Longitudinal Dietary Intake Data in Patients with Phenylketonuria from Europe: The Impact of Age and Phenylketonuria Severity
Abstract
:1. Introduction
2. Materials and Methods
2.1. Participating Centres
2.2. Patient Selection
2.3. Study Design and Data Collection
2.4. Statistical Analysis
2.5. Ethical Aspects
3. Results
3.1. Treatment Centres’ Characteristics
3.2. Subjects Characteristics
3.3. Overall Data on Metabolic Control and Prescribed Dietary Protein/Phe Intakes Per Centre
3.3.1. Phenylalanine and Natural Protein Intakes Per Centre
3.3.2. Protein Equivalent Intake from Protein Substitutes Per Centre
3.3.3. Total Protein Intakes Per Centre
3.4. Dietary Prescribed Protein/Phenylalanine Intakes by Gender and Age
3.4.1. Phenylalanine and Natural Protein Intakes by Gender and Age Group
3.4.2. Protein Equivalent Intake from Protein Substitutes by Gender and Age Group
3.4.3. Total Protein Intakes by Gender and Age Group
3.5. Dietary Prescribed Protein/Phenylalanine Intakes by PKU Severity
3.5.1. Phenylalanine and Natural Protein Intakes by PKU Severity
3.5.2. Protein Equivalent Intake from Protein Substitutes by PKU Severity
3.5.3. Total Protein Intakes by PKU Severity
3.6. Dietary Prescribed Phenylalanine/Natural Protein Intakes Comparing Patients on Sapropterin vs. Diet-Only Treatment
4. Discussion
Limitations
5. Conclusions
Supplementary Materials
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Conflicts of Interest
References
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Centre A | Centre B | Centre C | Centre D | Centre E | Centre F | Centre G | Centre H | Centre I | |
---|---|---|---|---|---|---|---|---|---|
Number of patients with data on dietary treatment (%) | 318 (99) | 97 (100) | 95 (94) | 81 (84) | 115 (99) | 59 (100) | 247 (79) | 62 (100) | 89 (56) |
Dietetic treatment guidelines: Exchange system (Yes/No) -If yes, type of exchange | Yes 1 exchange = 15 mg Phe, 0.5 g protein for fruits = 15 mg Phe, 0.6 g protein = 50 mg Phe, 1 g protein for bread, cereals | Yes 1 exchange = 50 mg Phe = 1 g protein | No | Yes 1 exchange = 20 mg Phe | Yes 1 exchange = 20 mg Phe | No | Yes 1 exchange = 25 mg Phe | No | Yes 1 exchange = 50 mg Phe = 1 g protein |
Unmeasured/unlimited use of fruit and vegetables (except potatoes) containing Phe ≤ 75 mg/100 g (Yes/No) | No | Yes | Yes | No | Yes | No | No | Yes | Yes |
Foods containing protein ≤0.5 g/100 g given in unlimited amounts | No | Yes | No | No | Yes | No | No | Yes | Yes |
