ddPCR enables rapid detection of bloodstream infections in patients on home parenteral nutrition: A prospective cohort study.

INTRODUCTION: Chronic intestinal failure patients (CIF) require a central venous access device (CVAD) to administer parenteral nutrition. Most serious complication related to a CVAD is a central line-associated bloodstream infection (CLABSI). The golden standard to diagnose a CLABSI are blood cultures, however, they may require 1-5 days before getting a result. Droplet digital polymerase chain reaction (ddPCR) for the detection of pathogen 16S/28S rRNA is a novel culture-independent molecular technique that has been developed to enhance and expedite infection diagnostics within two and a half hours. In this study, we prospectively compared ddPCR with blood cultures to detect pathogens in whole blood. METHODS: We included adult CIF patients with a clinical suspicion of CLABSI in this prospective single-blinded clinical study. Blood cultures were routinely collected and subsequently two central samples from the CVAD and two peripheral samples from a peripheral venous access point. Primary outcome was the sensitivity and specificity of ddPCR. RESULTS: In total, 75 patients with 126 suspected CLABSI episodes were included, with 80 blood samples from the CVAD and 114 from peripheral veins. The central ddPCR samples showed a sensitivity of 91% (95%CI 77-98), and specificity of 96% (95%CI 85-99). Peripheral ddPCR samples had a sensitivity of 63% (95%CI 46-77) and specificity of 99% (95%CI 93-100). CONCLUSION: ddPCR showed a high sensitivity and specificity relative to blood cultures and enables rapid pathogen detection and characterization. Clinical studies should explore if integrated ddPCR and blood culture outcomes enables a more rapid pathogen guided CLABSI treatment and enhancing patient outcomes.

Genomic characterization of methicillin-susceptible Staphylococcus aureus carriage in patients on home parenteral nutrition and their caregivers.

In this prospective study, patients on home parenteral nutrition were twice as likely to be colonized with Staphylococcus aureus if their caregivers were also carriers. Among S. aureus-positive patients and their caregivers, molecular analysis showed 68% genetically related strains. Despite decolonization, genetically related strains reappeared in 70% of patients.

Having breakfast has no clinically relevant effect on bioelectrical impedance measurements in healthy adults.

BACKGROUND: Bioelectrical impedance analysis (BIA) is commonly used to evaluate body composition as part of nutritional assessment. Current guidelines recommend performing BIA measurements in a fasting state of at least 2 h in a clinical setting and 8 h in a research setting. However, since asking patients with malnutrition or sarcopenia to fast is not desirable and literature to support the strategy in the guidelines is lacking, this study aimed to assess the impact of breakfast on BIA measurements. METHODS: We performed an explorative, prospective study in healthy volunteers aged between 18 and 70 years, with a normal fluid balance and a body mass index between 18.5 and 30 kg/m2. BIA measurements were performed according to the standard operating procedure in the fasting state, and 1, 2, 3, and 4 h after ingesting a standardized breakfast meal of about 400 kcal with a 150 mL drink, using the hand-to-food single-frequency BIA (Bodystat500 ®). The Kyle formula was used to calculate the primary outcome, i.e. fat-free mass (FFM, kg). A linear mixed model was used to compare baseline values with other time points. A difference of 1 kg in FFM was considered clinically relevant. RESULTS: Thirty-nine (85% female) volunteers were included, with a median age of 28 years (IQR 24-38). In 90% of the participants, having breakfast had no clinically relevant impact on the estimated FFM. For the group, the most pronounced mean difference, a statistically but not clinically significant higher value of 0.2 kg (0.4%), was observed after 3 h of fasting compared to baseline. No statistically significant differences were found at the other time points. CONCLUSION: Eating affects single-frequency BIA measurements, but differences in FFM remain below clinical relevance for most participants when using a standardized breakfast. Thus, the current study suggests performing a BIA measurement in a fasting state is not required.

Oral antimicrobial agents in patients with short bowel syndrome: worth a try!

