Efficacy and safety of manual acupuncture for the treatment of upper limb motor dysfunction after stroke: Protocol for a systematic review and meta-analysis.

INTRODUCTION: The incidence of stroke sequelae among patients is as high as 70%-80%. Flexor spasm is the most common stroke sequela, presenting a heavy burden to the patients and their families. This study will evaluate the results of randomized controlled trials to determine the efficacy and safety of hand manipulation acupuncture for the treatment of upper limb motor dysfunction after stroke. METHODS: Eight databases, including China National Knowledge Infrastructure, Chinese Scientific Journal Database, Cochrane Central Register of Controlled Trials, Embase, MEDLINE, PubMed, Wanfang Database, and Web of Science, will be searched using English and Chinese search strategies. In addition, manual retrieval of research papers, conference papers, ongoing experiments, and internal reports, among others, will supplement electronic retrieval. All eligible studies published on or before January 15, 2021 will be selected. To enhance the effectiveness of the study, only clinical randomized controlled trials related to the use of manual acupuncture for the treatment of upper limb motor dysfunction after stroke will be included. ANALYSIS: The Fugl-Meyer upper extremity assessment will be the primary outcome measure, whereas the Wolf Motor Function Test, Modified Ashworth Scale, arm movement survey test table, and upper extremity freehand muscle strength assessment scores will be the secondary outcomes. Side effects and adverse events will be included as safety evaluations. To ensure the quality of the systematic evaluation, study selection, data extraction, and quality assessment will be independently performed by two authors, and a third author will resolve any disagreement. ETHICS AND DISSEMINATION: This systematic review will evaluate the efficacy and safety of manual acupuncture for the treatment of upper limb motor dysfunction after stroke. Since all included data will be obtained from published articles, it does not require ethical approval and will be published in a peer-reviewed journal. INPLASY registration number: INPLASY202110071.

Coenzyme Q10 deficiency in patients with hereditary hemochromatosis.

AIM: Hereditary hemochromatosis (HH) is a group of inherited disorders that causes a slow and progressive iron deposition in diverse organs, particularly in the liver. Iron overload induces oxidative stress and tissue damage. Coenzyme Q10 (CoQ10) is a cofactor in the electron-transport chain of the mitochondria, but it is also a potent endogenous antioxidant. CoQ10 interest has recently grown since various studies show that CoQ10 supplementation may provide protective and safe benefits in mitochondrial diseases and oxidative stress disorders. In the present study we sought to determine CoQ10 plasma level in patients recently diagnosed with HH and to correlate it with biochemical, genetic, and histological features of the disease. METHODS: Plasma levels of CoQ10, iron, ferritin, transferrin and vitamins (A, C and E), liver tests (transaminases, alkaline phosphatase and bilirubin), and histology, as well as three HFE gene mutations (H63D, S654C and C282Y), were assessed in thirty-eight patients (32 males, 6 females) newly diagnosed with HH without treatment and in twenty-five age-matched normolipidemic healthy subjects with no HFE gene mutations (22 males, 3 females) and without clinical or biochemical signs of iron overload or liver diseases. RESULTS: Patients with HH showed a significant decrease in CoQ10 levels respect to control subjects (0.31 ±â€¯0.03 µM vs 0.70 ±â€¯0.06 µM, p < 0.001, respectively) independently of the genetic mutation, cirrhosis, transferrin saturation, ferritin level or markers of hepatic dysfunction. Although a decreasing trend in CoQ10 levels was observed in patients with elevated iron levels, no correlation was found between both parameters in patients with HH. Vitamins C and A levels showed no changes in HH patients. Vitamin E was significantly decreased in HH patients (21.1 ±â€¯1.3 µM vs 29.9 ±â€¯2.5 µM, p < 0.001, respectively), but no correlation was observed with CoQ10 levels. CONCLUSION: The decrease in CoQ10 levels found in HH patients suggests that CoQ10 supplementation could be a safe intervention strategy complementary to the traditional therapy to ameliorate oxidative stress and further tissue damage induced by iron overload.

COVID-19: Short and Long-Term Effects of Hospitalization on Muscular Weakness in the Elderly.

The COVID-19 pandemic has recently been the cause of a global public health emergency. Frequently, elderly patients experience a marked loss of muscle mass and strength during hospitalization, resulting in a significant functional decline. This paper describes the impact of prolonged immobilization and current pharmacological treatments on muscular metabolism. In addition, the scientific evidence for an early strength intervention, neuromuscular electrical stimulation or the application of heat therapy during hospitalization to help prevent COVID-19 functional sequels is analyzed. This review remarks the need to: (1) determine which potential pharmacological interventions have a negative impact on muscle quality and quantity; (2) define a feasible and reliable pharmacological protocol to achieve a balance between desired and undesired medication effects in the treatment of this novel disease; (3) implement practical strategies to reduce muscle weakness during bed rest hospitalization and (4) develop a specific, early and safe protocol-based care of functional interventions for older adults affected by COVID-19 during and after hospitalization.