Type of protein substitutes used | L-AA (powder, liquid) LNAA (powder, tablets) | L-AA (powder, liquid, tablets) GMP (powder, liquid) | L-AA (powder, liquid, tablets) | L-AA (powder, liquid) | L-AA (powder, liquid, tablets) GMP (powder, liquid, bars) LNAA (powder, tablets) | L-AA (powder, liquid) | L-AA (powder, liquid, tablets, bars) GMP (powder, liquid, bars) LNAA (powder, tablets) | L-AA (powder, liquid) | L-AA (powder, liquid, tablets) GMP (powder, liquid, bars) LNAA (powder, tablets) |
Therapeutic target range for blood Phe (μmol/L) | <12 y: 120–360 >12 y: 120–600 | <12 y: 120–360 >12 y: 120–600 | <12 y: 120–360 >12 y: 120–600 | 2012–2017: <12 y: 120–600 >12 y: 600–1200 2018: <12 y: 120–360 >12 y: 360–900 | <12 y: 120–360 >12 y: 120–480 | <12 y: 120–360 >12 y: 120–600 | 2012–2017: 0–4 y: 180–300 4–8 y: 180–400 8–10 y: 180–600 10–12 y: 180–700 >12 y: 180–900 2018: <12 y: 120–360 >12 y: 360–600 | 2012–2017: 0–6 y: 120–360 6–9 y: 120–540 10–18 y: 120–600 >18 y: 120–840 2018: <12 y: 120–360 >12 y: 360–600 | <12 y: 120–360 >12 y: 120–600 |
Frequency of clinic visits | 0–1 y: monthly; 1–2 y: 2 monthly; 2–3 y: 3 monthly; 4–18 y: 4 times a year >18 y: 6 monthly | 0–1 y: 3 monthly; 1–18 y: 6 monthly | 0–1 y: 2 monthly; 1–18 y: twice/three times a year; >18 y: once a year | 0–1 y: 3 monthly; 1–18 y: 6 monthly; >18 y: once a year | 0–6 m: monthly; 6–12 m: 2 monthly; 1–12 y: 3 monthly; 12–18 y: 2/3 times a yearly; >18 y: once/twice a year | 0–1 y: monthly; 2–5 y: 2 monthly; 6–12 y: 3/4 monthly; 13–18 y: 6 monthly; >18 y: once a year | 0–6 m: 2 monthly; 6–18 m: 3 monthly; 18 m-6 y: 6 monthly; >6 y: once a year | 0–1 y: monthly; 1–6 y: 3 monthly; >6 y: 6 monthly | 0–1 y: monthly; 1–12 y: 6 monthly; >12 y: once a year |
Number of dietitians full time dedicated to IMD | 3 (not specific to IMD) | 1.3 (specific to PKU) | 1.8 | 5 (not specific to IMD) | 2 | 1 | 2.7 | 0 | 2 |
Centre | % Blood Phe Levels in Target | Phe Mean ± SD (Median; Range) | Natural Protein Mean ± SD (Median; Range) | Protein Equivalent from Protein Substitute Mean ± SD (Median; Range) | Total Protein Mean ± SD (Median; Range) | ||||
---|---|---|---|---|---|---|---|---|---|
(N of Patients) | (Mean % ± SD) | mg/day | mg/kg/day | g/day | g/kg/day | g/day | g/kg/day | g/day | g/kg/day |
A (n = 320) | 70 ± 48 | 658 ± 492 (465; 180–3350) | 21 ± 22 (15; 0–176) | 13 ± 10 (9; 4–67) | 0.5 ± 0.4 (0.4; 0.1–3.5) | 17 ± 14 (19; 0–60) | 0.7 ± 0.5 (0.7; 0–3.2) | 36 ± 11 (35; 7–70) | 1.3 ± 0.6 (1.3; 0–4.0) |