BACKGROUND: The use of oral antimicrobial agents in patients with short bowel syndrome (SBS) is challenging due to the changes in gastrointestinal anatomy that may result in diminished absorption and altered drug bioavailability. Prospective studies evaluating bioavailability of antimicrobial agents after oral administration in SBS patients are lacking. OBJECTIVES: To determine the bioavailability of orally administered antimicrobial agents commonly used for treatment in SBS patients to guide clinical decision making when faced with infections. METHODS: We performed an explorative, clinical study investigating the pharmacokinetics (PK) of clindamycin, ciprofloxacin, flucloxacillin and fluconazole in SBS patients with intestinal failure. Participants received a combination of two antimicrobial agents simultaneously. To determine the oral bioavailability, participants received a single oral and IV dose of both agents on two occasions, after which they underwent intensive PK sampling on six predefined time points up to 12 hours after administration. Primary outcome was the oral bioavailability of these antimicrobial agents. Secondary outcomes were intravenous PK characteristics following non-compartmental analysis. RESULTS: Eighteen SBS patients were included: the mean (SD) age was 59 (17) years and 61% of participants were female. The median observed (IQR) bioavailability of ciprofloxacin, clindamycin, flucloxacillin and fluconazole were 36% (24-50), 93% (56-106), 50% (32-76) and 98% (61-107), respectively. CONCLUSION: The bioavailability of selected antimicrobial agents in certain patients with SBS appeared to be better than expected, providing a feasible treatment option. Due to the large observed differences between patients, therapeutic drug monitoring should be part of the treatment to safeguard adequate exposure in all patients. TRIAL REGISTRATION: Registered in the Dutch Trial Register (NL7796) and EudraCT number 2019-002587-28.

Outcomes and survival in patients with advanced intestinal neuroendocrine tumours on home parenteral nutrition, an international multicentre retrospective cohort study.

BACKGROUND AND AIMS: Only limited information is available on the use of home parenteral nutrition (HPN) in patients with advanced neuroendocrine tumours (NETs) causing intestinal failure (IF). This study aims to report the outcomes of the explore the use of HPN in this patient cohort, in the largest case series to date. METHODS: A retrospective study in the United Kingdom and the Netherlands was performed, using the UK National British Artificial Nutrition Survey (BANS) and local databases in the Netherlands. Data regarding age, sex, NET grading, staging, treatment, HPN characteristics and survival outcomes were collected. RESULTS: Data were collected on 41 patients (n = 18 males, 44%) with a median age of 65. Most primary tumours were in the small bowel (n = 35, 85%). The NETs were Grade 1 (n = 16, 39%), Grade 2 (n = 7, 17%), Grade 3 (n = 1, 2%). In 28 patients (n = 68%) there was stage IV disease with metastases located in the peritoneum, mesentery and or liver. There were two indications for HPN; short bowel syndrome (n = 27, 66%) and inoperable malignant bowel obstruction (n = 14, 34%). The median period on HPN was 11 months (interquartile range 4-25 months). 11 patients were still alive and receiving HPN treatment after 2 years, and 6 patients after 3 years. Six patients (22%) with short bowel syndrome (SBS) could be weaned from HPN. There was a statistically significant improved survival for patients with short bowel syndrome (median 24 months) compared to inoperable malignant bowel obstruction (median 7 months). The catheter-related bloodstream infection rate was comparable to other HPN patient cohorts at 1.0 per 1000 catheter days. CONCLUSION: This study shows that HPN can be used safely in patients with NET and IF to increase survival beyond that reasonably expected in the context of either short bowel syndrome or inoperable malignant bowel obstruction. Patients with short bowel syndrome are most likely to benefit. Further prospective studies are necessary to validate survival benefits and to demonstrate the effect of HPN on quality of life.

Randomized clinical trial: Long-term Staphylococcus aureus decolonization in patients on home parenteral nutrition.

BACKGROUND & AIMS: Staphylococcus aureus decolonization has proven successful in prevention of S. aureus infections and is a key strategy to maintain venous access and avoid hospitalization in patients receiving home parenteral nutrition (HPN). We aimed to determine the most effective and safe long-term S. aureus decolonization regimen. METHODS: A randomized, open-label, multicenter clinical trial was conducted. Adult intestinal failure patients with HPN support and carrying S. aureus were randomly assigned to a 'continuous suppression' (CS) strategy, a repeated chronic topical antibiotic treatment or a 'search and destroy' (SD) strategy, a short and systemic antibiotic treatment. Primary outcome was the proportion of patients in whom S. aureus was totally eradicated during a 1-year period. Secondary outcomes included risk factors for decolonization failure and S. aureus infections, antimicrobial resistance, adverse events, patient compliance and cost-effectivity. RESULTS: 63 participants were included (CS 31; SD 32). The mean 1-year S. aureus decolonization rate was 61% (95% CI 44, 75) for the CS group and 39% (95% CI 25, 56) for the SD group with an OR of 2.38 (95% CI 0.92, 6.11, P = 0.07). More adverse effects occurred in the SD group (P = 0.01). Predictors for eradication failure were a S. aureus positive caregiver and presence of a (gastro)enterostomy. CONCLUSION: We did not demonstrate an increased efficacy of a short and systemic S. aureus decolonization strategy over a continuous topical suppression treatment. The latter may be the best option for HPN patients as it achieved a higher long-term decolonization rate and was well-tolerated (NCT03173053).

