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We report a 59-year-old woman presenting with vulval white plaques on a background of psoriasis and chronic cutaneous lupus erythematosus, and discuss the histology, diagnosis and treatment options.

Six months of supplementation with a multi-ingredient nutrition supplement was investigated in older adults with low skeletal muscle mass given the recently purported benefits of such approaches. Community-dwelling older adults (age, 74.9 ± 3.6 y; M/F, 18/19) participated in a double-blind, placebo-controlled, randomized trial involving daily consumption of either fruit juice placebo (PLA) or supplement (SUPP) in the form of a 200-mL carton of a juice-based emulsion of long chain n-3 polyunsaturated fatty acids (LC n-3 PUFA) (3000 mg as 1500 mg docosahexaenoic acid and 1500 mg eicosapentaenoic acid), whey protein isolate (8 g), vitamin D3 (400 IU), and resveratrol (150 mg). Body composition, physical function, and circulating markers of metabolic health were assessed at baseline (PRE), and after 3 (MID) and 6 (POST) months of supplementation. Lean body mass (LBM) was unchanged in either group, but fat mass increased in SUPP by 1.41 (0.75, 2.07) kg at POST (+6.4%

The impact of a ketogenic diet (KD) ( 75% fat) on athletic performance has sparked much interest and self-experimentation in the past 3-4 years. Evidence shows 3-4-week adaptations to a KD in endurance-trained athletes were associated with maintenance of moderate (46-63% VO2max) and vigorous intensity (64-90% VO2max) endurance exercise, while at intensities >70% VO2max, increases in fat oxidation were associated with decreased economy (increased oxygen consumption), and in some cases, increased ratings of perceived exertion and heart rate. Two investigations in recreationally active endurance athletes noted no vigorous intensity exercise decrement following 3- and 12-week adaptations. Moderate (70-85% one repetition maximum) and near-maximal to maximal intensity (>85% 1RM) strength performance experienced no decrement following a 3-12-week KD adaptation. Beneficial effects were noted for 2000 m sprint and critical power test completed for short duration at vigorous intensity, while two additional tests noted no decrement. For sprint, near-maximal exercise (>91% VO2max), benefit of the KD was observed for six-second sprint, while no decrement in performance was noted for two additional maximal tests. When protein is equated (grams per kilogram), one investigation noted no decrement in muscle hypertrophy, while one noted a decrement. One investigation with matched protein noted the KD group lost more body fat. In conclusion, moderate-to-vigorous intensity exercise experiences no decrement following adaptation to a KD. Decreases in exercise economy are observed >70% VO2max in trained endurance athletes which may negate performance within field settings. Beneficial effects of the KD during short duration vigorous, and sprint bouts of exercises are often confounded by greater weight loss in the KD group. With more athletes pursuing carbohydrate-restricted diets (moderate and strict (KD)) for their proposed health benefits, more work is needed in the area to address both performance and health outcomes.

Preexercise ingestion of exogenous ketones alters the metabolic response to exercise, but effects on exercise performance have been equivocal.On two occasions in a double-blind, randomized crossover design, eight endurance-trained runners performed 1 h of submaximal exercise at approximately 65% V˙O2max immediately followed by a 10-km self-paced time trial (TT) on a motorized treadmill. An 8% carbohydrate-electrolyte solution was consumed before and during exercise, either alone (CHO + PLA), or with 573 mg·kg of a ketone monoester supplement (CHO + KME). Expired air, HR, and RPE were monitored during submaximal exercise. Serial venous blood samples were assayed for plasma glucose, lactate, and β-hydroxybutyrate concentrations.CHO + KME produced plasma β-hydroxybutyrate concentrations of approximately 1.0 to 1.3 mM during exercise (P0.001), but plasma glucose and lactate concentrations were similar during exercise in both trials. V˙O2, running economy, respiratory exchange ratio, HR, and RPE were also similar between trials. Performance in the 10-km TT was not different (P = 0.483) between CHO + KME (mean, 2402 s; 95% confidence interval, 2204-2600 s) and CHO + PLA (mean, 2422 s; 95% confidence interval, 2217-2628 s). Cognitive performance, measured by reaction time and a multitasking test, did not differ between trials.Compared with carbohydrate alone, coingestion of KME by endurance-trained athletes elevated plasma β-hydroxybutyrate concentrations, but did not improve 10-km running TT or cognitive performance.