Type of measurement used influences central and peripheral contributions to quadriceps weakness after anterior cruciate ligament (ACL) reconstruction.

OBJECTIVE: The relative contribution of muscle size and voluntary activation (VA) on quadriceps strength after anterior cruciate ligament (ACL) reconstruction remains inconclusive. Here, we aimed to determine the contributions of muscle size and VA on quadriceps strength in ACL-reconstructed patients and determine if contributions were similar if unilateral outcomes (i.e. ACL-reconstructed limb) or the LSI was used. DESIGN: A cross-sectional study. SETTING: A university research laboratory. PARTICIPANTS: Sixteen individuals 6-12 months after ACL reconstruction (Age: 22.3 ± 6.0yr, Height: 1.7 ± 0.1 m, Mass: 68.7 ± 11.5 kg) were recruited. MAIN OUTCOME MEASURES: Quadriceps isometric strength and VA, via the interpolated triplet technique, were assessed bilaterally. Ultrasound images were acquired of the vastus lateralis to calculate cross-sectional area (CSA) in both legs. LSI's were computed for all variables by expressing values of the reconstructed leg as a percent of the non-reconstructed leg. Separate stepwise linear regressions were performed to examine the contribution of VA and CSA on quadriceps strength. Model 1 used LSI for all outcomes and model 2 used outcomes from the reconstructed leg. RESULTS: We observed between limb deficits of 27.78% in quadriceps strength, 13.61% in vastus lateralis CSA, and 13.18% in VA (P < 0.05). Strength LSI was significantly predicted by VA LSI (R2 = 0.45, P < 0.01), but not by CSA LSI (R2 = 0.01, P =0.87). Reconstructed leg strength was significantly predicted by VL CSA (R2 = 0.50, P < 0.01) but not quadriceps VA (R2 = 0.08, P =0.11). CONCLUSIONS: The contributions of VA and CSA on quadriceps PT differed greatly if LSI or reconstructed leg outcomes were used. Evaluation of VA and CSA in unison may be provide a more holistic understanding of the sources of muscle weakness after ACL reconstruction.

Habitual dietary fat intake and risk of muscle weakness and lower-extremity functional impairment in older adults: A prospective cohort study.

BACKGROUND & AIMS: Fatty acid supplementation increases muscle mass and function in older adults, but the effect of habitual dietary intake is uncertain. Therefore, the objective of this study was to examine the association between habitual dietary fat intake and risk of muscle weakness and lower-extremity functional impairment (LEFI) in older adults. METHODS: Prospective study with 1873 individuals aged ≥60 years from the Seniors-ENRICA cohort. In 2008-10 and 2012, a validated face-to-face diet history was used to record the one-year consumption of up to 880 foods. Then, fatty acids, other nutrients and energy intake were estimated using standard food composition tables. Means of intake between these years were calculated to represent cumulative consumption over the follow-up. Study participants were followed up through 2015 to assess incident muscle weakness (lowest quintile of grip strength) and incident LEFI (Short Physical Performance Battery score ≤6). Analyses were performed with Cox regression and adjusted for the main confounders, including other types of fatty acids. RESULTS: Over a median follow-up of 5.2 years, 331 participants developed muscle weakness and 397 LEFI. Intake of saturated fatty acids (SFA) did not show an association with muscle weakness but was associated with higher risk of LEFI (multivariable hazard ratio (HR) for tertile 3 vs. tertile 1: 1.15; 95% confidence interval: 1.05-2.01; p-trend = 0.02). This association was mostly due to consumption of Spanish cold cuts and pastry and, to a lesser extent, dairy. Monounsaturated fatty acids (MUFA) intake was associated with lower risk of muscle weakness (HR t3 vs. t1: 0.73; 0.54-0.99; p trend = 0.04), and intake of n-3 polyunsaturated fatty acids (PUFA) was associated with reduced risk of both muscle weakness (0.70; 0.52-0.95; p-trend = 0.02) and LEFI (0.49; 0.35-0.68; p-trend <0.001). Olive oil and blue fish, the main sources of MUFA and PUFA, were also associated with lower risk of muscle weakness and LEFI. CONCLUSIONS: Habitual intake of SFA was associated with increased risk of LEFI. By contrast, habitual intake of MUFA and PUFA were associated with lower risk of physical performance impairment.

Dystonia-Ataxia with early handwriting deterioration in COQ8A mutation carriers: A case series and literature review.