Berlin Bowel Bothers: Might Adolf Hitler's Gut Problems Have Been Parkinson-Related?

It has been argued that Adolf Hitler (1889-1945) had Parkinson's disease. He also experienced several gastrointestinal symptoms, for which various explanations have been sought, both contemporaneously and by later authors. In this Historical Note, a possible relationship between Hitler's Parkinson's disease and his gastrointestinal symptoms is explored. Specifically, we posit the hypothesis that Hitler may have suffered from small-intestinal bacterial overgrowth (SIBO), thus providing an early example of SIBO occurring as a prodromal Parkinson's disease symptom.

Antiseptic barrier caps to prevent central line-associated bloodstream infections: A systematic review and meta-analysis.

BACKGROUND: Reliable and safe venous access is crucial for patients using central venous catheters (CVC). However, such CVCs carry a risk for central line-associated bloodstream infections (CLABSIs). Antiseptic barrier caps (ABCs) are a novel tool in the armamentarium for CVC disinfection. Our aim was to review the efficacy and safety of ABCs. METHOD: A literature search was conducted using MedLine, EMBASE, Cochrane library, and CINAHL. Primary aim was to compare CLABSI rates in patients using ABCs versus standard care. Secondary aims included efficacy of ABCs in relevant subgroups (age, ABC brand, clinical setting), safety, compliance, and costs. Fifteen studies were included in the meta-analysis. RESULTS: In total, 391 CLABSIs in 273,993 catheter days occurred in the intervention group versus 620 CLABSIs in 284,912 days in the standard care group, resulting in a risk ratio of 0.65 (95%CI 0.55-0.76; P < .00001). Subgroup analyses showed similar effects, except for nonintensive care unit. In general, ABCs were safe, highly appreciated by patients and caregivers, and cost-effective, while compliance was easy to monitor. In most studies, a substantial risk of bias was observed. CONCLUSION: In conclusion, while available evidence suggests that ABCs are effective, safe, easy in use, and cost-effective. However, due to the poor methodological quality of most available studies, more robust data should justify their use at this point.

The Antiseptic and Antineoplastic Agent Taurolidine Modulates Key Leukocyte Functions.

BACKGROUND/AIM: Although taurolidine is known to exert a wide spectrum of biological actions, its effects on immune cells have not been characterized in detail. In this study, we investigated the ex vivo effects of taurolidine on relevant innate and adaptive immune cell functions. MATERIALS AND METHODS: Leukocyte functions in whole blood were assessed following treatment with various taurolidine concentrations. Viability of peripheral blood mononuclear cells (PBMCs) and granulocytes was measured using the WST-1 assay. PBMC function was assessed by measuring TNFα and IFNγ production after stimulation with lipopolysaccharide (LPS) or Candida, respectively. Reactive oxygen species (ROS) production by granulocytes was measured in whole blood using luminol-enhanced chemiluminescence. Granulocyte degranulation and activation were evaluated by membrane expression of degranulation (CD63, CD66B) and adhesion markers (CD62L, CD11b) using immunofluorescent staining followed by flow-cytometric analysis. RESULTS: Taurolidine decreased viability of PBMCs and granulocytes: after 2 h, IC50 concentrations were 500 and 520 µg/ml, respectively. Following prolonged exposure (≥24 h) of PBMCs, the IC50 concentrations declined to 40 µg/ml. PBMC cytokine production significantly decreased at taurolidine concentrations below the cytotoxic threshold, whereas no changes in ROS production were observed. The expression of all granulocyte adhesion and degranulation markers increased at concentrations higher than 500 µg/ml (the cytotoxic level of taurolidine). CONCLUSION: Taurolidine exhibits a dose- and time-dependent cytotoxicity toward PBMCs and granulocytes. The effects on PBMCs, as exemplified by a decrease in cytokine production, occurred below the toxic threshold, whereas granulocyte function (ROS production) remained unchanged at these taurolidine concentrations. Granulocyte activation and degranulation markers only increased at cytotoxic taurolidine concentrations.

