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1.
Muscle weakness as an additional criterion for grading sarcopenia-related prognosis in patients with cancer.
Cereda, E, Tancredi, R, Klersy, C, Lobascio, F, Crotti, S, Masi, S, Cappello, S, Stobäus, N, Tank, M, Cutti, S, et al
Cancer medicine. 2022;(2):308-316
Abstract
BACKGROUND Low muscle strength has been pointed out as a key characteristic of sarcopenia, but the prognostic significance of muscle function next to reduced skeletal muscle mass (SMM) in patients with cancer has been scantily investigated. METHODS Data on muscle strength by handgrip (HG) dynamometry and total-body SMM estimated by bioelectrical impedance analysis (BIA) of Italian and German patients with cancer observed prospectively until death or censoring were analysed (N = 1076). Patients were stratified in four risk categories based on low HG (<10th percentiles of age and gender-specific normative values) and low total-body SMM according to SMM index cutoffs (<10.75 and <6.75 kg/m2 in men and women, respectively). RESULTS During a median follow-up of 58 months [25th-75th percentile, 37-60], 566 patients had died. Patients presenting low HG in combination or not with low SMM were characterised by shorter median survival (12.7 vs. 27.2 months, respectively; p < 0.001) compared to those with low SMM/normal HG and normal SMM/normal HG (>60 months for both). After adjusting for sex, age, body mass index and percentage of weight loss, disease's stage, performance status and type of cancer, compared to reference category (normal HG and SMM; N = 210) the hazard ratios were: low SMM/normal HG (N = 342), 0.83 [95% confidence interval, CI, 0.67-1.02] (p = 0.073); normal SMM/low HG (N = 158), 1.19 [95% CI, 1.07-1.32] (p = 0.002); low SMM/low HG (N = 366), 1.39 [95% CI, 1.27-1.53] (p < 0.001). CONCLUSIONS Muscle weakness was found to be a more powerful predictor of survival than BIA-estimated SMM and should be considered as an additional key feature of sarcopenia in patients with cancer.
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Activity Levels in Survivors of the Intensive Care Unit.
Gandotra, S, Files, DC, Shields, KL, Berry, M, Bakhru, RN
Physical therapy. 2021;(9)
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Abstract
OBJECTIVE Limited data exist on the quantification of activity levels and functional status in critically ill patients as they transition from the intensive care unit (ICU) to the wards and, subsequently, back into the community. The physical activity of critically ill patients from their ICU stay until 7 days after hospital discharge was characterized, as well as correlate physical activity levels with an objective measure of physical function. METHODS This prospective observational study of previously independent adults aged 55 or older, undergoing mechanical ventilation for up to 7 days, recruited participants at the time of spontaneous breathing trials or less than 24 hours after extubation. Participants received an accelerometer at enrollment to wear until 1 week after discharge. RESULTS Twenty-two participants received accelerometers; 15 were suitable for analysis. Participants had a mean (SD) age of 68 (9.6) years; 47% were female. Mean step counts were 95 (95% CI = 15-173) in the 3 days before ICU discharge, 257 (95% CI = 114-400) before hospital discharge, 1223 (95% CI = 376-2070) in the first 3 days at home, and 1278 (95% CI = 349-2207) between day 4 and 6 post-hospital discharge. Physical activity was significantly higher post- compared with pre-hospital discharge. Short Physical Performance Battery scores were poor at ICU and hospital discharge; however, they correlated moderately with physical activity levels immediately upon return home. CONCLUSIONS Physical activity remained low as survivors of critical illness transitioned from ICU to hospital wards, but significantly increased upon return to the community. Despite poor Short Physical Performance Battery scores at both ICU and hospital discharge, participants were significantly more active immediately after discharge than in their last 3 days of hospitalization. This may represent rapid functional improvement or, conversely, constrained physical activity in hospital. IMPACT This study highlights the need for further evaluation of physical activity constraints in hospital and ways to augment physical activity and function upon discharge. LAY SUMMARY Physical activity (step counts) increased modestly as survivors of critical illness transitioned from ICU to hospital wards, but significantly increased upon return to the community. This study highlights the need for further evaluation of physical activity constraints in the hospital setting and ways to augment physical activity and function postdischarge.
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Marked reduction in paralytic attacks in a patient with Andersen-Tawil syndrome switched from acetazolamide to dichlorphenamide.
Gupta, A, Iyadurai, S, Roggenbuck, J, LoRusso, S
Neuromuscular disorders : NMD. 2021;(7):656-659
Abstract
Andersen-Tawil syndrome is a rare, autosomal dominant, multisystem disorder for which the majority of cases are caused by pathogenic variants in the KCNJ2 gene. The syndrome is characterized by the clinical triad of episodic paralysis, cardiac conduction abnormalities, and dysmorphic facial and skeletal features. Treatment of Andersen-Tawil syndrome is primarily focused on management of cardiac arrhythmias and preventive management of paralytic attacks. Dichlorphenamide is approved by the US Food and Drug Administration for use in primary periodic paralysis based on several randomized, controlled trials but has not been studied in patients with Andersen-Tawil syndrome. Here, we report a case of the syndrome caused by a de novo pathogenic variant in the KCNJ2 gene (c.95_98del). The paralytic attack rate for this patient was better controlled with dichlorphenamide compared with acetazolamide, further supporting the use of dichlorphenamide in patients with Andersen-Tawil syndrome.
