) 26 (5.5) 25 (4.1) 23 (7.8) 28 (5.2) 26 (4.4) 24 (5.8) 26 (5.4) have been born before newborn screening at the time of analysis. The percentage of subjects within each range decreased as the age ranges increased (16 24 years, 45%; years, 4%; and years, 14%). When sex is considered, there was a higher percentage of males than females in the years age range (51% and 4% respectively), although more females than males in the years and years age ranges (43% and 37% and 17% and 11%, respectively). % of PKU adults Males Females Mean phenylalanine concentration and age range There was no statistically significant difference in mean (SD) phenylalanine concentrations between each of the three age ranges [16 24 years, 772 (288) lm; years, 82 (343) lm; years, 815 (293) lm] for all subjects (analysis of variance: P =.641). This was the same when looked at separately for males [16 24 years, 79 (277) lm; years, 878 (334) lm; years, 869 (231) lm] and females [16 24 years, 749 (32) lm; years, 74 (34) lm; years, 781 (326) lm] (P =.32 and P =.869, respectively). Body mass index Forty-one percent of PKU subjects fell into the healthy weight range ( kg m 2 ), 31% fell into the overweight range ( kg m 2 ) and 24% fell into the obese range (>3 kg m 2 ). There was no significant difference between the amount of males with a BMI >25 kg m 2 (56%) compared to females with a BMI >25 kg m 2 (56%) (Fisher s exact test P =.896), although there was significantly more females with a BMI >3 kg m 2 (34%) compared to males (17%) (Fisher s exact test P =.14) (Fig. 1). Body mass index of phenylketonuria adults compared to the healthy populations A 95% confidence interval was calculated for the PKU subjects as a result of the small sample size compared to the large population sizes studies in England (The Health and Social Care Information Centre, 21), Northern < > 3 Ireland (Northern Ireland Health and Social Wellbeing Survey, 25/26) and Scotland (The Scottish Health Survey, 28). The true value that would be estimated for a larger PKU population would fall between these intervals (Table 2). The percentages for overweight and obese UK populations for males and females all fell within or on the limits of the PKU confidence intervals; therefore, no statistical differences can be demonstrated between the PKU population and the UK healthy population (Fig. 2). Body mass index, age and sex BMI ( ) Figure 1 Body mass index (BMI) of male and female phenylketonuria (PKU) subjects. The percentage of PKU subjects with a BMI >25 kg m 2 increased with age (tau-b =.233, P.1). This was also seen within the male and female category (.224, P =.14 and.246, P =.5) (Figs 3 and 4). The percentage of PKU subjects with a BMI of >3 kg m 2 also increased with age in all subjects (.158, P =.11), although it was not statistically significant when looking at males and females alone (.124, P =.191 and.154, P =.76). Mean phenylalanine concentrations compared to body mass index There was a correlation between BMI and mean phenylalanine concentration. As the BMI increased, the mean Journal of Human Nutrition and Dietetics ª 213 The British Dietetic Association Ltd. 3

4 BMI in adult patients with diet-treated PKU L. V. Robertson et al. Table 2 Body mass index of phenylketonuria (PKU) subjects compared to English, Northern Irish and Scottish populations PKU (%) England (%) Northern Ireland (%) Scotland (%) Male (>25 kg m 2 ) 56 (95% confidence interval = 46 65%) Female (>25 kg m 2 ) 55 (95% confidence interval = 45 64%) Male (>3 kg m 2 ) 17 (95% confidence interval = 1 25%) Female (>3 kg m 2 ) 31 (95% confidence interval = 23 4%) % of PKU pts and populations phenylalanine concentration also increased (r =.243, P <.1) (Fig. 5). This was seen both in males and females (.193, P =.38 and.313, P <.1) (Fig. 6). Discussion Male (> 25) Female (> 25) Male (> 3) Female (> 3) Gender/BMI ( ) The results obtained in the present study show that our UK PKU patients have the same levels of overweight and obesity as the general UK population. BMI increases with age (which is also similar to the UK population) and female patients were found to be significantly more obese PKU England Northern Ireland Scotland Figure 2 Body mass index (BMI) of phenylketonuria (PKU) subjects compared to English, Northern Irish and Scottish healthy populations. % of PKU adults > < year 25 34year 35 41year Age Figure 3 Body mass index of male phenylketonuria (PKU) subjects split into age ranges. % of PKU adults > < year 25 34year 35 41year Age Figure 4 Body mass index of female phenylketonuria (PKU) subjects split into age ranges. Phe conc µm Mean phe < > 3 BMI ( ) Figure 5 Mean phenylalanine (phe) concentrations compared to body mass index (BMI). than males. The individual centre results are similar to those of the other centres in our study group. This is the first collaborative study investigating obesity in PKU adults from a number of UK centres that treat PKU. A recent audit at one large adult centre in the UK (94 PKU patients on a protein restricted diet) reported similar results to the present study, with 58% of males and 51% of females being overweight (Mobayed et al., 211). In Israel, 31 adults with PKU were found to have a mean BMI of 24.3 kg m 2 (mean age 24 years, phenylalanine concentration 823 lm) (Modan-Moses et al., 27). This was lower than that reported in the present study 4 Journal of Human Nutrition and Dietetics ª 213 The British Dietetic Association Ltd.