Catheter-related bloodstream infection management in patients receiving home parenteral nutrition: An observational cohort study.

BACKGROUND AND AIMS: Patients with intestinal failure receiving home parenteral nutrition (HPN) are susceptible to central-line associated bloodstream infections (CLABSIs), with crucial roles for adequate (empiric) antimicrobial therapy and effective catheter management strategies. Our aim was to link recent epidemiologic CLABSI data with clinical outcomes and to identify risk factors for therapeutic failure to decide on the safest and most accurate CLABSI management in patients receiving HPN. METHODS: A retrospective observational cohort study was conducted. All data on CLABSIs (period 2010-2020) in adult patients receiving HPN were retrieved. The efficacy of attempted catheter salvage and empiric antimicrobial treatment (ß-lactam antibiotics) in our center, with a low prevalence of methicillin-resistant staphylococci, was investigated. Multivariate cox-regression analysis was performed to identify risk factors for recurrent CLABSI. RESULTS: 389 CLABSIs occurred in 149 patients. The overall infection rate was 0.64 per 1000 central venous catheter (CVC) days. Most CLABSIs were caused by Coagulase-negative staphylococci (37%). Attempted CVC salvage was successful in 70% of the cases. Empiric antimicrobial therapy was found to be adequate in only 47% of cases, mainly because of insufficient Coagulase-negative staphylococci coverage. According to the Cox model, patients with a replaced CVC had a 50% lower risk of a new CLABSI than patients with a retained (salvaged) CVC during follow-up (HR 0.50; 95% CI 0.35-0.72, P < 0.001). CONCLUSIONS: CVC salvage can be achieved in most CLABSI cases but seems associated with a shorter CLABSI-free survival. Importantly, based on our findings, a glycopeptide containing antibiotic treatment regimen will increase the likelihood of adequate empiric coverage.

Anticoagulants decrease the risk for catheter-related venous thrombosis in patients with chronic intestinal failure: A long-term cohort study.

BACKGROUND: Catheter-related venous thrombosis (CRVT) is a severe complication of home parental nutrition. Although primary prevention of CRVT is crucial, there is no consensus on anticoagulant use to prevent this adversity. The aim was to compare CRVT risk in patients with chronic intestinal failure (CIF) in the presence or absence of anticoagulants, and to identify CRVT risk factors. METHODS: This retrospective cohort study comprised adult patients with CIF with a central venous access device (CVAD) between 2010 and 2020 that were treated at our national CIF referral center. Analyses were performed at a CVAD level. RESULTS: Overall, 1188 CVADs in 389 patients were included (540.800 CVAD days). Anticoagulants were used in 403 CVADs. In total, 137 CRVTs occurred in 98 patients, resulting in 0.25 CRVTs/1000 CVAD days (95% CI, 0.22-0.29). Anticoagulant use was associated with a decreased CRVT risk (odds ratio [OR] = 0.53; 95% CI, 0.31-0.89; P = 0.02). Left-sided CVAD insertion (OR = 2.00; 95% CI, 1.36-2.94), a history of venous thrombosis (OR = 1.73; 95% CI, 1.05-2.84), and a shorter period postinsertion (OR = 0.78; 95% CI, 0.65-0.92) were independently associated with an increased CRVT risk. CONCLUSION: Anticoagulants decreased the CRVT risk. In addition, we identified left-sided vein insertion, a history of venous thrombosis, and a shorter period post-CVAD insertion as CRVT risk factors. Further prospective studies should provide guidance whether prophylactic anticoagulant use, especially in higher-risk patients with a left-sided CVAD or a history of venous thrombosis, is justified.

Comparing success rates in central venous catheter salvage for catheter-related bloodstream infections in adult patients on home parenteral nutrition: a systematic review and meta-analysis.