Cerebellar ataxia is a hallmark of coenzyme Q10 (CoQ10) deficiency associated with COQ8A mutations. We present four patients, one with novel COQ8A pathogenic variants all with early, prominent handwriting impairment, dystonia and only mild ataxia. To better define the phenotypic spectrum and course of COQ8A disease, we review the clinical presentation and evolution in 47 reported cases. Individuals with COQ8A mutation display great clinical variability and unpredictable responses to CoQ10 supplementation. Onset is typically during infancy or childhood with ataxic features associated with developmental delay or regression. When disease onset is later in life, first symptoms can include: incoordination, epilepsy, tremor, and deterioration of writing. The natural history is characterized by a progression to a multisystem brain disease dominated by ataxia, with disease severity inversely correlated with age at onset. Six previously reported cases share with ours, a clinical phenotype characterized by slowly progressive or static writing difficulties, focal dystonia, and speech disorder, with only minimal ataxia. The combination of writing difficulty, dystonia and ataxia is a distinctive constellation that is reminiscent of a previously described clinical entity called Dystonia Ataxia Syndrome (DYTCA) and is an important clinical indicator of COQ8A mutations, even when ataxia is mild or absent.

Phenotype of carnitine palmitoyltransferase II (CPT II) deficiency: A questionnaire-based survey.

Wide spectrums of symptoms besides muscle weakness, different triggering factors and varied muscles involvement are associated with CPT II deficiency. However, systematic clinical characterization of CPT II deficiency is not known. A Questionnaire-based retrospective study on 13 biochemically and genetically confirmed CPT II deficient patients was performed to analyze these aspects. Attacks of myalgia (13/13 patients), weakness (13/13) and rhabdomyolysis (10/13 patients) were most frequently reported. The number of attacks ranged from 1 to 85/year. Common triggers were exercise (13/13), fasting (13/13), cold (12/13) and infections (12/13). Exercise lasting from 15 to 60 min was sufficient for attacks in 9/13 patients, 1-4 h in 3/13 patients and more than 4 h in 1/13 patient. 2/13 patients required dialysis. Limb muscles were affected slightly more often than other muscles. Mean intensity of pain in visual analogue scale (VAS) during regular attack was 4.77 (±1.36). Frequency and severity of attacks did not increase during the course of disease in 10/13 patients. 7/13 patients quit sports after the symptoms emerged. 3/13 patients changed their profession permanently. Increased number of attacks were positively correlated with higher BMI (P = 0.05). Body rest, carbohydrate-rich nutrients and fluid-supplement mitigated the pain. After the first attack [Mean: 9.7 (±4.46) years], diagnosis took an average of 26.7 (± 13.06) years. In myopathic CPT II deficiency, frequencies of attacks are highly variable. Generally, the myopathic form is a mild form. However, severe patients requiring dialysis due to kidney failure could be present. Individuals with higher BMI are at risk of developing more frequent attacks.

Antinuclear Matrix Protein 2 Autoantibodies and Edema, Muscle Disease, and Malignancy Risk in Dermatomyositis Patients.

OBJECTIVE: Dermatomyositis (DM) patients typically present with proximal weakness and autoantibodies that are associated with distinct clinical phenotypes. We observed that DM patients with autoantibodies recognizing the nuclear matrix protein NXP-2 often presented with especially severe weakness. The aim of this study was to characterize the clinical features associated with anti-NXP-2 autoantibodies. METHODS: There were 235 DM patients who underwent testing for anti-NXP-2 autoantibodies. Patient characteristics, including muscle strength, were compared between those with and without these autoantibodies. The number of cancer cases observed in anti-NXP-2-positive subjects was compared with the number expected in the general population. RESULTS: Of the DM patients, 56 (23.8%) were anti-NXP-2-positive. There was no significant difference in the prevalence of proximal extremity weakness in patients with and without anti-NXP-2. In contrast, anti-NXP-2-positive patients had more prevalent weakness in the distal arms (35% versus 20%; P = 0.02), distal legs (25% versus 8%; P < 0.001), and neck (48% versus 23%; P < 0.001). Anti-NXP-2-positive subjects were also more likely to have dysphagia (62% versus 35%; P < 0.001), myalgia (46% versus 25%; P = 0.002), calcinosis (30% versus 17%; P = 0.02), and subcutaneous edema (36% versus 19%; P = 0.01) than anti-NXP-2-negative patients. Five anti-NXP-2-positive subjects (9%) had cancer-associated myositis, representing a 3.68-fold increased risk (95% confidence interval 1.2-8.6) compared to the expected prevalence in the general population. CONCLUSION: In DM, anti-NXP-2 autoantibodies are associated with subcutaneous edema, calcinosis, and a muscle phenotype characterized by myalgia, proximal and distal weakness, and dysphagia. As anti-NXP-2-positive patients have an increased risk of cancer, we suggest that they undergo comprehensive cancer screening.