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A pediatric case report and literature review of facioscapulohumeral muscular dystrophy type1.
Xiao, T, Yang, H, Gan, S, Wu, L
Medicine. 2021;(47):e27907
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RATIONALE Early-onset facioscapulohumeral muscular dystrophy (FSHD) is defined as facial weakness before the age of 5 and shoulder weakness before the age of 10. Early-onset facioscapulohumeral muscular dystrophy is relatively rare in the clinic. This onset is relatively early, the symptoms are serious, and it is likely to be accompanied by retinal vascular disease, sensorineural deafness, epilepsy and other extramuscular multisystem diseases. We report the clinical characteristics of 2 patients with early-onset facial and shoulder brachial muscular dystrophy to improve clinicians' understanding of this particular condition. PATIENT CONCERNS We report 2 pediatric patients with FSHD type 1. Patient 1 is an 11-year-old boy with reduced facial expression for 9 years and proximal muscle weakness for 6 years. Patient 2 is a 4-year and 6-month-old girl with developmental delay for 3 years and facial weakness for 1 year. DIAGNOSIS According to the clinical manifestations and molecular genetic testing (such as Southern blot analysis), the patients were diagnosed with early-onset FSHD1. INTERVENTIONS The patients received cocktail therapy (vitamin B1 tablets, vitamin B2 tablets, vitamin B6 tablets, vitamin C tablets, vitamin E tablets, idebenone tablets, etc.) to improve their muscle metabolism. OUTCOMES Both patients' condition did not improve after being given cocktail treatment. According to a recent follow-up, the symptoms of facial weakness and proximal muscle weakness were aggravated. LESSONS Early-onset FSHD presents early and has frequent systemic features, and it is a severe subtype of FSHD. Early identification and genetic diagnosis should be performed to improve patient prognosis.
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Guidelines on clinical presentation and management of nondystrophic myotonias.
Stunnenberg, BC, LoRusso, S, Arnold, WD, Barohn, RJ, Cannon, SC, Fontaine, B, Griggs, RC, Hanna, MG, Matthews, E, Meola, G, et al
Muscle & nerve. 2020;(4):430-444
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The nondystrophic myotonias are rare muscle hyperexcitability disorders caused by gain-of-function mutations in the SCN4A gene or loss-of-function mutations in the CLCN1 gene. Clinically, they are characterized by myotonia, defined as delayed muscle relaxation after voluntary contraction, which leads to symptoms of muscle stiffness, pain, fatigue, and weakness. Diagnosis is based on history and examination findings, the presence of electrical myotonia on electromyography, and genetic confirmation. In the absence of genetic confirmation, the diagnosis is supported by detailed electrophysiological testing, exclusion of other related disorders, and analysis of a variant of uncertain significance if present. Symptomatic treatment with a sodium channel blocker, such as mexiletine, is usually the first step in management, as well as educating patients about potential anesthetic complications.
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[Intensive Care Unit-Acquired Weakness].
Siao, SF, Yen, YH, Yu, YF, Zong, SL, Chen, CC
Hu li za zhi The journal of nursing. 2020;(3):6-13
Abstract
Intensive care unit (ICU)-acquired weakness is a common neuromuscular complication of critical illness that is considered to be associated with prolonged duration on mechanical ventilation and systemic inflammatory response syndrome. In addition, nutrition and metabolic alternations, which are commonly seen in patients in the ICU, may further accelerate muscle wasting and increase the incidence of ICU-acquired weakness. The clinical features of ICU-acquired weakness include acute generalized muscle weakness that develops after the onset of critical illness. Diaphragmatic dysfunction, post-extubation dysphagia, and functional decline also are common in patients with ICU-acquired weakness. As the recovery of these physical functions is lengthy and difficult, a multidisciplinary team management is recommended. This mini-review was conducted to provide a scientific overview for ICU-acquired weakness, including its definition, etiology, diagnosis/screening, impacts, and potential intervention strategies. We hope that increasing the understanding of frontline staff will promote the timely planning and implementation of related screenings and interventions to enhance the functional recovery of patients receiving care in the ICU.
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Strength deficits in lower limb prosthesis users: A scoping review.
