A retrospective study of resting energy expenditure in children hospitalized with different nutritional status.

Background: Resting energy expenditure (REE) refers to the energy consumption of the body in a resting state without skeletal muscle activity. This study aimed to examine the REE among children hospitalized with varying nutritional status. Methods: This was a retrospective study. We enrolled 109 pediatric cases that underwent indirect calorimetry (IC) and divided into four groups: mild malnutrition group (15 cases), moderate malnutrition group (30 cases), severe malnutrition group (32 cases), and obesity group (32 cases). We compared and analyzed the measured REE (mREE) using IC with the predicted REE (pREE) using five energy equations. The paired t-test was used to compare the results of two samples. Pearson analysis was used to assess the correlation between two values. The agreement analysis was performed using the Bland-Altman method. Results: There was no significant difference in mREE between the mild, moderate, and severe malnutrition groups, but each differed significantly from the obesity group. All populations exhibited significant correlation between the mREEs and all five energy equations, and the equation with the highest predictive accuracy was the Schofield equation, which achieved an accuracy of 47.7%. In subgroup analysis, there was no significant difference between mREE and pREE for each of the five equations in the mild, moderate malnutrition groups. Only the prediction result of the Liu equation was not significantly different from the mREE in the severe malnutrition group. The prediction accuracy of the Liu equation was relatively the highest (34.4%). However, in the obese group, there were significant differences in pREE and mREE between the Liu equation and Mifflin equation. Under different nutritional statuses, the results of the Bland-Altman analysis suggested that deviation values between REEs predicted by each equation and mREE were greater than ±10%. Conclusions: There were differences in REE among children with different nutritional status. The results obtained from the five predictive energy equations deviated from the IC results. When REE cannot be measured by IC, it is essential to choose an appropriate predictive energy equation based on the nutritional status of the individual.

Triiodothyronine (T3) promotes browning of white adipose through inhibition of the PI3K/AKT signalling pathway.

Obesity arises from an imbalance between energy consumption and energy expenditure, and thyroid hormone levels serve as a determinant of energy expenditure. We conducted experiments at the animal and cellular levels and combined those findings with clinical data to elucidate the role of triiodothyronine (T3) in facilitating the browning of white adipose tissue (WAT) and its underlying mechanism. The results showed (i) the impaired metabolic function of local WAT and the compensatory elevation of systemic thermogenesis in obesity; (ii) T3 treatment of white adipocytes in vitro and local WAT in vivo induced a shift towards a morphologically "brown" phenotype, accompanied by upregulation of mRNA and protein expression of browning-related and mitochondrial function markers, which suggest that T3 intervention promotes the browning of WAT; and (iii) the aforementioned processes could be modulated through inhibition of the PI3K/AKT signalling pathway; however, whether T3 affects the PI3K/AKT signalling pathway by affecting insulin signalling remains to be studied and clarified. The results of our study indicate that T3 treatment promotes browning of WAT through inhibition of the PI3K/AKT signalling pathway; these findings offer novel perspectives regarding the potential of localised therapies for addressing WAT volume in individuals with obesity.

Development of a new equation and validation of earlier resting energy expenditure predicting equations in adults living in Tehran.

Background: Predictive equations have been considered as a practical approach for estimating resting energy expenditure (REE) across multiple populations, but their accuracy for each community remains to be determined. Thus, the purposes of this study were to determine the validity of REE predictive equations and to develop a new REE predictive equation in adults living in Tehran. Methods: The study included 284 subjects (158 females) aged 18-60 years old from two cross-sectional studies conducted in Tehrani populations. Anthropometric measurements were assessed using standard protocols. REE was measured using indirect calorimetry (IC) and was estimated using preexisting equations. A new equation was also developed based on the REE from IC and variables such as age, sex, height, and weight. Measured REE was compared to new equation and preexisting predictive equations via correlation, linear regression, and Bland-Altman tests. Results: The new equation and the equations by Mifflin-St. Jeor, Livingston, Frankenfield, Nichols, Müller, and Ganpule demonstrated the best predictive value at a group level (mean percentage error=-2.2 to 2.4 %). At an individual level, the new equation and the equations by Mara, Frankenfield, Roza, Nikooyeh, and Harris & Benedict showed the greatest accuracies compared to measured REE (accuracy prediction=50-53%). Conclusion: This study highlights the importance of considering race when predicting REE. It also demonstrates that the newly developed equation is more appropriate in a clinical setting at group but not individual level. Thus, further research is needed to examine the new equation in an independent sample.