5 L. V. Robertson et al. BMI in adult patients with diet-treated PKU Phe conc µm Males Females < > 3 BMI ( ) Figure 6 Mean phenylalanine (phe) concentrations compared to body mass index (BMI) for males and females. (mean BMI 26 kg m 2 ), although only 17 of the adults were compliant with diet. Other studies have reported levels of obesity similar to their matched controls (Paci et al., 29; Scagliono et al. 24, Huemer et al., 27). By contrast, a study in Spain found that PKU females aged >13 years and PKU males aged >18 years had significantly higher BMIs compared to the reference population (Belanger-Quintana & Martinez- Pardo, 211). In theory, a low phenylalanine diet is healthy because patients are encouraged to eat plenty of fruit and vegetables and avoid saturated animal fat, although the present study shows that 51% of patients with PKU were overweight. The data show that PKU patients become more overweight with age. There are no longitudinal data available to indicate at what point they are likely to become overweight and whether this is a result of patients consuming excess calories or not exercising sufficiently. Over time, the calorie content of their diet may increase as a result of several factors. As patients become older, they may relax their diet after having moved away from home or started college or work. They may be under more peer pressure from people around them to fit in and eat normal foods. They may eat less low protein prescription foods and look for alternatives. They often choose convenience foods that are higher in fat and protein, such as crisps, chips, chocolate and biscuits, because these are readily available. These foods would increase the calorie and protein content of the diet and this could explain why there is a direct correlation between increasing BMI and increasing phenylalanine concentrations. The mean phenylalanine concentration of our group was 789 lm, which is slightly above the target range of 7 lm for adults with PKU in the UK (Medical Research Council Working Party on Phenylketonuria, 1993). High phenylalanine concentrations can affect organisational skills and executive functioning (VanZutphen et al., 27; Christ et al., 21; Gentile et al., 21). If phenylalanine levels are high, the PKU adults may not be able to organise themselves to plan healthy meals, buy healthy foods and order low protein foods on prescription. They may therefore buy and eat more readily available foods that are higher in fat and calories, increasing the energy content of their diet. High phenylalanine concentrations are also associated with a negative effect on mood (Anjema et al., 211; ten Hoedt et al., 211), which could negatively affect the patient s food choices. Low protein foods on prescription (e.g. bread, pasta, rice, biscuits, energy replacement bars) could be blamed as the cause of obesity when an emphasis is placed on encouraging these foods to achieve target phenylalanine concentrations. If eaten in large quantities, they could increase the energy content of the diet, which could lead to weight gain in adults. This may not always be the case because, if these foods were used in large amounts instead of regular protein-containing foods, we would expect the mean phenylalanine concentration of patients to be lower. Decreased activity levels could be another reason for the increased levels of obesity in PKU patients. A lack of organisational skills (Gentile et al., 21) could prevent people with PKU from organising themselves to take exercise or go to the gym. There is little data available in the literature about how much exercise people with PKU undertake. Increasing obesity rates in female PKU subjects may partly be a result of the need to follow a strict diet before conception and throughout pregnancy. Weight loss in preconception and the early part of pregnancy is associated with a loss of phenylalanine control. To help prevent this, increased amounts of low protein foods and energy supplements may be prescribed to prevent weight loss (Maillot et al., 27). A female patient with PKU could be on a preconception diet for several months before conceiving, and then continue a hyper calorie diet postnatally leading to weight gain. The limitations of the present study are that information was not collected with respect to adherence to diet, eating and exercise patterns. Phenylalanine concentrations could imply metabolic control, although this does not mean that the patients were compliant with diet. Milder PKU patients may have lower phenylalanine concentrations and be on a more relaxed diet than a patient with severe PKU on a strict diet. To obtain a more accurate understanding of why patients were becoming overweight, data should be collected regarding adherence, eating patterns and exercise patterns. Further studies are needed to investigate patient food choice, exercise patterns and changes to diet post-pregnancy. Journal of Human Nutrition and Dietetics ª 213 The British Dietetic Association Ltd. 5

