The Impact of the First 2020 COVID-19 Lockdown on the Metabolic Control of Patients with Phenylketonuria
Abstract
:1. Introduction
2. Materials and Methods
- The number of patients who did and did not perform Phe tests in the lockdown and non-lockdown intervals (period P vs. periods NP1 to NP3),
- Phe concentrations in the assessed time intervals (periods P and NP1 to NP3 in patients who completed the Phe test in all four assessed time intervals,
- The number of Phe tests in non-lockdown time intervals (periods NP1 to NP3) in patients who did and did not complete the Phe test in the lockdown period (period P),
- The number of Phe tests in the assessed time intervals (periods P and NP1 to NP3) in patients with a yearly Phe median below (n = 258) and over (n = 264) the median for their age group (in 2018, patients born between January 2002 and December 2018 were identified. Within the individual birth years, patients were ranked according to the increasing median of Phe test results in 2019. The median of the results was determined for each age group, and the patients were classified into one of two groups: below the median or above the median of the results. In the case of years with an odd number of patients, the middle one was qualified to the group above the median. Groups created for individual years were assigned to two groups regardless of age: those in the group consisting of people born in a specific year with results below and above the median),
- The odds of performing only one Phe blood test in the assessed time intervals (lockdown period vs. non-lockdown periods) for patients who completed the Phe test in all four assessed time intervals.
3. Results
4. Discussion
5. Conclusions
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Conflicts of Interest
Abbreviations
References
- Scriver, C.R. ThePAH gene, phenylketonuria, and a paradigm shift. Hum. Mutat. 2007, 28, 831–845. [Google Scholar] [CrossRef] [PubMed]
- Riccio, E.; Pieroni, M.; Limoneglli, G.; Pisani, A. Impact of COVID-19 pandemic on patients with Fabry disease: An Italian experience. Mol. Genet. Metab. 2020, 131, 124–125. [Google Scholar] [CrossRef]
- Şenkalfa, B.P.; Eyuboglu, T.S.; Aslan, A.T.; Gursoy, T.R.; Soysal, A.S.; Yapar, D.; Ilhan, M.N. Effect of the COVID-19 pandemic on anxiety among children with cystic fibrosis and their mothers. Pediatr. Pulmonol. 2020, 55, 2128–2134. [Google Scholar] [CrossRef] [PubMed]
- Havermans, T.; Houben, J.; Vermeulen, F.; Boon, M.; Proesmans, M.; Lorent, N.; de Soir, E.; Vos, R.; Dupont, L. The impact of the COVID-19 pandemic on the emotional well-being and home treatment of Belgian patients with cystic fibrosis, including transplanted patients and paediatric patients. J. Cyst. Fibros. 2020, 19, 880–887. [Google Scholar] [CrossRef] [PubMed]