Resting energy expenditure in patients with liver cirrhosis: Indirect calorimetry vs. predictive equations.

BACKGROUND AND OBJECTIVES: The objective of our study was to explore the accuracy of previously published prediction equations in predicting resting energy expenditure (REE) in patients with liver cirrhosis (LC). We also aimed to develop a novel equation to estimate REE for Chinese patients with LC. METHODS AND STUDY DESIGN: In 90 patients with LC, the agreement between REE measured by Indirect calorimetry (IC) and predictive equations was quantified using paired T-test and visualized using a Bland-Altman Plot. Pearson correlation coefficient (R) was used to measure a linear correlation between REE measured by IC and different predictive equations. Stepwise multiple regression analysis was used to create a new REE equation. RESULTS: The estimated REEs of previous equations were underestimated against REE measured by IC (1610 ± 334 kcal). Lean body mass (LBM) was positively correlated with REE measured by IC (r = 0.723, p < 0.01). The newly derived estimation equation for REE (kcal) was 1274.3 - 209.0 * sex - 5.73 * age + 3.69 * waist circumference + 22.89 * LBM. The newly derived estimation equation was found to have a Pearson-r value of 0.765 compared with REE measured by IC. CONCLUSIONS: REE in liver cirrhosis was underestimated by using predictive equations. The new predictive equation developed by using age, sex, waist circumference, and LBM may help estimate REE in Chinese patients with LC accurately and easily.

Clarifying main nutritional aspects and resting energy expenditure in children with Smith-Magenis syndrome.

Our study aims to define resting energy expenditure (REE) and describe the main nutritional patterns in a single-center cohort of children with Smith-Magenis syndrome (SMS). REE was calculated using indirect calorimetry. Patients' metabolic status was assessed by comparing measured REE (mREE) with predictive REE (pREE). Patients also underwent multidisciplinary evaluation, anthropometric measurements and an assessment of average energy intake, using a 3-day food diary, which was reviewed by a specialized dietitian. Twenty-four patients (13 M) were included, the median age was 9 years (IC 95%, 6-14 years), 84% had 17p11.2 deletion, and 16% had RAI1 variants. REE was not reduced in SMS pediatric patients, and the mREE did not differ from the pREE. In patients with RAI1 variants (16%, n = 3/24), obesity was more prevalent than those with 17p11.2 deletion (100% vs 38%). Lower proteins intake and higher total energy intake were reported in obese and overweight patients, compared to healthy weight children. No significant difference was found between males and females in energy or macronutrient intake. CONCLUSIONS: In SMS, the onset of obesity is not explained by REE abnormalities, but dietary factors seem to be crucial. Greater concern should be addressed to patients with RAI1 variants. A better understanding of the molecular mechanisms causing obesity in SMS patients could set the basis for possible future targeted therapies. WHAT IS KNOWN: • More than 90% of SMS patients after the age of 10 are overweight or obese. WHAT IS NEW: • Onset of overweight and obesity in SMS pediatric patients is not explained by abnormal resting energy expenditure. • The development of syndrome-specific dietary guidelines for SMS patients should be of utmost relevance and are highly needed.

A combination of Citrus aurantifolia fruit rind and Theobroma cacao seed extracts supplementation enhances metabolic rates in overweight subjects: a randomized, placebo-controlled, cross-over study.