BACKGROUND: Catheter-related bloodstream infection (CRBSI) is a life-threatening complication of parenteral nutrition. Therefore, optimal management, ideally with catheter salvage, is required to maintain long-term venous access. OBJECTIVES: We aimed to evaluate successful catheter salvage rates in patients on home parenteral nutrition (HPN). METHODS: Studies were retrieved from medical databases, conference proceedings, and article reference lists. Data were collected relating to clinical outcomes of 3 treatments: systemic antibiotics, antimicrobial lock therapy (ALT), and catheter exchange. ORs and 95% CIs were calculated from a mixed logistic effects model. RESULTS: From 10,036 identified publications, 28 met the inclusion criteria (22 cohort studies, 5 case-control studies, and 1 randomized clinical trial), resulting in a total of 4911 CRBSIs. To achieve successful catheter salvage, the addition of an antimicrobial lock solution was superior to systemic antibiotics alone (OR: 1.75; 95% CI: 1.21, 2.53; P = 0.003). Recurrence of infection was less common in studies that used ALT than in those that used systemic antibiotics alone (OR: 0.26; 95% CI: 0.11, 0.61; P = 0.002). The catheter exchange group was excluded from multilevel regression analysis because only 1 included study applied this treatment. Successful salvage rates were highest for coagulase-negative staphylococci, followed by Gram-negative rods and Staphylococcus aureus . CONCLUSIONS: The addition of an antimicrobial lock solution seems beneficial for successful catheter salvage in HPN-dependent patients with a CRBSI. Future prospective randomized studies should identify the most effective and pathogen-specific strategy.This review was registered at www.crd.york.ac.uk/PROSPERO as CRD42018102959.

Effect of a Preoperative Home-Delivered, Protein-Rich Meal Service to Improve Protein Intake in Surgical Patients: A Randomized Controlled Trial.

BACKGROUND: The preoperative period likely provides an important opportunity to improve postoperative recovery, as suggested by the finding that low nutrition status is a predictor of increased postoperative complications and longer length of stay (LOS). It was investigated whether a home-delivered, protein-rich meal service improves protein intake relative to requirements within 3 weeks prior to surgery compared to usual care (UC). METHODS: This randomized controlled trial included adults (n = 126) with planned surgery performed at the orthopedics, urology, gynecology, or general surgery departments. The intervention group received 6 protein-rich dishes per day for 3 weeks, and the control group sustained their usual diet. Dietary intake, nutrition status, hand grip strength, physical performance, and quality of life were assessed at baseline and after 3 weeks. Patient satisfaction was reported after 3 weeks, and data on complications and LOS were reported 30 days after surgery. RESULTS: Protein intake relative to requirements significantly improved by 16%, and energy intake relative to requirements increased by 19% for the meal service, as compared with UC. The intervention group experienced significantly less stress with preparing meals and were more satisfied with the presentation of the meals than the control group. No significant effects of the intervention were detected on other secondary outcomes. CONCLUSION: The home-delivered, protein-rich meal service was successfully implemented before surgery and improved protein and energy intake relative to requirements within 3 weeks while patient satisfaction maintained. The preoperative period serves as a window of opportunity to prepare patients before hospitalization.

Association of Body Composition, Physical Functioning, and Protein Intake in Adult Patients With Mitochondrial Diseases.

BACKGROUND: Whether decreased physical functioning of patients with mitochondrial disease (MD) is related to altered body composition or low protein intake needs clarification at the background of the nutrition state. METHODS: In this 2-site cross-sectional study, MD patients were age-, body mass index (BMI)-, and gender-matched to controls. Body composition was assessed by dual-energy x-ray absorptiometry. Physical functioning was measured by handgrip strength, 6-minute walking test, 30-second sit-to-stand test (30SCT), and 6-minute mastication test. Total daily protein intake was calculated by 3-day food records. Malnutrition was assessed by Patient-Generated Subjective Global Assessment and the Global Leadership Initiative on Malnutrition (GLIM) criteria and sarcopenia by the 2018 consensus. Data were analyzed using independent samples t-tests, Fisher exact test, and Spearman and Pearson correlation coefficients. RESULTS: Thirty-seven MD patients (42 ± 12 years, BMI: 23 ± 4 kg/m2 , 59% females) and 37 matched controls were included. Handgrip strength was moderate, inversely related to fat mass index in both MD patients and controls, whereas it correlated with fat-free mass index in controls solely. Protein intake was associated with muscle strength (handgrip strength and 30SCT) in MD patients but not in controls. Twenty-seven MD patients (73%) were malnourished, and 5 (14%) were classified as sarcopenic. CONCLUSIONS: Muscle strength is related to body composition and protein intake in MD patients. This, in combination with the high incidence of both malnutrition and sarcopenia, warrants individual nutrition assessment in MD patients.

Protein Intake at the First Day of Full-Oral Intake During Hospitalization Is Associated With Complications and Hospital Length of Stay.