B * (n = 97) | 83 ± 30 | 417 ± 367 (250; 123–1750) | 18 ± 19 (12; 0–125) | 8 ± 7 (5; 2–35) | 0.4 ± 0.4 (0.2; 0–2.5) | 52 ± 20 (60; 2–90) | 2.0 ± 0.7 (2.0; 0.5–3.5) | 61 ± 20 (64; 5–102) | 2.3 ± 0.7 (2.4; 0.6–4.5) |
C (n = 101) | 79 ± 53 | 1065 ± 816 (800; 100–4500) | 17 ± 19 (12; 0–173) | 21 ± 16 (16; 4–90) | 0.4 ± 0.4 (0.3; 0.0–3.5) | 41 ± 21 (42; 0–84) | 0.8 ± 0.5 (0.8; 0–2.4) | 62 ± 20 (65; 10–109) | 1.3 ± 0.6 (1.1; 0.4–4.4) |
D (n = 96) | 67 ± 52 | 887 ± 748 (565; 104–4725) | 29 ± 34 (18; 0–219) | 22 ± 18 (15; 2–105) | 0.8 ± 0.7 (0.5; 0–4.9) | 23 ± 21 (22; 0–75) | 0.7 ± 0.6 (0.8; 0–2.2) | 45 ± 21 (42; 5–108) | 1.5 ± 0.6 (1.5; 0.1–4.9) |
E + (n = 116) | 87 ± 49 | 2097 ± 1738 (1427; 262–9009) | 45 ± 37 (32; 0–215) | 45 ± 34 (31; 6–182) | 1.0 ± 0.8 (0.7; 0.1–4.4) | 37 ± 24 (39; 0–94) | 0.8 ± 0.5 (0.8; 0–2.4) | 80 ± 29 (82; 0–182) | 1.7 ± 0.6 (1.6; 0.5–4.4) |
F (n = 59) | 66 ± 32 | 379 ± 194 (350; 83–1200) | 16 ± 12 (12; 3–64) | 8 ± 4 (7; 2–24) | 0.3 ± 0.2 (0.2; 0.1–1.3) | 44 ± 21 (42; 4–90) | 1.5 ± 0.5 (1.4; 0.5–3.3) | 51 ± 22 (49; 8–110) | 1.8 ± 0.5 (1.8; 0.9–3.6) |
G (n = 314) | 65 ± 54 | 984 ± 951 (579; 0–7500) | 18 ± 24 (12; 0–205) | 22 ± 20 (14; 0–150) | 0.5 ± 0.5 (0.4; 0–4.1) | 45 ± 25 (40; 0–120) | 1.2 ± 0.7 (1.1; 0–5.3) | 67 ± 26 (68; 6–192) | 1.7 ± 0.7 (1.6; 0.2–6.4) |
H (n = 62) | 71 ± 44 | 710 ± 667 (500; 0–3000) | 14 ± 18 (7; 0–129) | 15 ± 17 (10; 0–60) | 0.4 ± 0.7 (0.2; 0–2.6) | 53 ± 29 (60; 0–100) | 1.2 ± 0.8 (1.2; 0–3.3) | 76 ± 19 (84; 16–128) | 1.8 ± 0.7 (1.5; 0.7–3.9) |
I (n = 158) | 88 ± 49 | 942 ± 875 (620; 104–5000) | 16 ± 23 (12; 0–171) | 21 ± 19 (14; 2–100) | 0.5 ± 0.5 (0.4; 0–3.4) | 36 ± 23 (36; 0–100) | 1.0 ± 0.8 (1.1; 0–3.0) | 57 ± 23 (58; 10–120) | 1.7 ± 0.7 (1.6; 0.1–3.6) |
Total (n = 1323) | 71 ± 46 | 904 ± 761 (565; 0–9009) | 22 ± 23 (15; 0–219) | 19 ± 16 (14; 0–182) | 0.5 ± 0.5 (0.4; 0–10.0) | 39 ± 22 (40; 0–120) | 1.1 ± 0.6 (1.1; 0–5.3) | 59 ± 21 (64; 10–192) | 1.7 ± 0.6 (1.6; 0.1–6.4) |
Sex and Age (Number of Patients) | Mean ± SD (Median; Range) | WHO/FAO/UNU 2007 Safe Levels for Total Protein | % of Mean Total Protein Intake Meeting Recommendations | |||||||
---|---|---|---|---|---|---|---|---|---|---|
Phe | Natural Protein | Protein Equivalent from Protein Substitute | Total Protein | |||||||
mg/day | mg/kg/day | g/day | g/kg/day | g/day | g/kg/day | g/day | g/kg/day | g/kg/day | ||