Background and objective: LN19183 is a proprietary, synergistic combination of Citrus aurantifolia fruit rind and Theobroma cacao seed extracts that increased resting energy expenditure (REE) in high-fat diet (HFD)-fed obese rats. The objective of this study was to validate the thermogenic potential of LN19183 in obese Sprague Dawley (SD) rats and to assess its clinical efficacy in a proof-of-concept, randomized, placebo-controlled, cross-over human trial. Methods: In the rat study, HFD-fed obese rats were supplemented with either HFD alone or with 45, 90, or 180 mg LN19183 per kg body weight (BW) for 28 days. In the human study, 60 overweight adults (male and female, aged 20-39 years) were randomized. Subjects took LN19183 (450 mg) or a matched placebo capsule on two consecutive days in phases one and two of the study, separated by a 10-day washout period. In each phase, on day 1, REE at pre-dose, 60-, 120-, and 180-min post-dose, and on day 2, metabolic rates at pre-dose and post-dose during and 20 min after exercise were measured using indirect calorimetry. Results: In rats, LN19183 significantly increased REE, reduced BW gain and fat masses, and increased fat and carbohydrate metabolism marker proteins including beta 3 adrenergic receptor (ß3-AR), phospho-AMP-activated protein kinase (AMPK), glucagon-like peptide-1 receptor (GLP-1R) in the liver, and serum adiponectin levels. Furthermore, LN19183-supplemented human volunteers increased (P < 0.05, vs. placebo) the metabolic rates at rest and with exercise; their fat oxidation was increased (P < 0.05, vs. placebo) at rest and 20 min post-exercise. The groups' systolic and diastolic blood pressure (BP), heart rates (HR), and safety parameters were comparable. Conclusion: These observations suggest that LN19183 is a thermogenic botanical composition with no stimulatory effects on BP and HR.

Predictive Energy Equations Inaccurately Estimate Metabolic Demands of Older Adult Trauma Patients.

INTRODUCTION: Suboptimal nutrition promotes unfavorable outcomes in trauma patients, particularly among those aged 60 and over. While many institutions employ predictive energy equations to determine patients' energy requirements, mounting evidence shows these equations inaccurately estimate caloric needs. In this pilot randomized controlled trial, we sought to quantify the discrepancy between predictive equations and indirect calorimetry (IC)-the gold standard for determining energy requirements-in the older adult trauma population. METHODS: This is a nested cohort study within a pilot randomized control trial in which 32 older adult trauma patients were randomized 3:1 to receive IC-guided nutrition delivery versus standard of care. IC requirements of patients in the intervention arm were compared to Mifflin St. Jeor (MSJ), Harris-Benedict (HB), and the American Society for Parenteral and Enteral Nutrition-Society of Critical Care Medicine (ASPEN-SCCM) predictive energy equations. RESULTS: Twenty patients underwent IC to assess measured resting energy expenditure (mREE), yielding a mean (standard deviation) mREE of 23.1 ± 4.8 kcal/kg/d. MSJ and HB gave mean predictive resting energy expenditures of 17.5 ± 2.0 and 18.5 ± 2.0 kcal/kg/d in these patients, demonstrating that IC-derived values were 32.1% and 25.0% higher, respectively. When patients were stratified by body mass index (BMI), MSJ, and HB more severely underestimated caloric requirements in individuals with BMI <30 versus BMI 30-50. While the mean mREE fell within the mean predictive resting energy expenditure range prescribed by ASPEN-SCCM equations (21.4 ± 4.1 to 26.2 ± 4.3 kcal/kg/d), individuals' IC-derived values fell within their personal range in 8 of 20 cases. CONCLUSIONS: The MSJ and HB predictive energy equations consistently and significantly underpredict metabolic demands of older adult trauma patients compared to IC and perform worse in lower BMI individuals. ASPEN-SCCM equations frequently overpredict or underpredict resting energy expenditure. While these findings should be confirmed in a larger randomized control trial, this study suggests that institutions should prioritize IC to accurately identify the metabolic demands of older trauma patients.

No effect of circulating leptin on energy metabolism in normal weight or overweight/obese lactating mothers: The case-control Breastmilk and the Link to Overweight/Obesity and Maternal diet (BLOOM) study.

