TABLE 2.
Summary of studies investigating differences in the components of energy expenditure in individuals with or without PWS1
| Author, year (ref.) | Study design and purpose | Study population (mean ± SD) | Measurements | Outcomes |
| Schoeller et al., 1988 (5) | Cross-sectional study comparing TEE, BMR, and BC | PWS, n = 10, 5 F/5 M | TEE: DLW, 7 d | TEE compared with controls: |
| Age: 16 ± 4 y | BEE: IC ventilated hood, 30 min | 47% lower in PWS subjects | ||
| BMI, kg/m2: 28.9 ± 8.1 (PWS lower than controls) | AEE: estimated based on predictive equation that includes FFM | BEE compared with predictive equations: | ||
| FFM, kg: 29.8 ± 9.6 (PWS lower than controls) | BC: total body water | |||
| FM, %: 48 ± 7 | Measured 3–12% lower than predictive equations. Predictive equation that included FFM not different from measured BMR | |||
| GH treatment: no information | Estimated AEE compared with controls: | |||
| PWS subtype: 5 DEL/5 not reported | 66% lower in PWS subjects; no difference per kg BW | |||
| PWS for BMR, n = 6, 3 F/3 M | ||||
| Age: 24 ± 4 y | ||||
| BMI: 22.7 ± 1.8 | ||||
| PWS subtype: not reported | ||||
| Controls, n = 10; 5 F/5 M | ||||
| Age: 15 ± 3.6 y | ||||
| BMI: 36.3 ± 7.5 | ||||
| FFM, kg: 56.4 ± 13.8 | ||||
| FM, %: 45 ± 6 | ||||
| Davies and Joughin, 1993 (13) | Cross-sectional study to assess TEE and REE | PWS, n = 10, 5 F/5 M | TEE: DLW, details not provided | TEE compared with controls: |
| Age, y: 11.65 ± 3.5 | REE: IC hood, 20 min | 28% lower in PWS subjects, unadjusted; no difference with adjustment for age, FFM, and sex | ||
| BMI: not reported | AEE: TEE/REE | REE compared with controls: | ||
| FFM, kg: 23.9 ± 10.3 | BC: total body water | 18% lower in PWS subjects, unadjusted; no difference with adjustment for age, FFM, and sex | ||
| FM, %: 43 ± 10.5 | Estimated AEE compared with controls: | |||
| GH treatment: no information | 13% lower in PWS subjects, unadjusted; lower with adjustment for age | |||
| PWS subtype: 7 DEL/8 not reported | ||||
| Controls, n = 60, M/F: no information | ||||
| Age, y: 12.56 ± 4.2 | ||||
| BMI: not reported | ||||
| FFM, kg: 34.2 ± 15.1 kg | ||||
| FM, %: 23.8 ± 9 (controls lower than PWS) | ||||
| van Mil et al., 2000 (14) | Cross-sectional study to measure AEE, corrected for body size | PWS, n = 17, 10 F/7 M | TEE: DLW, 14 d | Absolute TEE compared with controls: |
| Age, y: 11.9 ± 3.4 | BEE: ventilated hood, 10 min | 28% lower in PWS subjects, unadjusted | ||
| BMI: 23.5 ± 6 | AEE: 0.9 × (TEE – BEE), correcting for 10% DIT | TEE/FFM (per kg FFM): | ||
| FFM, kg: 27.5 ± 9.9 (PWS lower than controls) | BC: total body water | 42% and 38% lower in PWS subjects with BW and BW covariates, respectively | ||
| FM, kg: 22.4 ± 11.7 | BEE compared with controls: | |||
| FM, %: 43.7 ± 7.9 | 16% lower in PWS subjects, unadjusted; 21% lower with BW as a covariate; no difference with FFM as a covariate | |||
| Bone age, y: 12.7 ± 2.9 | AEE compared with controls: | |||
| PWS subtype: DEL or UPD | 58% lower for PWS subjects unadjusted; 50% lower with BW as a covariate for PWS subjects adjusting for BW/d | |||
| GH: none ever received GH | ||||
| Controls, n = 17; 10 F/7 M | ||||
| Age, y: 11.3 ± 2.6 | ||||
| BMI: 26 ± 6.5 | ||||
| FFM, kg: 35.9 ± 13.4 | ||||
| FM, kg: 25.6 ± 12.7 | ||||
| FM, %: 39.1 ± 8.8 | ||||
| Bone age, y: 12.7 ± 3.2 | ||||
| Bekx et al., 2003 (1) | Cross-sectional study to evaluate BC in relation to energy expenditure in infants and toddlers with PWS | PWS, n = 16, 8 F/8 M | TEE: DLW, 7 d | Absolute TEE compared with published normative data: |