IntroductionKetogenic diets (KDs) that elevate ketones into a range referred to as nutritional ketosis represent a possible nutrition approach to address the emerging physical readiness and obesity challenge in the military. An emerging body of evidence demonstrates broad-spectrum health benefits attributed to being in nutritional ketosis, but no studies have specifically explored the use of a KD in a military population using daily ketone monitoring to personalize the diet prescription.Materials and MethodsTo evaluate the feasibility, metabolic, and performance responses of an extended duration KD, healthy adults (n = 29) from various military branches participated in a supervised 12-wk exercise training program. Fifteen participants self-selected to an ad libitum KD guided by daily measures of capillary blood ketones and 14 continued their normal mixed diet (MD). A battery of tests were performed before and after the intervention to assess changes in body mass, body composition, visceral fat, liver fat, insulin sensitivity, resting energy metabolism, and physical performance.ResultsAll KD subjects were in nutritional ketosis during the intervention as assessed by daily capillary beta-hydroxybutyrate (βHB) (mean βHB 1.2 mM reported 97% of all days) and showed higher rates of fat oxidation indicative of keto-adaptation. Despite no instruction regarding caloric intake, the KD group lost 7.7 kg body mass (range −3.5 to −13.6 kg), 5.1% whole-body percent fat (range −0.5 to −9.6%), 43.7% visceral fat (range 3.0 to −66.3%) (all p < 0.001), and had a 48% improvement in insulin sensitivity; there were no changes in the MD group. Adaptations in aerobic capacity, maximal strength, power, and military-specific obstacle course were similar between groups (p > 0.05).ConclusionsUS military personnel demonstrated high adherence to a KD and showed remarkable weight loss and improvements in body composition, including loss of visceral fat, without compromising physical performance adaptations to exercise training. Implementation of a KD represents a credible strategy to enhance overall health and readiness of military service members who could benefit from weight loss and improved body composition.

Background Low-carbohydrate diets have recently grown in popularity among endurance athletes, yet little is known about the long-term (> 4 wk) performance implications of consuming a low-carbohydrate high fat ketogenic diet (LCKD) in well-trained athletes. Methods Twenty male endurance-trained athletes (age 33 ± 11 y, body mass 80 ± 11 kg; BMI 24.7 ± 3.1 kg/m2) who habitually consumed a carbohydrate-based diet, self-selected into a high-carbohydrate (HC) group (n = 11, %carbohydrate:protein:fat = 65:14:20), or a LCKD group (n = 9, 6:17:77). Both groups performed the same training intervention (endurance, strength and high intensity interval training (HIIT)). Prior to and following successful completion of 12-weeks of diet and training, participants had their body composition assessed, and completed a 100 km time trial (TT), six second (SS) sprint, and a critical power test (CPT). During post-intervention testing the HC group consumed 30–60 g/h carbohydrate, whereas the LCKD group consumed water, and electrolytes. Results The LCKD group experienced a significantly greater decrease in body mass (HC − 0.8 kg, LCKD − 5.9 kg; P = 0.006, effect size (ES): 0.338) and percentage body fat percentage (HC − 0.7%, LCKD − 5.2%; P = 0.008, ES: 0.346). Fasting serum beta-hydroxybutyrate (βHB) significantly increased from 0.1 at baseline to 0.5 mmol/L in the LCKD group (P = 0.011, ES: 0.403) in week 12. There was no significant change in performance of the 100 km TT between groups (HC − 1.13 min·s, LCKD − 4.07 min·s, P = 0.057, ES: 0.196). SS sprint peak power increased by 0.8 watts per kilogram bodyweight (w/kg) in the LCKD group, versus a − 0.1 w/kg reduction in the HC group (P = 0.025, ES: 0.263). CPT peak power decreased by − 0.7 w/kg in the HC group, and increased by 1.4 w/kg in the LCKD group (P = 0.047, ES: 0.212). Fat oxidation in the LCKD group was significantly greater throughout the 100 km TT. Conclusions Compared to a HC comparison group, a 12-week period of keto-adaptation and exercise training, enhanced body composition, fat oxidation during exercise, and specific measures of performance relevant to competitive endurance athletes.