BACKGROUND & AIMS: Pregnancy and lactation are associated with metabolic changes, including alterations in energy metabolism, which are closely linked to body mass and composition due to hormonal status. Therefore, the objective of this study was to investigate the energy metabolism in exclusively or predominantly breastfeeding mothers with normal weight (NW) and overweight/obesity (OW/OB) and evaluate its associations with fasting serum leptin. METHODS: This cross-sectional BLOOM study was conducted among 39 mothers (n = 19 NW, n = 20 OW/OB) in 15.5 ± 1.2 weeks of lactation. The leptin was analyzed in a blood sample using an enzyme-linked immunosorbent assay, body composition was analyzed by dual-energy X-ray absorptiometry, and resting metabolic rate (RMR) was measured by indirect calorimetry method. RESULTS: The average RMR for all groups was 1747.5 ± 281.9 kcal/d, with a statistically significant difference between groups (1932.7 ± 222.6 vs. 1550.5 ± 190.6, p < 0.001, respectively in the OW/OB and NW group). The OW/OB mothers had higher oxygen uptake (VO2) and exhaled carbon dioxide (VCO2), but not respiratory quotient (RQ), carbohydrate (CHO%) and lipid oxidation (FAT%). When analyzing correlations stratified by BMI category, we found that serum leptin was correlated with CHO% negatively, and with FAT% positively in the NW but not in OW/OB mothers. Additionally, serum leptin was a significant predictor of RMR, VCO2, VO2, CHO%, and RMR/kg of total body weight. However, after adjusting for confounders, the observed associations were no longer statistically significant (RMR: ß = 0.113, 95% CI -0.354-0.319; VO2: ß = 0.141, 95% CI -0.462-0.744; VCO2: ß = 0.238, 95% CI -0.411-0.888; CHO%: ß = -0.146, 95% CI -0.151-0.444; RMR/kg of total body weight: ß = -0.294, 95% CI -0.831-0.244). CONCLUSIONS: Our results did not support the hypothesis that leptin plays a role in regulating energy homeostasis during lactation.

Effect of Intake of Bifidobacteria and Dietary Fiber on Resting Energy Expenditure: A Randomized, Placebo-Controlled, Double-Blind, Parallel-Group Comparison Study.

Bifidobacterium animalis subsp. lactis GCL2505 in combination with inulin has been shown to have several health benefits, including an improvement in the intestinal microbiota and a reduction in human visceral fat. Previous studies have suggested that the visceral fat reduction of GCL2505 and inulin may be achieved by improving daily energy expenditure. This parallel, placebo-controlled, randomized, double-blind study was conducted to evaluate the effects of GCL2505 and inulin on resting energy expenditure (REE) in overweight or mildly obese Japanese adults (n = 44). Participants ingested 1 × 1010 colony forming units of GCL2505 and 5.0 g of inulin daily for 4 weeks. REE score at week 4 was set as the primary endpoint. At week 4, the REE score of the GCL2505 and inulin group was significantly higher than that of the placebo group, with a difference of 84.4 kcal/day. In addition, fecal bifidobacteria counts were significantly increased in the GCL2505 and inulin group. Our results indicated that the intake of GCL2505 and inulin improves energy balance, which is known to be a major factor of obesity, by modulating the microbiota in the gut. This is the first report to demonstrate the effects of probiotics and dietary fiber on REE in humans.

Weight History Correlates of Resting Energy Expenditure in Women With Bulimia Nervosa.

OBJECTIVE: Among those with bulimia nervosa, weight suppression has been associated with illness severity and treatment prognosis. Although significant weight loss is known to reduce metabolic rate, the relation between weight suppression and resting energy expenditure (REE) in bulimia nervosa has not been examined. This study tested the hypothesis of an inverse relation between weight suppression and REE in a sample of women with bulimia nervosa (N = 84). METHODS: In primary analyses, linear regressions were conducted between weight suppression and REE, corrected for fat-free mass. In follow-up, exploratory analyses, stepwise linear regressions were conducted to explore the main and interaction effects of weight history and weight suppression on REE. RESULTS: Neither traditional (TWS) nor developmental weight suppression (DWS) correlated with REE. Results from exploratory analyses, however, revealed a medium-to-large inverse relation between several weight history variables and REE (highest past weight, sr2 = 0.05; lowest postmorbid weight, sr2 = 0.07; current weight, sr2 = 0.05). Additionally, DWS interacted with current (sr2 = 0.08) and highest premorbid (sr2 = 0.05) z-BMI to influence REE with a medium-to-large effect. For individuals low in current and premorbid z-BMIs, higher DWS associated with lower REE levels. However, for individuals at higher premorbid z-BMIs, higher DWS unexpectedly associated with greater REE levels. DISCUSSION: In this sample of women with bulimia nervosa, reduced REE associated with higher weights across all timepoints. If the interaction effect between DWS and z-BMI history persists in future studies, this may indicate unique challenges faced by individuals low in z-BMI and high in DWS related to weight gain and normalization of eating.