Female (n = 666) | 881 ± 875 (500; 0–5820) | 20 ± 25 (13; 0–219) | 19 ± 19 (11; 0–181) | 0.5 ± 0.6 (0.3; 0.0–4.9) | 35 ± 26 (32; 0–120) | 1.0 ± 0.7 (1.0; 0.0–4.6) | 57 ± 25 (55; 0–167) | 1.6 ± 0.7 (1.5; 0.0–5.8) | NA | NA |
Male (n = 657) | 1027 ± 1200 (550; 0–9009) | 29 ± 34 (12; 0–215) | 22 ± 24 (12; 0–182) | 0.5 ± 0.6 (0.4; 0.0–4.2) | 35 ± 25 (31; 0–113) | 1.0 ± 0.7 (1.0; 0–5.0) | 60 ± 28 (56; 0–192) | 1.8 ± 0.7 (1.6; 0.1–6.4) | NA | NA |
p-value | 0.218 | 0.019 | 0.250 | 0.019 | 0.830 | 0.013 | 0.850 | <0.001 | ||
<2 y (n= 47) | 318 ± 206 (250; 0–1297) | 34 ± 35 (29; 0–162) | 7 ± 5 (5; 0–30) | 1.0 ± 0.7 (0.9; 0.0–3.5) | 7 ± 9 (7; 0–54) | 1.1 ± 1.1 (1.1; 0.0–5.3) | 17 ± 9 (15; 6–58) | 2.4 ± 1.3 (2.4; 0.0–6.4) | 1.14 1–1.31 2 | 210–183% |
2–5 y (n = 213) | 521 ± 468 (352; 100–3802) | 29 ± 32 (21; 0–219) | 11 ± 10 (8; 0–78) | 0.8 ± 0.7 (0.5; 0.0–4.9) | 19 ± 15 (19; 0–60) | 1.3 ± 0.9 (1.4; 0.0–5.3) | 33 ± 12 (32; 0–92) | 2.2 ± 0.8 (2.2; 0.0–6.4) | 0.94 | 234% |
6–12 y (n = 353) | 750 ± 756 (450; 0–5360) | 22 ± 25 (15; 0–206) | 16 ± 17 (10; 0–181) | 0.5 ± 0.6 (0.3; 0.0–4.2 | 36 ± 22 (34; 0–106) | 1.1 ± 0.7 (1.1; 0.0–3.3) | 54 ± 18 (52; 10–115) | 1.7 ± 0.6 (1.7; 0.0–4.2) | 0.89 3–0.94 4 | 191–181% |
13–18 y (n = 268) | 1301 ± 1349 (750; 0–9009) | 20 ± 25 (11; 0–215) | 27 ± 26 (17; 2–158) | 0.5 ± 0.5 (0.3; 0.0–3.5) | 47 ± 26 (48; 0–116) | 0.8 ± 0.4 (0.8; 0.0–2.3) | 75 ± 22 (8; 0–175) | 1.3 ± 0.4 (1.2; 0.0–3.6) | 0.84 5–0.89 6 | 155–146% |
19–30 y (n = 280) | 1419 ± 1328 (900; 0–9009) | 18 ± 19 (11; 0–123) | 31 ± 28 (20; 0–182) | 0.4 ± 0.4 (0.3; 0.0–2.5) | 48 ± 25 (53; 0–110) | 0.7 ± 0.4 (0.8; 0.0–1.6) | 78 ± 24 (79; 0–182) | 1.2 ± 0.4 (1.2; 0.2–2.5) | 0.84 | 143% |
31–40 y (n = 107) | 1377 ± 1027 (1148; 100–4725) | 14 ± 13 (10; 0–62) | 30 ± 23 (25; 4–105) | 0.4 ± 0.3 (0.3; 0.0–1.8) | 47 ± 26 (52; 0–120) | 0.6 ± 0.4 (0.7; 0.0–1.3) | 78 ± 23 (76; 0–143) | 1.0 ± 0.3 (1.0; 0.1–2.0) | 0.84 | 119% |
≥41 y (n = 55) | 1255 ± 917 (750; 350–3750) | 7 ± 11 (0; 0–53) | 27 ± 20 (15; 7–75) | 0.3 ± 0.3 (0.2; 0.0–1.1) | 47 ± 24 (50; 0–106) | 0.6 ± 0.3 (0.7; 0.0–1.2) | 80 ± 22 (77; 35–192) | 1.0 ± 0.3 (1.0; 0.4–2.1) | 0.84 | 119% |
PKU Severity | Age Group | Mean ± SD (Median; Range) | |||||||
---|---|---|---|---|---|---|---|---|---|
Phe | Natural Protein | Protein Equivalent from Protein Substitute | Total Protein | ||||||
mg/day | mg/kg/day | g/day | g/kg/day | g/day | g/kg/day | g/day | g/kg/day | ||