The impact of a ketogenic diet (KD) (75% fat) on athletic performance has sparked much interest and self-experimentation in the past 3–4 years. Evidence shows 3–4-week adaptations to a KD in endurance-trained athletes were associated with maintenance of moderate (46–63% VO2max) and vigorous intensity (64–90% VO2max) endurance exercise, while at intensities >70% VO2max, increases in fat oxidation were associated with decreased economy (increased oxygen consumption), and in some cases, increased ratings of perceived exertion and heart rate. Two investigations in recreationally active endurance athletes noted no vigorous intensity exercise decrement following 3- and 12-week adaptations. Moderate (70–85% one repetition maximum) and near-maximal to maximal intensity (>85% 1RM) strength performance experienced no decrement following a 3-12-week KD adaptation. Beneficial effects were noted for 2000 m sprint and critical power test completed for short duration at vigorous intensity, while two additional tests noted no decrement. For sprint, near-maximal exercise (>91% VO2max), benefit of the KD was observed for six-second sprint, while no decrement in performance was noted for two additional maximal tests. When protein is equated (grams per kilogram), one investigation noted no decrement in muscle hypertrophy, while one noted a decrement. One investigation with matched protein noted the KD group lost more body fat. In conclusion, moderate-to-vigorous intensity exercise experiences no decrement following adaptation to a KD. Decreases in exercise economy are observed >70% VO2max in trained endurance athletes which may negate performance within field settings. Beneficial effects of the KD during short duration vigorous, and sprint bouts of exercises are often confounded by greater weight loss in the KD group. With more athletes pursuing carbohydrate-restricted diets (moderate and strict (KD)) for their proposed health benefits, more work is needed in the area to address both performance and health outcomes.

BACKGROUND: Metabolic syndrome (MetS) is highly correlated with obesity and cardiovascular risk, but the importance of dietary carbohydrate independent of weight loss in MetS treatment remains controversial. Here, we test the theory that dietary carbohydrate intolerance (i.e., the inability to process carbohydrate in a healthy manner) rather than obesity per se is a fundamental feature of MetS. METHODS: Individuals who were obese with a diagnosis of MetS were fed three 4-week weight-maintenance diets that were low, moderate, and high in carbohydrate. Protein was constant and fat was exchanged isocalorically for carbohydrate across all diets. RESULTS: Despite maintaining body mass, low-carbohydrate (LC) intake enhanced fat oxidation and was more effective in reversing MetS, especially high triglycerides, low HDL-C, and the small LDL subclass phenotype. Carbohydrate restriction also improved abnormal fatty acid composition, an emerging MetS feature. Despite containing 2.5 times more saturated fat than the high-carbohydrate diet, an LC diet decreased plasma total saturated fat and palmitoleate and increased arachidonate. CONCLUSION: Consistent with the perspective that MetS is a pathologic state that manifests as dietary carbohydrate intolerance, these results show that compared with eucaloric high-carbohydrate intake, LC/high-fat diets benefit MetS independent of whole-body or fat mass. TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT02918422. FUNDING: Dairy Management Inc. and the Dutch Dairy Association.

Werner syndrome is a rare autosomal recessive disorder caused by mutations in the Werner syndrome WRN gene, on chromosome 8. Those affected manifest early the features of ageing. Cataract surgery is prone to post-operative complications in those with Werner syndrome. The development of cystoid macular oedema (CMO) is likely multifactorial. Patients with WS have diabetes mellitus type 2 which can contribute to macular oedema. There is a deposition of abnormal WRN proteins in the macula which also predisposes to macular oedema. The trauma of cataract surgery appears to be the main stimulus for the development of CMO. CMO may, as a result, be difficult to manage in Werner syndrome patients. Further study is needed to elucidate the precise role of retinal WRN protein expression in the development of CMO in those with Werner syndrome. A tailored and more successful approach to the treatment of CMO in such patients may result.

Background Adult onset idiopathic isolated focal dystonia presents with a number of phenotypes. Reported prevalence rates vary considerably; well-characterized cohorts are important to our understanding of this disorder. Aim To perform a nationwide epidemiological study of adult onset idiopathic isolated focal dystonia in the Republic of Ireland. Methods Patients with adult onset idiopathic isolated focal dystonia were recruited from multiple sources. Diagnosis was based on assessment by a neurologist with an expertise in movement disorders. When consent was obtained, a number of clinical features including family history were assessed. Results On the prevalence date there were 592 individuals in Ireland with adult onset idiopathic isolated focal dystonia, a point prevalence of 17.8 per 100 000 (95% confidence interval 16.4−19.2). Phenotype numbers were cervical dystonia 410 (69.2%), blepharospasm 102 (17.2%), focal hand dystonia 39 (6.6%), spasmodic dysphonia 18 (3.0%), musician's dystonia 17 (2.9%) and oromandibular dystonia six (1.0%). Sixty-two (16.5%) of 375 consenting index cases had a relative with clinically confirmed adult onset idiopathic isolated focal dystonia (18 multiplex and 24 duplex families). Marked variations in the proportions of patients with tremor, segmental spread, sensory tricks, pain and psychiatric symptoms by phenotype were documented. Conclusions The prevalence of adult onset idiopathic isolated focal dystonia in Ireland is higher than that recorded in many similar service-based epidemiological studies but is still likely to be an underestimate. The low proportion of individuals with blepharospasm may reflect reduced environmental exposure to sunlight in Ireland. This study will serve as a resource for international comparative studies of environmental and genetic factors in the pathogenesis of the disorder.