Methods of Determining Energy Expenditure in Individuals with Prader-Willi Syndrome: A Systematic Literature Review.

Prader-Willi syndrome (PWS) is a rare disorder characterised by varying nutritional phases that occur throughout the lifespan, ranging from failure to thrive to hyperphagia. If uncontrolled, the imbalance between energy intake and expenditure results in obesity development and increased morbidity and mortality risk. Although measures of energy requirements for accurate nutrition assessment are vital, the evidence appears sparse and heterogeneous; hence, the aim of this review was to examine the available literature on energy expenditure predicted or measured using various methods in individuals with PWS. Studies were sought that presented methods and results on resting energy expenditure or basal metabolic rate. A narrative synthesis was completed to present the study characteristics and results. Methods of determining energy requirements included predictive equations and indirect calorimetry. Differences amongst ages, growth hormone therapy, fasting status, and measures in which results were presented were limitations to appropriately summarising and identifying trends in energy expenditure. Indirect calorimetry was identified as the most accurate method; however, it is not widely available in all settings. Further research is encouraged to support the development of valid and reliable predictive equations that will better inform and improve the efficiency of clinical practice in supporting people with PWS.

Resting energy expenditure in women with polycystic ovary syndrome.

STUDY QUESTION: Is resting energy expenditure (REE) altered in women with polycystic ovary syndrome (PCOS)? SUMMARY ANSWER: Women with PCOS have a reduction in REE, when corrected for fat-free mass, independent of PCOS clinical phenotypes and BMI categories. WHAT IS KNOWN ALREADY: Obesity is an important issue in women with PCOS, in terms of frequency and pathophysiological implications. It has been hypothesized that obesity may be favoured by alterations in REE, but the studies have been limited and conflicting. STUDY DESIGN, SIZE, DURATION: This case-control study was a comparison of 266 women with PCOS and 51 healthy controls, recruited in the Verona 3P study from 2010 to 2021. PARTICIPANTS/MATERIALS, SETTING, METHODS: Women with PCOS diagnosed by the Rotterdam criteria, with normal thyroid function and no interfering medications, were referred to the outpatient clinic of a tertiary care centre of endocrinology and metabolism for a measurement of REE. Healthy controls were recruited in the same period and submitted to the same procedure. In all subjects, REE was measured by indirect calorimetry and serum androgens were measured by LC-MS/MS. In women with PCOS, insulin sensitivity was assessed using the hyperinsulinemic-euglycemic clamp. MAIN RESULTS AND THE ROLE OF CHANCE: REE was similar in women with PCOS and controls. However, REE corrected for fat-free mass (REE/FFM) was significantly lower in women with PCOS than in controls (31.8 ± 4.0 vs 35.4 ± 3.9 kcal/kgFFM·day, P < 0.001). REE/FFM did not differ between normal-weight, overweight, or obese women with PCOS, and each of these subgroups showed lower REE/FFM values than controls. Reduced REE/FFM values were found in each phenotype of the syndrome. In multiple regression analysis, REE/FFM was independently associated with age and PCOS status, but not with fat mass. In PCOS women, REE/FFM was independently and directly associated with ovarian follicle number. LIMITATIONS, REASONS FOR CAUTION: Limitations of the study are the cross-sectional design, which limits the causal inference of the results, and the unavailability of precise information about lifestyle factors, which may be potential confounders. Further prospective studies are needed to establish the importance of this phenomenon in contributing to the weight excess of PCOS. WIDER IMPLICATIONS OF THE FINDINGS: A reduction of REE could potentially favour weight gain in women with PCOS and possibly contribute to the altered metabolic profile typical of this condition, even counteracting the therapeutic strategies aimed to reduce excess body fat in these women. Nevertheless, the presence of this abnormality in both obese/overweight and normal-weight patients suggests that other factors must play a role in this phenomenon. STUDY FUNDING/COMPETING INTEREST(S): This work was supported by academic grants to PM from the University of Verona (FUR 2010-2022). All authors declare no conflict of interest. TRIAL REGISTRATION NUMBER: N/A.