6 BMI in adult patients with diet-treated PKU L. V. Robertson et al. Conclusions The trend of becoming overweight and obese for the PKU population on a low phenylalanine diet is very similar to the healthy UK population. An increased BMI and the development of associated co-morbidities will make dietary management with this metabolic population more complex. The present study has highlighted the need for early intervention and support aiming to ensure that the BMI in PKU patients does not increase as it is predicted for the UK population. Ongoing research into this growing patient group is required to ensure optimal care. Acknowledgments We thank Peter Nightingale, Statistician, University Hospitals Birmingham NHS Foundation Trust. Conflict of interest, source of funding and authorship The authors declare that there are no conflicts of interest. The authors received no funding in the preparation of this study. LR carried out data collection, data analysis and the writing of the manuscript. AMc and SB carried out the writing of the manuscript. NM, SR, EW, SD, SA, AM carried out data collection and the writing of the manuscript. All authors critically reviewed the manuscript and approved the final version submitted for publication. References Anjema, K., van Rijn, M., Verkerk, P.H., Burgerhof, J.G., Heiner Fokkema, M.R. & van Spronsen, F.J. (211) PKU: high plasma phenylalanine concentrations are associated with increased prevalence of mood swings. Mol. Genet. Metab. 14, Belanger-Quintana, A. & Martinez-Pardo, M. (211) Physical development in patients with phenylketonuria on dietary treatment: a retrospective study. Mol. Genet. Metab. 14, Blau, N., van Spronsen, F. & Levy, H. (21) Phenylketonuria. Lancet. 376, Butland, B., Jebb, S., Kopelman, P., Mcpherson, K., Thomas, S., Mardell, J. & Parry, V. (27) Foresight, Tackling Obesities: Future Choices Project Report, 2nd edn. London: Government Office for Science. Christ, S.E., Huijbregts, S.C., de Sonnerville, L.M. & White, D.A. (21) Executive function in early treated phenylketonuria: profile and underlying mechanisms. Mol. Genet. Metab. 99(Suppl. 1), S22 S32. Gentile, J.K., Ten Hoedt, A.E. & Bosch, A.M. (21) Psychosocial aspects of PKU: hidden disabilities-a review. Mol. Genet. Metab. 99(Suppl. 1), S64 S67. Harding, C.O. & Blau, N. (21) Advances and challenges in phenylketonuria. J. Inherit. Metab. Dis. 33, ten Hoedt, A.E., de Sonneville, L.M.J., Francois, B., ter Horst, N.M., Janssen, M.C.H., Rubio- Gozalbo, M.E., Wijburg, F.A., Hollak, C.E.M. & Bosch, A.M. (211) High phenylalanine levels directly affect mood and sustained attention in adults with phenylketonuria: a randomised, double-blind, placebo-controlled, crossover trial. J. Inherit. Metab. Dis. 34, Huemer, M., Huemer, C., Moslinger, D., Huter, D. & Stockler-Ipsiroglu, S. (27) Growth and body composition in children with classical phenylketonuria: results in 34 and review of literature. J. Inherit. Metab. Dis. 3, Maillot, F., Cook, P., Lilburn, M. & Lee, P.J. (27) A practical approach to maternal phenylketonuria management. J. Inherit. Metab. Dis. 3, Medical Research Council Working Party on Phenylketonuria. (1993) Recommendations on the dietary management of phenylketonuria. Arch. Dis. Child. 68, Mobayed, E., Maritz, C., Chan, H., Grey, C., Ellerton, C., Freedman, F., Lachman, R. & Murphy, E. (211) Raised body mass index of adult patients with PKU. J. Inherit. Metab. Dis. 34, S49 S286. Modan-Moses, D., Vered, I., Schwartz, G., Anikster, Y., Abraham, S., Segev, R. & Efrati, O. (27) Peak bone mass in patients with phenylketonuria. J. Inherit. Metab. Dis. 3, Northern Ireland Health and Social Wellbeing Survey. (25/ 26) Northern Ireland Statistics and Research Agency in Conjunction with the Department of Health, Social Services and Public Health. Belfast: Northern Ireland Health and Social Wellbeing Survey. Paci, S., Sala, F., Gasparri, M., Zuvadelli, J., Salvaticia, E., Minghetti, D. & Riva, E. (29) Body mass index in PKU patients versus paediatric population in the city of Milan. Mol. Genet. Metab. 98, Scaglioni, S., Verduci, E., Fiori, L., Lammardo, A.M., Rossi, S., Radaelli, G. & Giovannini, M. (24) Body mass index rebound and overweight at 8 years of age in hyperphenylalaninaemic children. Acta Paediatr. 93, The Health and Social Care Information Centre. (21) Statistics on Obesity, Physical Activity and Diet: England. Leeds: The Health and Social Care Information Centre. The National Society for Phenylketonuria (UK). (24) Management of PKU, A Consensus Document for the Diagnosis and Management of Children, Adolescents and Adults with Phenylketonuria. London: The National Society for Phenylketonuria (UK). The Scottish Health Survey. (28) A National Statistics Publication for Scotland. Edinburgh: The Scottish Health Survey. VanZutphen, K.H., Packman, W., Sporri, L., Needham, M.C., Morgan, C., Weisiger, K. & Packman, S. (27) Excutive functioning in children and adolescents with phenylketonuria. Clin. Genet. 72, Journal of Human Nutrition and Dietetics ª 213 The British Dietetic Association Ltd.

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