- Monzani, A.; Lionetti, E.; Felici, E.; Fransos, L.; Azzolina, D.; Rabbone, I.; Catassi, C. Adherence to the Gluten-Free Diet during the Lockdown for COVID-19 Pandemic: A Web-Based Survey of Italian Subjects with Celiac Disease. Nutrients 2020, 12, 3467. [Google Scholar] [CrossRef]
- Macdonald, A.; Gokmen-Ozel, H.; Van Rijn, M.; Burgard, P. The reality of dietary compliance in the management of phenylketonuria. J. Inherit. Metab. Dis. 2010, 33, 665–670. [Google Scholar] [CrossRef]
- Jurecki, E.; Cederbaum, S.; Kopesky, J.; Perry, K.; Rohr, F.; Sanchez-Valle, A.; Viau, K.; Sheinin, M.; Cohen-Pfeffer, J. Adherence to clinic recommendations among patients with phenylketonuria in the United States. Mol. Genet. Metab. 2017, 120, 190–197. [Google Scholar] [CrossRef] [PubMed]
- Christ, S.E.; Huijbregts, S.C.; De Sonneville, L.M.; White, D.A. Executive function in early-treated phenylketonuria: Profile and underlying mechanisms. Mol. Genet. Metab. 2010, 99, S22–S32. [Google Scholar] [CrossRef]
- Bilginsoy, C.; Waitzman, N.; Leonard, C.O.; Ernst, S.L. Living with phenylketonuria: Perspectives of patients and their families. J. Inherit. Metab. Dis. 2005, 28, 639–649. [Google Scholar] [CrossRef]
- Olsson, G.M.; Montgomery, S.M.; Alm, J. Family conditions and dietary control in phenylketonuria. J. Inherit. Metab. Dis. 2007, 30, 708–715. [Google Scholar] [CrossRef] [PubMed]
- Walkowiak, D.; Bukowska-Posadzy, A.; Walkowiak, J.; Kałużny, Ł.; Ołtarzewski, M.; Staszewski, R.; Musielak, M. Therapy compliance in children with phenylketonuria younger than 5 years: A cohort study. Adv. Clin. Exp. Med. 2019, 28, 1385–1391. [Google Scholar] [CrossRef] [PubMed]
- Freehauf, C.; Van Hove, J.L.; Gao, D.; Bernstein, L.; Thomas, J.A. Impact of geographic access to care on compliance and metabolic control in phenylketonuria. Mol. Genet. Metab. 2013, 108, 13–17. [Google Scholar] [CrossRef] [PubMed]
- Mlčoch, T.; Puda, R.; Ješina, P.; Lhotáková, M.; Štěrbová, Š.; Doležal, T. Dietary patterns, cost and compliance with low-protein diet of phenylketonuria and other inherited metabolic diseases. Eur. J. Clin. Nutr. 2017, 72, 87–92. [Google Scholar] [CrossRef] [PubMed]
- Levy, H.; Lamppu, D.; Anastosoaie, V.; Baker, J.L.; DiBona, K.; Hawthorne, S.; Lindenberger, J.; Kinch, D.; Seymour, A.; McIlduff, M.; et al. 5-year retrospective analysis of patients with phenylketonuria (PKU) and hyperphenylalaninemia treated at two specialized clinics. Mol. Genet. Metab. 2020, 129, 177–185. [Google Scholar] [CrossRef]
- Lilleväli, H.; Reinson, K.; Muru, K.; Saarsalu, S.; Künnapas, K.; Kahre, T.; Murumets, Ü.; Õunap, K. The evaluation of phenylalanine levels in Estonian phenylketonuria patients during eight years by electronic laboratory records. Mol. Genet. Metab. Rep. 2019, 19, 100467. [Google Scholar] [CrossRef]
- Güttler, F. Hyperphenylalaninemia: Diagnosis and classification of the various types of phenylalanine hydroxylase deficiency in childhood. Acta Paediatr. Scand. Suppl. 1980, 280, 1–80. [Google Scholar]