Measurement of Energy Expenditure by Indirect Calorimetry with a Whole-Room Calorimeter.

Energy plays a vital role in biological processes. To assess energy metabolism status in a large population cohort, the standard operating procedure for measuring energy expenditure measurement using a whole-room calorimeter was purposed in this study. This protocol illustrates the procedure and specific details for validating methanol burning and evaluating the metabolic status of volunteers. In metabolic status evaluation, the (1) O2 consumption, (2) CO2 production, (3) energy expenditure, and (4) respiratory exchange ratio were first measured at resting and provided as basic phenotype items in Human Phenotype Atlas. Besides, it includes the procedure and results for measuring exercise-related activity thermogenesis and evaluating the impact of environmental temperature on energy metabolism. These results demonstrate the broader utility of the whole-room calorimeter. The implementation of this protocol is expected to enhance the data comparability in Human Phenotype Atla and provide a valuable reference for metabolism-related studies.

Resting Energy Expenditure, comparison of predictive formulas with bioimpedance: Peruvian population / Gasto Energético en Reposo, comparación de fórmulas predictivas con la bioimpedancia: población peruana

Introduction: In areas with limited access to healthcare systems, Resting Energy Expenditure (REE) estimation is performed using predictive equations to calculate an individual's caloric requirement. One problem is that these equations were validated in populations with different characteristics from those in Latin America, such as race, height, or body mass, leading to potential errors in the prediction of this parameter. Objective: To determine the REE using predictive formulas compared with bioimpedance in Peruvians. Materials and methods: A comparative analytical cross-sectional study with secondary database analysis of the CRONICAS cohort. Results: we worked with a total of 666 subjects. The Mjeor equation was the one with the highest rating of 0.95, a lower mean absolute percentage error (MAPE) of 4.69%, and equivalence was found with the REE values. In the multiple regression, it was observed that the Mjeor equation was the one that least overestimated the REE, increasing 0.77 Kcal/day (95% CI: 0.769-0.814; p<0.001) for each point that increased the REE determined by bioimpedance. The strength of association between Mjeor and bioimpedance was 0.9037. Furthermore, in the regression of the data (weight, height, age) in the Mjeor equation it was observed that the coefficients obtained were the same as those used in the original equation. Conclusions: The Mjeor equation seems to be the most adequate to estimate the REE in the Peruvian population. Future prospective studies should confirm the usefulness of this formula with potential utility in primary health care(AU)

Introducción: En zonas con acceso limitado a sistemas de salud, la estimación del Gasto Energético en Reposo (GER) se realiza utilizando ecuaciones predictivas para calcular el requerimiento calórico de un individuo. Uno de los problemas es que estas ecuaciones fueron validadas en poblaciones con características diferentes a las latinoamericanas, como raza, talla o masa corporal, lo que conlleva a potenciales errores en la predicción de este parámetro. Objetivo: Determinar el GER mediante fórmulas predictivas comparadas con la bioimpedancia en peruanos. Materiales y métodos: Estudio transversal analítico comparativo con análisis secundario de base de datos de la cohorte CRONICAS. Resultados: Se trabajó con un total de 666 sujetos. La ecuación de Mjeor fue la que obtuvo la puntuación más alta de 0,95, un error medio porcentual absoluto (MAPE) inferior de 4,69%, y se encontró equivalencia con los valores del GER. En la regresión múltiple, se observó que la ecuación de Mjeor fue la que menos sobreestimó el GER, aumentando 0,77 Kcal/día (IC 95%: 0,769-0,814; p<0,001) por cada punto que aumentaba el GER determinado por bioimpedancia. La fuerza de asociación entre Mjeor y bioimpedancia fue de 0,9037. Además, en la regresión de los datos (peso, talla, edad) de la ecuación de Mjeor se observó que los coeficientes obtenidos eran los mismos que los utilizados en la ecuación original. Conclusiones: La ecuación de Mjeor parece ser la más adecuada para estimar el GER en la población peruana. Futuros estudios prospectivos deberán confirmar la utilidad de esta fórmula para su potencial utilidad en la atención primaria de salud(AU)

Impact of lumacaftor/ivacaftor on nutrition and growth in modulator-naïve children over 24 weeks.