HPA | <2 y | 656 ± 286 (590; 175–1297) | 19 ± 43 (0; 0–162) | 14 ± 7 (13; 3–30) | 1.3 ± 1.2 (1.1; 0–3.5) | 1 ± 3 (0; 0–13) | 0.1 ± 0.4 (0; 0–1.7) | 18 ± 8 (15; 10–37) | 1.4 ± 1.5 (1.7; 0–4.4) |
2–5 y | 1385 ± 734 (1359; 150–3802) | 26 ± 50 (0; 0–219) | 29 ± 15 (28; 4–78) | 1.6 ± 1.2 (1.8; 0–4.9) | 2 ± 5 (0; 0–36) | 0.1 ± 0.3 (0; 0–1.8) | 34 ± 13 (31; 5–82) | 1.6 ± 1.3 (1.9; 0–4.9) | |
6–12 y | 2074 ± 1281 (1719; 400–5360) | 31 ± 43 (0; 0–206) | 44 ± 27 (40; 8–111) | 1.3 ± 0.9 (1.2; 0–4.2) | 9 ± 13 (0; 0–50) | 0.3 ± 0.4 (0; 0–1.6) | 58 ± 22 (51; 24–111) | 1.6 ± 1.0 (1.6; 0–4.2) | |
13–18 y | 3325 ± 1577 (3219; 500–7724) | 40 ± 41 (34; 0–170) | 70 ± 32 (70; 10–158) | 1.1 ± 0.8 (1.1; 0–3.5) | 13 ± 17 (0; 0–70) | 0.2 ± 0.3 (0; 0–1.5) | 84 ± 28 (89; 35–158) | 1.5 ± 0.7 (1.5; 0–3.6) | |
19–30 y | 2851 ± 2128 (2300; 239–7473) | 24 ± 31 (6; 0–106) | 59 ± 44 (50; 7–153) | 0.7 ± 0.6 (0.6; 0–2.2) | 28 ± 30 (23; 0–108) | 0.3 ± 0.4 (0.1; 0–1.1) | 85 ± 32 (80; 0–153) | 1.2 ± 0.4 (1.1; 0.4–2.2) | |
31–40 y | 1104 ± 389 (1250; 663–1400) | 4 ± 9 (0; 0–22) | 23 ± 6 (25; 16–28) | 0.3 ± 0.2 (0.3; 0–0.4) | 47 ± 3 (45; 45–50) | 0.9 ± 0.1 (0.9; 0.8–1.0) | 70 ± 4 (70; 66–73) | 1.3 ± 0.0 (1.3; 1.2–1.3) | |
≥41 y | n/a | n/a | n/a | n/a | n/a | n/a | n/a | n/a | |
HPA Total | 2270 ± 1586 (1910; 150–7724) | 29 ± 45 (0; 0–219) | 48 ± 33 (41; 3–158) | 1.3 ± 1.0 (1.1; 0–4.9) | 8 ± 15 (0; 0–108) | 0.2 ± 0.4 (0; 0–1.8) | 61 ± 31 (52; 0–158) | 1.5 ± 1.0 (1.6; 0.0–4.9) | |
mPKU | <2 y | 334 ± 222 (258; 0–1225) | 45 ± 37 (41; 0–128) | 7 ± 4 (5; 0–25) | 1.1 ± 0.7 (1.1; 0–2.6) | 7 ± 5 (7; 0–22) | 1.3 ± 1.1 (1.3; 0–4.4) | 15 ± 5 (15; 6–32) | 2.6 ± 1.2 (2.5; 0–6.4) |
2–5 y | 615 ± 452 (432; 150–2450) | 38 ± 31 (28; 0–175) | 13 ± 9 (9; 3–49) | 0.9 ± 0.6 (0.7; 0–3.9) | 20 ± 14 (20; 0–60) | 1.4 ± 0.9 (1.4; 0–4.4) | 34 ± 12 (33; 0–72) | 2.3 ± 0.8 (2.3; 0–6.4) | |
6–12 y | 941 ± 664 (750; 184–4306) | 28 ± 24 (21; 0–122) | 20 ± 14 (16; 4–95) | 0.7 ± 0.5 (0.5; 0–2.9) | 31 ± 20 (30; 0–84) | 1.0 ± 0.7 (1.1; 0–2.8) | 53 ± 17 (52; 20–106) | 1.7 ± 0.5 (1.7; 0–3.7) | |
13–18 y | 1637 ± 1131 (1371; 250–6014) | 23 ± 21 (16; 0–110) | 34 ± 23 (29; 5–125) | 0.6 ± 0.4 (0.4; 0–2.2) | 37 ± 23 (36; 0–84) | 0.6 ± 0.4 (0.6; 0–1.5) | 72 ± 23 (74; 0–128) | 1.2 ± 0.4 (1.1; 0–2.7) | |
19–30 y | 1892 ± 1529 (1500; 295–9009) | 22 ± 23 (15; 0–123) | 40 ± 32 (30; 7–182) | 0.6 ± 0.5 (0.5; 0–2.5) | 38 ± 22 (38; 0–110) | 0.6 ± 0.4 (0.6; 0–1.4) | 78 ± 27 (78; 0–182) | 1.2 ± 0.4 (1.1; 0.4–2.5) | |