- Van Wegberg, A.M.J.; Macdonald, A.; Ahring, K.; BéLanger-Quintana, A.; Blau, N.; Bosch, A.M.; Burlina, A.; Campistol, J.; Feillet, F.; Giżewska, M.; et al. The complete European guidelines on phenylketonuria: Diagnosis and treatment. Orphanet J. Rare Dis. 2017, 12, 1–56. [Google Scholar] [CrossRef] [Green Version]
- Modi, A.C.; Pai, A.; Hommel, A.K.; Hood, K.K.; Cortina, S.; Hilliard, M.; Guilfoyle, S.M.; Gray, W.N.; Drotar, D. Pediatric Self-management: A Framework for Research, Practice, and Policy. Pediatrics 2012, 129, e473–e485. [Google Scholar] [CrossRef] [Green Version]
- Di Renzo, L.; Gualtieri, P.; Pivari, F.; Soldati, L.; Attinà, A.; Cinelli, G.; Leggeri, C.; Caparello, G.; Barrea, L.; Scerbo, F.; et al. Eating habits and lifestyle changes during COVID-19 lockdown: An Italian survey. J. Transl. Med. 2020, 18, 229. [Google Scholar] [CrossRef]
- Sánchez-Sánchez, E.; Ramírez-Vargas, G.; Avellaneda-López, Y.; Orellana-Pecino, J.I.; García-Marín, E.; Díaz-Jimenez, J. Eating Habits and Physical Activity of the Spanish Population during the COVID-19 Pandemic Period. Nutrients 2020, 12, 2826. [Google Scholar] [CrossRef]
- Sidor, A.; Rzymski, P. Dietary Choices and Habits during COVID-19 Lockdown: Experience from Poland. Nutrients 2020, 12, 1657. [Google Scholar] [CrossRef]
- Martinez-Ferran, M.; De La Guía-Galipienso, F.; Sanchis-Gomar, F.; Pareja-Galeano, H. Metabolic Impacts of Confinement during the COVID-19 Pandemic Due to Modified Diet and Physical Activity Habits. Nutrients 2020, 12, 1549. [Google Scholar] [CrossRef]
- Walkowiak, D.; Kaluzny, L.; Bukowska-Posadzy, A.; Oltarzewski, M.; Staszewski, R.; Moczko, J.A.; Musielak, M.; Walkowiak, J. Overweight in classical phenylketonuria children: A retrospective cohort study. Adv. Med. Sci. 2019, 64, 409–414. [Google Scholar] [CrossRef] [PubMed]
- Nicola, M.; Alsafi, Z.; Sohrabi, C.; Kerwan, A.; Al-Jabir, A.; Iosifidis, C.; Agha, M.; Agha, R. The socio-economic implications of the coronavirus pandemic (COVID-19): A review. Int. J. Surg. 2020, 78, 185–193. [Google Scholar] [CrossRef] [PubMed]
- Carroll, N.; Sadowski, A.; Laila, A.; Hruska, V.; Nixon, M.; Ma, D.W.; Haines, J.; on behalf of the Guelph Family Health Study. The Impact of COVID-19 on Health Behavior, Stress, Financial and Food Security among Middle to High Income Canadian Families with Young Children. Nutrients 2020, 12, 2352. [Google Scholar] [CrossRef] [PubMed]
- Pérez-Escamilla, R.; Cunningham, K.; Moran, V.H. COVID-19 and maternal and child food and nutrition insecurity: A complex syndemic. Matern. Child Nutr. 2020, 16, e13036. [Google Scholar] [CrossRef]
- Gokden, Y.; Hot, S.; Adas, M.; Koc, D.O.; Atak, S.; Hot, A.B. Celiac disease and COVID-19 pandemic: Should we worry? Acta Gastroenterol. Belg. 2020, 83, 517–525. [Google Scholar]
- Bartoszek, A.; Walkowiak, D.; Bartoszek, A.; Kardas, G. Mental Well-Being (Depression, Loneliness, Insomnia, Daily Life Fatigue) during COVID-19 Related Home-Confinement—A Study from Poland. Int. J. Environ. Res. Public Health 2020, 17, 7417. [Google Scholar] [CrossRef]