BACKGROUND: Cystic fibrosis transmembrane conductance regulator (CFTR) modulators improve nutritional status and are of importance in achieving normal growth among younger children with CF. The study was designed to examine CFTR modulator-associated changes in nutrition status, including bile acids and fatty acids after lumacaftor/ivacaftor therapy for 24 weeks. METHODS: Children 2 to 5.9 years were recruited from US and Canadian CF Centers. Eligible children were lumacaftor/ivacaftor naïve and approved to initiate therapy. Anthropometrics, diet, energy expenditure, nutrition biomarkers, pancreatic status, serum and fecal calprotectin, serum bile acids and plasma fatty acids were measured. Changes from baseline at 12 and 24 weeks were examined using mixed effects linear regression modeling. RESULTS: Weight-for-age z-score (WAZ) increased at 12 (0.15 ± 0.1, p = 0.01) and 24 weeks (0.23 ± 0.1, p = 0.001) from baseline following modulator therapy. Head circumference-for-age (HCZ) increased at 12 weeks compared to baseline (0.22 ± 0.1, p = 0.03) and subscapular Z score increased from baseline at 24 weeks following therapy (0.33 ± 0.1, p = 0.02). There were no changes in energy expenditure. Serum total bile acids (6.7 ± 2.0, p = 0.001), chenodeoxycholic acid (CDCA) (2.4 ± 1.1, p = 0.001), and cholic acid (CA) (3.5 ± 0.8, p < 0.0001) increased at 24 weeks compared to baseline. Fecal calprotectin decreased at 12 and 24 weeks compared to baseline (-463 ± 310, p = 0.03 and 566 ± 347, p = 0.047). A number of plasma fatty acids changed over the course of 24 weeks of therapy. Noteably, alpha-linolenic acid (ALA) decreased at 12 and 24 weeks (-24 ± 10,p = 0.03 and -18 ± 10, p = 0.02, respectively). CONCLUSIONS: Overall, young children experienced favorable changes in nutritional and growth, with the exception of plasma ALA status in the first 24 weeks of lumacaftor/ivacaftor therapy.

Effect of nutritional therapy in Emery-Dreifuss muscular dystrophy: a case report.

Emery-Dreifuss muscular dystrophy (EDMD) is a rare, inherited human disease. Similar to other neuromuscular dystrophies, EDMD is clinically characterized by muscle atrophy and weakness, multi-joint contractures with spine rigidity, and cardiomyopathy. Over time, muscular weakness can lead to dysphagia and a severe lowering of body mass index (BMI), worsening the prognosis. We present the case of a young male patient affected by EDMD, admitted to the hospital for pneumothorax in a severe state of undernourishment. The patient was treated with total parenteral nutrition (TPN) with Smofkabiven®, supplemented with micronutrients (vitamins and trace elements), and with minimal enteral nutrition through food. Within a year, the patient gained 8.5 kg and kept his body weight stable for the 6 years of the follow-up. In this study, we show that TPN ensures the nutritional requirements of EDMD patients in a safe and well-tolerated manner, allowing a considerable and stable improvement in nutritional status, which has a positive impact on the disease itself and the patients' quality of life.

The recent WHO guideline on acute malnutrition overestimates therapeutic energy requirement.

The World Health Organization has recently updated the guideline on the prevention and management of wasting and nutritional oedema (acute malnutrition) in infants and children under 5 years. Apart from differences with regard to the nutritional framework that defines the quantity of energy required as Ready-to-Use Therapeutic Food (RUTF) for the outpatient treatment of severe wasting and/or nutritional oedema, there are also important gaps in the practical guidance. Instead of the recommended energy intake of 150-185 kcal/kg/day, our alternative calculations indicate the requirement to be only 105-120 kcal/kg/day. If true, the implementation of such caloric overfeeding can have adverse consequences. Gaps in practical guidance also need to be addressed, including the timing of transition to home-based diets, maximal duration of therapeutic feeding, especially in non-responders (∼50% in South Asia), and the role of augmented home foods as the primary therapeutic food option.

Energy expenditure during nutritional rehabilitation: a scoping review to investigate hypermetabolism in individuals with anorexia nervosa.