| Age, mo: 12.4 ± 6 | BC: DXA, DLW | 24% lower in PWS | ||
| DXA FFM, kg: 5.7 ± 1.4 | TEE adjusted as linear regression for FFM (kg FFM): | |||
| FM, %: 28.8 ± 6 | Rates for PWS subjects comparable with published normative data | |||
| DLW FM, %: 34.9 ± 7 | ||||
| PWS subtype: 10 DEL/5 UPD/ 1 ID | ||||
| GH: 14 current GH | ||||
| Butler et al., 2007 (15) | Cross-sectional study to determine the relations among body composition, activity amounts, and metabolic rates | PWS, n = 48; 27 F/21 M | TEE: whole body IC, 8 h | TEE compared with controls: 20% lower in PWS subjects, kcal/8 h, unadjusted; lower with adjustment for FM; no difference with adjustment for LBM |
| Age, y: 23 ± 9 | REE: whole body IC, 30 min | REE compared with controls: | ||
| BMI: 34 ± 9 (PWS lower than controls) | AEE: energy expended during the periods when the participants were instructed and encouraged to do exercises | 16% lower in PWS subjects, kcal/min; lower with adjustment for FM; no difference with adjustments for LBM or BW | ||
| LBM, kg: 35 ± 7 (PWS lower than controls) | BC: DXA | AEE compared with controls: | ||
| FM, kg: 39 ± 14 (PWS lower than controls) | 38% lower in PWS subjects, kcal/min, with a 35% reduction in mechanical work >8 h | |||
| FM, %: 51 ± 8 | ||||
| PWS subtype: 27 DEL/21 UPD | ||||
| GH: none receiving GH | ||||
| Controls, n = 24; 15 F/9 M | ||||
| Age, y: 27 ± 13 | ||||
| BMI: 41 ± 8 | ||||
| LBM, kg: 51 ± 12 | ||||
| FM, kg: 50 ± 14 | ||||
| FM, %: 50 ± 7 | ||||
| Hill et al., 1990 (16) | Cross-sectional study to determine whether the relation between REE and BW or BC is different in individuals with PWS | PWS, n = 22; 13 F/9 M | REE: IC hood, 15–20 min | REE compared with controls: |
| Age, y: 13 ± 1 | BC: BIA | 37% lower in PWS subjects than in obese controls and 20% lower than in lean controls, unadjusted, no difference with adjustment for FFM | ||
| BMI: 24 ± 1 | ||||
| PWS subtype and GH: no information | ||||
| Obese controls, n = 11; 7 F/4 M | ||||
| Age, y: 10 ± 1 | ||||
| BMI: 28 ± 1 | ||||
| Lean controls n = 20; 9 F/11 M | ||||
| Age 10 ± 1 y | ||||
| BMI 19 ± 1 | ||||
| FM and FFM data not reported. | ||||
| van Mil et al., 2000 (17) | Cross-sectional study to measure SEE, adjusted for FFM | Same participants as van Mil et al. (14) | SEE: respiratory camper, overnight | Absolute SEE compared with controls: |
| 17% lower in PWS subjects, unadjusted; lower with adjustment for BW; no difference with adjustment for FFM | ||||
| Goldstone et al., 2002 (18) | Cross-sectional study to assess REE | PWS, n = 8 F | REE: whole body IC, ≥20 min | Absolute REE compared with controls: |
| Age, y: 25 ± 2 | BC: MRI | 8% lower in PWS subjects than in obese controls, unadjusted; lower after adjustment for age, height, and weight and after adjustment for age and FM; higher after adjustment for age and FFM; no difference after adjustment for age, FFM, and FM | ||
| BMI: 42.1 ± 3 (higher than obese and lean controls) | ||||
| FFM, kg: 46.1 ± 3.7 (lower than obese controls) | ||||
| FM, kg: 49.6 ± 6.3 (higher than obese and lean controls) | ||||
| FM, %: 50.8 ± 2 | ||||
| PWS subtype: not reported | ||||
| GH: none receiving GH | ||||
| Obese controls, n = 13 F | ||||
| Age, y: 26 ± 3 | ||||
| BMI: 37.1 ± 1.6 | ||||
| FFM, kg: 58.5 ± 1.8 | ||||
| FM, kg: 43.2 ± 2.9 | ||||
| FM, %: 42.1 ± 1.2 | ||||
| Lean controls, n = 28 F | ||||
| Age, y: 31 ± 1 | ||||
| BMI: 23.7 ± 0.5 | ||||
| FFM, kg: 46.1 ± 0.9 | ||||