BACKGROUND: Malnutrition at admission is associated with complication-related readmission and prolonged hospital stay. This underscores the importance of an adequate intake - more particular, protein intake - to prevent further deterioration and treat malnutrition during hospitalization. Our objective was to assess whether protein intake relative to requirements at the first day of full oral intake is associated with complications and hospital length of stay (LOS) in medical and surgical patients. METHODS: This was a post hoc analysis of a prospective cohort study in patients on the wards of gastroenterology, orthopedics, urology, and gynecology. Protein intake was measured by subtracting the weight of each dish at the end of each mealtime from the weight at serving time. Complications and LOS were reported using patients' medical records. RESULTS: In total, complications were observed in 92 of 637 (14.4%) patients, with a median LOS of 5 days (3.0-7.0). An absolute increase of 10% protein intake relative to requirements reduced the relative complication risk by 10% (odds ratio, 0.900; 95% CI, 0.83-0.97; P < .05). Also, LOS was shortened by 0.23 days for each increase of 10% in protein intake relative to requirements (95% CI, -0.3 to -0.2; P < .05). CONCLUSION: Protein intake relative to requirements at the first day of full-oral intake is associated with the risk of complications and hospital LOS. This analysis bolsters the evidence for the importance of any hospital meal service that increases protein intake.

Eradication of Staphylococcus aureus colonization by chronic use of mupirocin in patients on home parenteral nutrition.

OBJECTIVES: Although in other groups Staphylococcus aureus eradication has proven to be an effective infection prevention measure, to our knowledge, no such studies have been performed in patients on home parenteral nutrition (HPN). The aim of this study was to investigate the efficacy of chronic nasal mupirocin use on S. aureus eradication and prevention of catheter related infections in patients on HPN. METHODS: This was a cohort study with data collected from adult patients on HPN who were screened for S. aureus carriage. In case of carriage, the patient was instructed to apply mupirocin nasal ointment monthly. Outcomes were the percentage of successful S. aureus eradication and the effect on the incidence of catheter-related infections and development of mupirocin resistance. RESULTS: S. aureus nasal carriage was found in 54% of the patients. Eradication was successful in 66% (70 of 106) of patients treated with mupirocin. Overall S. aureus catheter-related infection rates decreased by 50% (P = 0.02). The decrease was mostly due to a drop in central line-associated bloodstream infection (CLABSI) rates (0.26versus 0.1 per 1000 central venous catheter days; P = 0.04). The overall CLABSI rates decreased as well (incidence ratio rate, 0.43; 95% confidence interval. 0.24-0.76; P < 0.01). Low-level mupirocin resistance was observed in four patients. CONCLUSIONS: Findings from the present study highlighted the potential usefulness of mupirocin ointment prophylaxis to establish S. aureus eradication in patients on HPN. However, awareness for the development of mupirocin resistance is prudent. Further research needs to be carried out to validate these findings.

Optimal Estimate for Energy Requirements in Adult Patients With the m.3243A>G Mutation in Mitochondrial DNA.

AIM: We aimed to identify the optimal method to estimate total energy expenditure (TEE) in mitochondrial disease (MD) patients. METHODS: Resting energy expenditure (REE) was measured in MD patients carrying the m3243A>G mutation using indirect calorimetry (IC) and compared with results of 21 predictive equations (PEs) for REE and with REE-IC measurements in healthy controls. Physical activity level (PAL) was measured using accelerometery (SenseWear) and compared with a fixed average PAL (1.4) as well as patients' self-estimated activity levels. TEE was calculated as REE-IC × PAL SenseWear and compared with usual care and energy recommendations for healthy adults. RESULTS: Thirty-eight MD patients (age: 48 ± 13 years; body mass index 24 ± 4 kg/m2 ; male 20%) and 25 matched controls were included. The accuracy of most PEs was between 63% and 76%. The difference in REE-IC in healthy controls (1532 ± 182 kcal) and MD patients (1430 ± 221) was borderline not significant (P = .052). Patients' estimations PAL were 18%-34% accurate at the individual level. The fixed activity factor was 53% accurate. Patients overestimated their PAL. Usual care predicted TEE accurately in only 32% of patients. CONCLUSION: TEE is lower in these MD patients than the recommendations for healthy adults because of their lower physical activity. In MD patients, 6 PEs for REE provide a reliable alternative for IC, with an accuracy of 71%-76%. As PAL is highly variable and not reliably estimated by patients, measurement of PAL using accelerometery is recommended in this population.