- Prime, H.; Wade, M.; Browne, D.T. Risk and resilience in family well-being during the COVID-19 pandemic. Am. Psychol. 2020, 75, 631–643. [Google Scholar] [CrossRef]
- Borghi, L.; Salvatici, E.; Banderali, G.; Riva, E.; Giovannini, M.; Vegni, E. Psychological wellbeing in parents of children with phenylketonuria and association with treatment adherence. Minerva Pediatr. 2018. [Google Scholar] [CrossRef]
- Giavoli, C.; Profka, E.; Giancola, N.; Rodari, G.; Giacchetti, F.; Ferrante, E.; Arosio, M.; Mantovani, G. Growth hormone therapy at the time of Covid-19 pandemic: Adherence and drug supply issues. Eur. J. Endocrinol. 2020, 183, 13. [Google Scholar] [CrossRef] [PubMed]
Parameter Studied | Period P | Period NP1 | Period NP2 | Period NP3 | |
---|---|---|---|---|---|
Number of patients | 535 | 533 | 502 | 499 | |
Gender | Boys Girls | 281 254 | 281 252 | 262 240 | 260 239 |
Age (years) | Median (IQR) | 7.8 (4.3–12) | 7.6 (4.2–11.9) | 7.6 (4.2–11.6) | 7.6 (4.1–11.6) |
Mean (SD) | 8.2 (4.8) | 8.1 (4.8) | 7.9 (4.7) | 7.9 (4.8) | |
Median Phe (mg/dL) | Range | 0.4–29.2 | 0.4–29.4 | 0.3–29.8 | 0.4–29.3 |
Median (IQR) | 5.4 (3.1–9.4) | 5.3 (3.2–8.5) | 5.3 (3.0–8.8) | 5.4 (3.1–9.4) | |
Mean (SD) | 6.9 (5.0) | 6.6 (4.8) | 6.6 (4.9) | 6.9 (5.0) |
Age (Years)/(Number of Patients) | |||
---|---|---|---|
0–6 (n = 191) | 7–12 (n = 210) | 13–18 (n = 121) | |
Number of Phe tests | |||
Range | 2–108 | 1–47 | 1–42 |
Median (IQR) | 22 (11–40) | 9 (5–16) | 6 (4–10) |
Mean (SD) | 26.4 (19.3) | 11.5 (8.3) | 7.8 (5.7) |
Yearly median Phe | |||
Range | 1.5–19 | 1–27.4 | 1.7–22.8 |
Median (IQR) | 4.4 (3.1–6.6) | 5.5 (3.5–8.7) | 9.9 (5.9–13.6) |
Mean (SD) | 5.3 (3.2) | 6.8 (4.7) | 10.1 (5.2) |
Lockdown Period | Non-Lockdown Periods | ||||
---|---|---|---|---|---|
Phe Tests | Period P N (%) | Period NP1 N (%) | Period NP2 N (%) | Period NP3 N (%) | |
0–6 years | No | 30 (15.3) | 11 (5.7) | 11 (5.6) | 15 (7.7) |
Yes | 166 (84.7) | 183 (94.3) | 186 (94.4) | 179 (92.3) | |
LR (vs. Period P) | 9.97 | 9.94 | 5.58 | ||
p | 0.002 | 0.001 | 0.02 | ||
7–12 years | No | 74 (36.1) | 35 (17.1) | 34 (17.4) | 43 (22.1) |
Yes | 131 (63.9) | 170 (82.9) | 161 (82.6) | 152 (77.9) | |
LR (vs. Period P) | 19.34 | 18.01 | 9.62 | ||
p | <0.001 | <0.001 | 0.002 | ||
13–18 years | No | 71 (53.0) | 37 (27.6) | 31 (28.2) | 28 (25.5) |
Yes | 63 (47.0) | 97 (72.4) | 79 (71.8) | 82 (74.5) | |
LR (vs. Period P) | 18.17 | 15.56 | 19.45 | ||
p | <0.001 | <0.001 | <0.001 | ||
Number of patients | 535 | 533 | 502 | 499 |
Age Group (Years) | Period P | Period NP1 | Period NP2 | Period NP3 | p | ||
---|---|---|---|---|---|---|---|
0–6 | Number of patients | 122 | 122 | 145 | 145 | ||
Phe (mg/dL) | Range | 0.4–22.1 | 1.2–19.7 | 0.7–18.9 | 0.5–20 | ||
Median (IQR) | 3.9 (2.7–7.2) | 4.4 (2.9–6.2) | 4.6 (2.8–7) | 4.4 (2.7–6.6) | ns | ||