| FM, kg: 19.5 ± 0.9 | ||||
| FM, %: 29.1 ± 0.8 | ||||
| Lloret-Linares et al., 2013 (19) | Cross-sectional study to compare REE of adults with PWS or lesional genetic hypothalamic obesity with obese controls | PWS, n = 16 F | REE: IC hood, details not provided | Absolute REE compared with controls: |
| Age, y: 25.8 ± 4.9 | BC: DXA | Female PWS subjects had rates similar to controls and subjects with HD, unadjusted; when adjusted for age, PWS subjects had 13% higher rates than controls and similar rates to HD subjects when adjusted for LBM | ||
| BMI: 46.6 ± 8.5 | Male PWS subjects had 19% lower rates than controls and similar rates to HD subjects, unadjusted; no differences when adjusted for age and LBM | |||
| LBM, kg: 46.7 ± 10 (*lower than females with HD) | ||||
| FM, kg: 53 ± 13.2 | ||||
| FM, %: 52.5 ± 4 | ||||
| PWS, n = 11 M | ||||
| Age, y: 25.5 ± 10.4 | ||||
| BMI: 46.6 ± 9.1 | ||||
| LBM, kg: 58.6 ± 12.6 (*lower than male HD and control subjects) | ||||
| FM, kg: 58.6 ± 19.2 | ||||
| FM, %: 48.9 ± 7.3 | ||||
| PWS subtype (all subjects): 18 DEL/8 UPD/1 ID | ||||
| GH (all subjects): 1 current, 5 in childhood | ||||
| HD, n = 5 F | ||||
| Age, y: 42.2 ± 16.1 | ||||
| BMI: 49 ± 7.6 | ||||
| LBM, kg: 61.7 ± 11.7 | ||||
| FM, kg: 57.9 ± 13.4 | ||||
| FM, %: 47 ± 1.6 | ||||
| HD, n = 10 M | ||||
| Age, y: 33.8 ± 13.2 | ||||
| BMI: 47.2 ± 7.7 | ||||
| LBM, kg: 75 ± 12.3 | ||||
| FM, kg: 58 ± 14 | ||||
| FM, %: 40 ± 7.4 | ||||
| GH (all HD subjects): 1 current, 5 in childhood | ||||
| Controls, n = 176 F | ||||
| Age, y: 36 ± 8.4 | ||||
| BMI: 46.8 ± 6.3 | ||||
| LBM, kg: 61.7 ± 8.3 | ||||
| FM, kg: 60.5 ± 12.3 | ||||
| FM, %: 47.5 ± 4.5 | ||||
| Controls, n = 30 M | ||||
| Age, y: 41.7 ± 7.9 | ||||
| BMI: 46.3 ± 5.7 | ||||
| LBM, kg: 78.8 ± 8.3 | ||||
| FM, kg: 58.5 ± 14 FM, %: 40.4 ± 4.6 | ||||
| Purtell et al., 2015 (20) | Cross-sectional study to measure changes in energy expenditure in response to meals | PWS, n = 11, 4 F/7 M | REE: IC hood, 30 min | Absolute REE compared with controls: |
| Age, y: 27.5 ± 2.7 | Postprandial REE: IC hood, 240 min | Rates for PWS subjects were comparable with those of obese and lean controls, unadjusted; no differences when adjusted for FFM and FM | ||
| BMI: 37.35 ± 2.9 (*higher than controls) | BC: DXA | Absolute postprandial REE compared with controls | ||
| LBM, kg: 43.21 (*lower than obese controls) | Rates for PWS subjects were comparable with those of obese and lean controls, unadjusted; no differences when adjusted for FFM and FM | |||
| FM, kg: 3.06 | ||||
| FM, %: 47.68 | ||||
| PWS subtype: 6 DEL/5 UPD | ||||
| GH: none ever received GH Obese controls, n = 12; 5 F/7 M Age, y: 32.25 ± 2.5 BMI: 34.21 ± 1.2 LBM, kg: 52.67 FM, kg: 40.33 FM, %: 46.25 |
||||
| Lean controls, n = 10; 5 F/5 M | ||||
| Age, y: 28.8 ± 1.1 | ||||
| BMI: 21.4 ± 0.4 | ||||
| LBM, kg: 71.54 | ||||
| FM, kg: 14.63 FM, %: 24.32 |
1
AEE, BEE, REE, and TEE values are expressed as kcal/d unless otherwise indicated. AEE, activity energy expenditure; BC, body composition; BEE, basal energy expenditure; BIA, bioelectrical impedance analysis; BMR, basal metabolic rate; BW, body weight; DEL, deletion; DLW, doubly labeled water; F, female; FFM, fat-free mass; FM, fat mass; GH, growth hormone; HD, hypothalamic damage; IC, indirect calorimetry; ID, imprinting center defects, LBM, lean body mass; M, male; PWS, Prader-Willi syndrome; REE, resting energy expenditure; ref., reference; SEE, sleeping energy expenditure; TEE, total energy expenditure; UPD, uniparental disomy.