Hewson, A, Dent, S, Sawers, A
Prosthetics and orthotics international. 2020;(5):323-340
Abstract
BACKGROUND Strength deficits may play a central role in the severity of balance, mobility, and endurance impairments in lower limb prosthesis users. A body of literature detailing the scope and specifics of muscle weakness in lower limb prosthesis users is emerging, but has yet to be summarized. A synopsis of strength deficits, and their impact on functional abilities in lower limb prosthesis users, may inform rehabilitation and research needs. OBJECTIVES Synthesize reported strength deficits in lower limb prosthesis users, and discuss possible causes, consequences, and solutions. STUDY DESIGN Scoping review. METHODS A search of biomedical databases was performed, and inclusion/exclusion criteria were applied to identify publications relevant to the purpose of the review. RESULTS In all, 377 publications were identified, of which 12 met the inclusion/exclusion criteria. When compared with the controls and the intact limb, the primary strength outcome, peak torque, was lower in transtibial residual limb knee flexors and extensors, as well as transfemoral residual limb hip muscles. CONCLUSIONS The reviewed studies provide evidence of strength deficits in lower limb prosthesis users. These deficits appear to be consequential, as they may contribute to balance, mobility, and endurance impairments. Additional research exploring alternative strength metrics, clinical tests, and causal links to functional impairments is required. CLINICAL RELEVANCE Evidence of muscle weakness among lower limb prosthesis users, and its influence on balance, mobility, and endurance, suggests that greater clinical attention and scientific inquiry into physical conditioning of lower limb prosthesis users is merited and required.
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Weak and Dizzy-Another Explanation to Explore: Poor Nutrition in the Older Adult.
Somes, J
Journal of emergency nursing. 2020;(4):541-545
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Disorders of Human Coenzyme Q10 Metabolism: An Overview.
Hargreaves, I, Heaton, RA, Mantle, D
International journal of molecular sciences. 2020;(18)
Abstract
Coenzyme Q10 (CoQ10) has a number of vital functions in all cells, both mitochondrial and extramitochondrial. In addition to its key role in mitochondrial oxidative phosphorylation, CoQ10 serves as a lipid soluble antioxidant, plays an important role in fatty acid, pyrimidine and lysosomal metabolism, as well as directly mediating the expression of a number of genes, including those involved in inflammation. In view of the central role of CoQ10 in cellular metabolism, it is unsurprising that a CoQ10 deficiency is linked to the pathogenesis of a range of disorders. CoQ10 deficiency is broadly classified into primary or secondary deficiencies. Primary deficiencies result from genetic defects in the multi-step biochemical pathway of CoQ10 synthesis, whereas secondary deficiencies can occur as result of other diseases or certain pharmacotherapies. In this article we have reviewed the clinical consequences of primary and secondary CoQ10 deficiencies, as well as providing some examples of the successful use of CoQ10 supplementation in the treatment of disease.
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Putative Cut-Points in Sarcopenia Components and Incident Adverse Health Outcomes: An SDOC Analysis.
Cawthon, PM, Manini, T, Patel, SM, Newman, A, Travison, T, Kiel, DP, Santanasto, AJ, Ensrud, KE, Xue, QL, Shardell, M, et al
Journal of the American Geriatrics Society. 2020;(7):1429-1437
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OBJECTIVES Analyses performed by the Sarcopenia Definitions and Outcomes Consortium (SDOC) identified cut-points in several metrics of grip strength for consideration in a definition of sarcopenia. We describe the associations between the SDOC-identified metrics of low grip strength (absolute or standardized to body size/composition); low dual-energy x-ray absorptiometry (DXA) lean mass as previously defined in the literature (appendicular lean mass [ALM]/ht2 ); and slowness (walking speed <.8 m/s) with subsequent adverse outcomes (falls, hip fractures, mobility limitation, and mortality). DESIGN Individual-level, sex-stratified pooled analysis. We calculated odds ratios (ORs) or hazard ratios (HRs) for incident falls, mobility limitation, hip fractures, and mortality. Follow-up time ranged from 1 year for falls to 8.8 ± 2.3 years for mortality. SETTING Eight prospective observational cohort studies. PARTICIPANTS A total of 13,421 community-dwelling men and 4,828 community-dwelling women. MEASUREMENTS Grip strength by hand dynamometry, gait speed, and lean mass by DXA. RESULTS Low grip strength (absolute or standardized to body size/composition) was associated with incident outcomes, usually independently of slowness, in both men and women. ORs and HRs generally ranged from 1.2 to 3.0 for those below vs above the cut-point. DXA lean mass was not consistently associated with these outcomes. When considered together, those who had both muscle weakness by absolute grip strength (<35.5 kg in men and <20 kg in women) and slowness were consistently more likely to have a fall, hip fracture, mobility limitation, or die than those without either slowness or muscle weakness. CONCLUSION Older men and women with both muscle weakness and slowness have a higher likelihood of adverse health outcomes. These results support the inclusion of grip strength and walking speed as components in a summary definition of sarcopenia. J Am Geriatr Soc 68:1429-1437, 2020.