mPKU | 31–40 y | 1744 ± 962 (1550; 100–4650) | 17 ± 14 (19; 0–50) | 38 ± 22 (31; 8–105) | 0.5 ± 0.3 (0.4; 0–1.8) | 41 ± 24 (48; 0–100) | 0.5 ± 0.3 (0.6; 0–1.2) | 80 ± 20 (78; 10–128) | 1.1 ± 0.3 (1.1; 0.1–2.0) |
≥41 y | 1433 ± 995 (1057; 363–3750) | 8 ± 14 (0; 0–53) | 33 ± 23 (24; 7–75) | 0.4 ± 0.3 (0.3; 0–1.1) | 42 ± 24 (42; 0–76) | 0.5 ± 0.4 (0.4; 0–1.1) | 77 ± 16 (78; 35–105) | 1.0 ± 0.3 (0.9; 0.4–1.5) | |
mPKU Total | 1262 ± 1166 (900; 0–9009) | 28 ± 26 (21; 0–175) | 26 ± 23 (20; 0–182) | 0.7 ± 0.5 (0.5; 0–3.9) | 32 ± 22 (30; 0–110) | 0.9 ± 0.7 (0.9; 0–4.4) | 60 ± 28 (58; 0–182) | 1.7 ± 0.7 (1.5; 0–6.4) | |
cPKU | <2 y | 251 ± 102 (234; 83–500) | 38 ± 23 (34; 0–125) | 5 ± 2 (5; 0–10) | 0.8 ± 0.4 (0.8; 0–2.5) | 12 ± 9 (9; 2–54) | 1.8 ± 0.9 (1.5; 0–5.3) | 18 ± 10 (14; 6–58) | 2.6±1.1 (2.5; 0–6.3) |
2–5 y | 337 ± 155 (310; 100–1631) | 24 ± 13 (22; 0–88) | 7 ± 3 (7; 0–35) | 0.5 ± 0.2 (0.5; 0–1.8) | 26 ± 11 (24; 2–60) | 1.8 ± 0.6 (1.7; 0–5.3) | 33 ± 11 (31; 6–92) | 2.3±0.6 (2.2; 0.7–6.3) | |
6–12 y | 414 ± 207 (400; 0–2000) | 14 ± 8 (13; 0–70) | 9 ± 8 (8; 0–75) | 0.3 ± 0.4 (0.3; 0–4.2) | 45 ± 19 (42; 0–106) | 1.4 ± 0.5 (1.4; 0–3.3) | 54 ± 18 (51; 23–115) | 1.7±0.5 (1.7; 0.6–3.5) | |
13–18 y | 654 ± 474 (500; 0–4500) | 11 ± 9 (9; 0–66) | 14 ± 11 (11; 2–90) | 0.2 ± 0.2 (0.2; 0–1.3) | 58 ± 22 (60; 0–116) | 1.0 ± 0.4 (1.0; 0–2.3) | 72 ± 20 (74; 16–175) | 1.2±0.3 (1.2; 0.3–2.4) | |
19–30 y | 1033 ± 904 (719; 0–6096) | 13 ± 14 (10; 0–87) | 23 ± 20 (15; 0–129) | 0.3 ± 0.3 (0.2; 0–1.8) | 53 ± 24 (60; 0–106) | 0.8 ± 0.4 (0.8; 0–1.6) | 76 ± 21 (78; 0–146) | 1.2±0.3 (1.2; 0.2–2.4) | |
31–40 y | 1206 ± 1060 (845; 171–4725) | 12 ± 13 (12; 0–62) | 26 ± 23 (17; 4–105) | 0.3 ± 0.3 (0.2; 0–1.3) | 50 ± 26 (60; 0–120) | 0.6 ± 0.4 (0.7; 0–1.3) | 78 ± 25 (77; 0–143) | 1.0±0.3 (1.0; 0.1–1.9) | |
≥41 y | 1284 ± 949 (714; 350–3085) | 6 ± 11 (0; 0–45) | 27 ± 20 (17; 7–71) | 0.3 ± 0.3 (0.2; 0–1.0) | 48 ± 25 (54; 16–106) | 0.6 ± 0.3 (0.7; 0–1.2) | 83 ± 25 (77; 45–192) | 1.0±0.3 (1.0; 0.4–2.1) | |
cPKU Total | 593 ± 585 (425; 0–6096) | 16 ± 13 (13; 0–125) | 13 ± 14 (9; 0–129) | 0.4 ± 0.3 (0.3; 0–4.2) | 44 ± 23 (40; 0–120) | 1.2 ± 1.6 (1.2; 0–5.3) | 57 ± 25 (54; 0–192) | 1.7 ± 0.7 (1.5; 0–6.3) |
Age Group | Differences by Severities | Difference of Mean Values between Severities | |||||
---|---|---|---|---|---|---|---|
Phe | Natural Protein | Protein Equivalent from Protein Substitute | |||||
mg/Day | mg/kg/Day | g/Day | g/kg/Day | g/Day | g/kg/Day | ||
<2 y | HPA-cPKU mPKU-cPKU | +405 +83 | −19 +7 | +9 +2 | +0.5 +0.3 | −12 −5 | −1.7 −0.5 |