BACKGROUND: Weight gain and nutritional rehabilitation are essential first steps to achieve medical stabilization in anorexia nervosa, and frequent resistance to weight gain requires patients to consume high kilocalorie loads. Adaptive hypometabolism is common when patients begin treatment, and rebound hypermetabolism is suspected to be a significant barrier to weight gain. The aim of this review was to summarize existing data describing metabolic changes in anorexia nervosa during weight restoration. The reported findings challenge current hypotheses of weight gain resistance and highlight key areas for future research. METHODS: Using scoping review guidelines, three databases were searched for studies investigating metabolic changes in anorexia nervosa before and after renourishment. Two reviewers systematically screened the titles and abstracts of 447 articles, and full-text versions of 106 studies were assessed for eligibility. A total of 36 studies were included for review. Data regarding the study description, sample population (including age, weight, BMI, duration of treatment, and caloric intake), and metabolic variable descriptions were extracted. RESULTS: Female patients with anorexia nervosa from studies across 13 countries were included. Across the studies, average BMI increased from 13.7 kg/m2 at admission to 17.57 kg/m2. Patients presented to treatment with clinically reduced energy expenditure levels. After varying levels of nutritional rehabilitation and weight restoration, measured energy expenditure increased significantly in 76% of the studies. Energy expenditure values at the second timepoint increased to the standard range for normal weight female teenagers and adults. Despite these increases, the studies do not indicate the presence of a hypermetabolic state during renourishment. Additionally, all studies including both measured and predicted energy expenditure reported that predicted energy expenditure overestimated measured values. CONCLUSION: This study provides a detailed evaluation of the literature investigating energy expenditure and metabolic rate in patients with anorexia nervosa before and following a period of renourishment. The findings from this review identify important gaps in the current beliefs of energy expenditure in anorexia nervosa and highlight a need for further exploration of metabolic alterations during weight restoration.

Nutritional rehabilitation and weight restoration are two primary goals of anorexia nervosa treatment that pose significant physiological and psychological challenges for patients. Patients often require high caloric loads to continue an adequate weight gain trajectory, but the underlying cause of weight gain resistance remains unknown. We completed a scoping review of research into energy expenditure and metabolic rate during treatment. Our search identified 447 relevant articles from academic databases, and 106 were deemed eligible after screening. We extracted data, including sample characteristics, kilocalorie intake, energy expenditure, and treatment information, from 36 studies. When individuals arrived for treatment, their energy expenditure was lower than that of individuals without an eating disorder due to the prolonged state of nutrient deprivation. After varying amounts of time and kilocalorie intake, most studies reported significant increases in energy expenditure. However, energy expenditure after a period of renourishment did not indicate an overactive metabolism (i.e., "hypermetabolism"). Funders should consider supporting exploration of additional factors that may be functioning as barriers to weight gain during treatment, in pursuit of making treatment more efficient and long-lasting. Additionally, future research describing metabolism in anorexia nervosa should provide more consistent methodologies, robust statical testing, and comprehensive reporting of dietary intake.

The effects of dietary macronutrient composition on resting energy expenditure following active weight loss: A systematic review and meta-analysis.

A systematic review and meta-analysis was conducted to evaluate the relative effectiveness of different dietary macronutrient patterns on changes in resting energy expenditure (REE) in relation to weight loss, categorized as minimal (<5%) and moderate to high (>5%). Changes in REE were assessed using a DerSimonian and Laird random-effects meta-analysis. A diet lower in carbohydrates (CHO) or higher in fat and protein was associated with smaller reductions in REE, with these trends being more pronounced among participants who experienced moderate to high weight loss. Adjusted meta-regression analysis indicated that, within the participants who experienced moderate to high weight loss, each 1% increase in CHO intake was associated with a reduction of 2.30 kcal/day in REE (95% CI: -4.11 to -0.47, p = 0.013). In contrast, a 1% increase in protein and fat intake was correlated with an increase in REE by 3.00 (95% confidence interval [CI] [1.02, 5.07], p = 0.003) and 0.5 (95% CI [-2.43, 3.41], p = 0.740) kcal/day, respectively. No significant associations were found among participants who experienced minimal weight loss. These findings indicate that, under a caloric deficit, the impact of dietary macronutrient composition on REE may vary depending on the degree of weight loss and individual metabolic responses.