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Background/Objectives: Brown adipose tissue (BAT) activation has important metabolic health implications, yet the relationship between habitual dietary intake and BAT activity in humans remains to be fully understood. Methods: We compared dietary intake among adult men with (BATpositive, age: 34.8 ± 5.4 years, BMI: 28.2 ± 5.3 kg/m2, n = 12) and without (BATnegative, age: 39.1 ± 4.1 years, BMI: 31.1 ± 6.7 kg/m2, n = 11) cold-induced BAT activation. Activation of BAT was measured immediately following 2 h of cold exposure using 18F fluorodeoxyglucose positron emission tomography and computed tomography reported as maximum standardized uptake (SUVmax). Participants categorized as BATpositive had an SUVmax > 1.5 g/mL that was normalized to lean body mass (SUVlean) for analysis. Shivering intensity was recorded every 15 min during cold exposure and dietary intake was estimated from 7 consecutive 24 h dietary recalls. Results: The BATnegative group was significantly older than the BATpositive group (p = 0.046). Although BATnegative participants consumed an average of 281.2 kcal/day more than BATpositive, there were no significant differences in dietary intake between groups (p ≥ 0.202). Further, no statistically significant associations between SUVlean and dietary intake among BATpositive participants were observed (p ≥ 0.175). Participants who shivered (n = 9) during cold exposure tended to be shorter (p = 0.056) and have a lower waist-to-hip ratio (p = 0.097) but did not differ in dietary intake (p ≥ 0.204) or BAT activity (p = 0.964) when compared to the non-shivering (n = 11) group. Conclusions: Our results indicate that BAT activity and shivering during cold exposure are more strongly related to variables such as age and body size or composition rather than habitual dietary intake. We conclude that habitual dietary intake likely has a negligible influence on BAT activity among adult men. [ABSTRACT FROM AUTHOR]

Pompe disease is a rare autosomal recessive disorder caused by the deficiency of acid α-glycosidase resulting in accumulation of glycogen in the lysosomes. The late-onset form of the disease (LOPD) causes primarily progressive muscle weakness and respiratory insufficiency. Enzyme replacement therapy (ERT) introduced in 2006, showed mild improvement or stabilization of the symptoms although long-term data are limited. Aim of the study was to describe the progression of body composition and walking ability in LOPD patients receiving ERT consistently for 9 years. Lean body mass, bone mineral density, body fat and 6 min walking distance were assessed in three male and three female LOPD patients (height 165.8 ± 11.2 cm, age 42.3 ± 11.8yrs, body mass 71.1 ± 20.8 kg, at study entry), every three years, for 9 years since ERT initiation (T0, T3, T6, T9). Total body and upper extremities' lean mass remained unchanged (p < 0.05), but it was decreased for the lower extremities (T3:13.06 ± 3.848 kg vs. T9:11.63 ± 3.49 kg, p < 0.05). Lean body mass was not significantly different after 9 years of ERT compared to before the ERT initiation (T0 to T9). Bone mineral density remained unchanged. Percent body fat increased (T0:39.1 ± 10.3%, vs. T9:43.1 ± 10.4%, p < 0.05). Six minute walking distance tended to increase after 3 years of ERT and decreased gradually thereafter, with no difference between T0-T9. Lean mass of the lower extremities adjusted for body weight was significantly correlated with 6 min walking distance (r = 0.712, p < 0.05). The current data show that enzyme replacement therapy may preserve lean body mass, bone mineral density and walking capacity in LOPD patients. [ABSTRACT FROM AUTHOR]

Byline: Argyro. Krase, Giorgos. Papadimas, Gerasimos. Terzis Sir, Pompe disease is a rare inherited autosomal recessive disorder caused by the partial or total deficiency of acid a-glucosidase, resulting in lysosomal [...]

White adipose tissue (WAT) thermogenic activity may play a role in whole-body energy balance and two of its main regulators are thought to be environmental temperature (Tenv) and exercise. Low Tenv may increase uncoupling protein one (UCP1; the main biomarker of thermogenic activity) in WAT to regulate body temperature. On the other hand, exercise may stimulate UCP1 in WAT, which is thought to alter body weight regulation. However, our understanding of the roles (if any) of Tenv and exercise in WAT thermogenic activity remains incomplete. Our aim was to examine the impacts of low Tenv and exercise on WAT thermogenic activity, which may alter energy homeostasis and body weight regulation. We conducted a series of four experimental studies, supported by two systematic reviews and meta-analyses. We found increased UCP1 mRNA (p = 0.03; but not protein level) in human WAT biopsy samples collected during the cold part of the year, a finding supported by a systematic review and meta-analysis (PROSPERO review protocol: CRD42019120116). Additional clinical trials (NCT04037371; NCT04037410) using Positron Emission Tomography/Computed Tomography (PET/CT) revealed no impact of low Tenv on human WAT thermogenic activity (p > 0.05). Furthermore, we found no effects of exercise on UCP1 mRNA or protein levels (p > 0.05) in WAT biopsy samples from a human randomized controlled trial (Clinical trial: NCT04039685), a finding supported by systematic review and meta-analytic data (PROSPERO review protocol: CRD42019120213). Taken together, the present experimental and meta-analytic findings of UCP1 and SUVmax, demonstrate that cold and exercise may play insignificant roles in human WAT thermogenic activity. Abbreviations: WAT:White adipose tissue; Tenv: Environmental temperature; UCP1: Uncoupling protein one; BAT: Brown adipose tissue; BMI:Body mass index; mRNA: Messenger ribonucleic acid; RCT: Randomized controlled trial; WHR: Waist-to-hip ratio; PRISMA: Preferred Reporting Items for Systematic Reviews and Meta-analyses; PET/CT: Positron Emission Tomography and Computed Tomography; REE: Resting energy expenditure; 18F-FDG: F18 fludeoxyglucose; VO2peak:Peak oxygen consumption; 1RM: One repetition maximum; SUVmax: Maximum standardized uptake value; Std: Standardized mean difference [ABSTRACT FROM AUTHOR]