2–5 y | HPA-cPKU mPKU-cPKU | +1048 +278 | +2 +14 | +22 +6 | +1.1 +0.4 | −24 −6 | −1.7 −0.4 |
6–12 y | HPA-cPKU mPKU-cPKU | +1660 +527 | +17 +14 | +35 +11 | +1.0 +0.4 | −36 −14 | −1.1 −0.4 |
13–18 y | HPA-cPKU mPKU-cPKU | +2671 +983 | +29 +12 | +56 +20 | +0.9 +0.4 | −45 −21 | −0.8 −0.4 |
19–30 y | HPA-cPKU mPKU-cPKU | +1818 +859 | +11 +9 | +36 +17 | +0.4 +0.3 | −25 −15 | −0.5 −0.2 |
31–40 y | HPA-cPKU mPKU-cPKU | −102 +538 | −8 +5 | −3 +12 | 0 +0.2 | −3 −9 | +0.3 −0.1 |
≥41 y | HPA-cPKU mPKU-cPKU | n/a +149 | n/a +2 | n/a +6 | n/a +0.1 | n/a −6 | n/a −0.1 |
Type of Treatment (Number of Patients) | Mean ± SD (Median; Range) | |||||||
---|---|---|---|---|---|---|---|---|
Phe | Natural Protein | Protein Equivalent from Protein Substitute | Total Protein | |||||
mg/Day | mg/kg/Day | g/Day | g/kg/Day | g/Day | g/kg/Day | g/Day | g/kg/Day | |
BH4 (n = 222) | 1649 ± 919 (1500; 100–6774) | 50 ± 43 (24; 0–173) | 35 ± 19 (30; 5–139) | 0.9 ± 0.6 (0.7; 0.0–3.5) | 24 ± 22 (22; 0–84) | 0.6 ± 0.5 (0.5; 0–2.8) | 63 ± 23 (62; 10–139) | 1.5 ± 0.6 (1.4; 0.1–4.5) |
Diet-only (n = 1101) | 846 ± 1036 (487; 0–9009) | 29 ± 30 (32; 6–192) | 19 ± 83 (10; 0–182) | 0.5 ± 1.5 (0.4; 0–4.9) | 37 ± 25 (34; 0–120) | 1.2 ± 0.7 (1.1; 0–5.3) | 59 ± 85 (54; 0–192) | 1.7 ± 1.6 (1.6; 0–6.4) |
p-value | <0.001 | <0.001 | <0.001 | <0.001 | <0.001 | <0.001 | 0.067 | <0.001 |
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Pinto, A.; Ahring, K.; Almeida, M.F.; Ashmore, C.; Bélanger-Quintana, A.; Burlina, A.; Coşkun, T.; Daly, A.; van Dam, E.; Dursun, A.; et al. Longitudinal Dietary Intake Data in Patients with Phenylketonuria from Europe: The Impact of Age and Phenylketonuria Severity. Nutrients 2024, 16, 2909. https://doi.org/10.3390/nu16172909
Pinto A, Ahring K, Almeida MF, Ashmore C, Bélanger-Quintana A, Burlina A, Coşkun T, Daly A, van Dam E, Dursun A, et al. Longitudinal Dietary Intake Data in Patients with Phenylketonuria from Europe: The Impact of Age and Phenylketonuria Severity. Nutrients. 2024; 16(17):2909. https://doi.org/10.3390/nu16172909
Chicago/Turabian StylePinto, Alex, Kirsten Ahring, Manuela Ferreira Almeida, Catherine Ashmore, Amaya Bélanger-Quintana, Alberto Burlina, Turgay Coşkun, Anne Daly, Esther van Dam, Ali Dursun, and et al. 2024. "Longitudinal Dietary Intake Data in Patients with Phenylketonuria from Europe: The Impact of Age and Phenylketonuria Severity" Nutrients 16, no. 17: 2909. https://doi.org/10.3390/nu16172909