Mean (SD) | 5.3 (4.1) | 5.2 (3.6) | 5.4 (3.5) | 5.1 (3.4) | |||
7–12 | Number of patients | 103 | 103 | 94 | 94 | ||
Phe (mg/dL) | Range | 0.4–21.9 | 0.8–18.9 | 0.7–16.5 | 1.5–20.3 | ||
Median (IQR) | 5.2 (3.3–7) | 5.4 (3.3–7) | 4.2 (2.4–7.4) | 5.0 (3–9.5) | ns | ||
Mean (SD) | 5.7 (3.4) | 5.7 (3.3) | 5.3 (3.8) | 6.3 (4.1) | |||
13–18 | Number of patients | 41 | 41 | 27 | 27 | ||
Range | 0.7–26.7 | 0.4–23.3 | 1–21.8 | 0.5–24.5 | |||
Phe (mg/dL) | Median (IQR) | 7.3 (4.5–12.5) | 7.5 (3.4–11.8) | 9.9 (6.6–14) | 9.4 (6.5–13) | ns | |
Mean (SD) | 8.8 (6.6) | 8.3 (5.8) | 10.2 (5.3) | 10.5 (5.7) |
Period NP1 | Period NP2 | Period NP3 | |||||
---|---|---|---|---|---|---|---|
Phe test performed in period P | No | Yes | No | Yes | No | Yes | |
Number of patients | 175 | 358 | 172 | 330 | 172 | 327 | |
Phe tests in non-lockdown period (N) | Range | 0–4 | 0–14 | 0–13 | 0–13 | 0–19 | 0–16 |
Median (IQR) | 1 (0–1) | 2 (1–4) | 1 (0–1) | 2 (1–4) | 1 (0–1) | 2 (1–4) | |
Mean (SD) | 0.9 (0.8) | 2.8 (2.3) | 1.1 (1.4) | 3.0 (2.6) | 1.1 (1.6) | 2.8 (2.4) | |
p | <0.001 | <0.001 | <0.001 |
Lockdown Period | Non-Lockdown Periods | ||||||||
---|---|---|---|---|---|---|---|---|---|
Period P | Period NP1 | Period NP2 | Period NP3 | ||||||
Below | Over | Below | Over | Below | Over | Below | Over | ||
Number of Phe tests | Range | 0–12 | 0–13 | 0–12 | 0–11 | 0–12 | 0–13 | 0–13 | 0–12 |
Median (IQR) | 1 (0–2) | 1 (0–2) | 1 (1–2.7) | 1 (1–2) | 1 (1–3) | 1 (1–3) | 1 (1–3) | 1 (1–3) | |
Mean (SD) | 1.7 (2.1) | 1.3 (1.7) | 1.9 (2.0) | 1.7 (1.6) | 2.2 (2.4) | 2.0 (2.1) | 2.1 (2.1) | 1.9 (1.9) | |
p | 0.03 | 0.13 | 0.29 | 0.33 |
Odds of Having Only One Phe Test in a Period | |||
---|---|---|---|
Period P vs. Period NP1 | Period P vs. Period NP2 | Period P vs. Period NP3 | |
OR | 1.43 | 1.60 | 1.52 |
95% CI | 1.01–2.04 | 1.11–2.30 | 1.06–2.19 |
p | 0.02 | 0.005 | 0.01 |
Publisher’s Note: MDPI stays neutral with regard to jurisdictional claims in published maps and institutional affiliations. |
© 2021 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
Share and Cite
Walkowiak, D.; Mikołuć, B.; Mozrzymas, R.; Kałużny, Ł.; Didycz, B.; Jaglowska, J.; Kurylak, D.; Walkowiak, J. The Impact of the First 2020 COVID-19 Lockdown on the Metabolic Control of Patients with Phenylketonuria. Nutrients 2021, 13, 2024. https://doi.org/10.3390/nu13062024
Walkowiak D, Mikołuć B, Mozrzymas R, Kałużny Ł, Didycz B, Jaglowska J, Kurylak D, Walkowiak J. The Impact of the First 2020 COVID-19 Lockdown on the Metabolic Control of Patients with Phenylketonuria. Nutrients. 2021; 13(6):2024. https://doi.org/10.3390/nu13062024
Chicago/Turabian StyleWalkowiak, Dariusz, Bożena Mikołuć, Renata Mozrzymas, Łukasz Kałużny, Bożena Didycz, Joanna Jaglowska, Danuta Kurylak, and Jarosław Walkowiak. 2021. "The Impact of the First 2020 COVID-19 Lockdown on the Metabolic Control of Patients with Phenylketonuria" Nutrients 13, no. 6: 2024. https://doi.org/10.3390/nu13062024