A Questionnaire Study to Assess the Value of the Vulnerable Elders Survey, G8, and Predictors of Toxicity as Screening Tools for Frailty and Toxicity in Geriatric Cancer Patients.

BACKGROUND: The aim of this study was to identify an appropriate screening instrument for the identification of frail elderly patients in a tertiary cancer center. In order to improve cancer care for older patients, the use of a geriatric assessment (GA) has been proposed to identify frail patients or those who are at a higher risk for chemotherapy-related toxicities. In busy clinical routine, an appropriate screening instrument could be used to spare time- and resource-consuming application of GA. PATIENTS AND METHODS: We administered the Vulnerable Elders Survey (VES-13), G8 questionnaire, and Predictors of Toxicity (POT) as well as a GA at the first visit of 84 consecutive patients at a single Comprehensive Cancer Center. Analysis for patients' characteristics as well as sensitivity, specificity, and positive and negative predictive value (npv) was conducted. RESULTS: The median age of the patients was 73 years (range 63-93 years), 61.9% were male, most (63%) suffered from gastrointestinal tumors, 39.3% had a multiple cancer diagnosis, and 53.6% had metastasis. 30 (35.7%) individuals were classified as 'frail' by the GA. Sensitivity of G8 was 38.3%, and the npv was 63.8%. Sensitivity for VES-13 was 57.1%, and npv was 76.3%. Sensitivity of POT was 72.7%, and the npv was 80.6%. CONCLUSION: For the first time, the VES-13, G8, and POT are compared in a sample of older German patients. The POT seems to be a sufficient screening tool to identify frail patients in a tertiary referral cancer center and helps to save time and resources compared with a complete GA.

Physical performance and muscle strength in older patients with and without diabetes from a public hospital in Lima, Peru. / Rendimiento físico y fuerza muscular en pacientes adultos mayores con diabetes y sin diabetes de un hospital público de Lima (Perú).

OBJECTIVE: To assess the relationship between physical performance (PP) and muscle strength (MS) in elderly subjects with and without diabetes in a public hospital of Lima, Peru. SUBJECTS AND METHOD: A cross-sectional analysis of subjects aged 60 years or older with and without diabetes. MS was measured with a handheld dynamometer, and PP with the «timed get-up-and-go¼ test. Nutritional status was determined using body mass index, body fat percentage measured with a handheld fat loss monitor and protein intake based on the 24-hour recall. Age, sex, and history of hospitalization and supplementation were also recorded. The association was assessed using adjusted prevalence ratios. RESULTS: Overall, 139 patients with diabetes (26.6% with low PP and 13.7% with decreased MS) and 382 subjects without diabetes (36.6% with low PP and 23.0% with decreased MS) were evaluated. No association was found between T2DM and MS (aPR: 0.99; 95% CI: 0.67-1.57) or PP (aPR: 1.13; 95% CI: 0.84-1.52). Protein and supplement consumption was also unrelated (P>.05); however, history of hospitalization, age, sex, nutritional status, and body fat percentage were related (P>.05). CONCLUSIONS: No association was found between T2DM, MS, and PP. However, low PP was associated to female sex and overweight/obesity, and decreased MS was associated to high body fat percentage and underweight. Moreover, MS and PP were related to older age and history of hospitalization.

Normativa SEPAR sobre disfunción muscular de los pacientes con enfermedad pulmonar obstructiva crónica / Guidelines for the evaluation and treatment of muscle dysfunction in patients with chronic obstructive pulmonary disease

La disfunción muscular de pacientes con enfermedad pulmonar obstructiva crónica (EPOC) constituye una de las comorbilidades más importantes, con repercusiones negativas en su capacidad de ejercicio y calidad de vida. En la presente normativa se ha resumido la literatura publicada más recientemente sobre los diferentes aspectos del tema y se ha utilizado también la escala Grading of Recommendations Assessment, Development, and Evaluation (GRADE) de recomendaciones sobre el grado de evidencia de las diferentes propuestas de la normativa. Respecto a una población control, se estima que en un tercio de los pacientes EPOC la fuerza del cuádriceps es un 25% inferior incluso en estadios precoces de su enfermedad. Aunque tanto los músculos respiratorios como los de las extremidades están alterados, estos últimos suelen verse mayormente afectados. Diversos factores y mecanismos biológicos están involucrados en la disfunción muscular de los pacientes. Se proponen diversas pruebas para evaluar y diagnosticar el grado de afectación de los músculos respiratorios y de las extremidades (periféricos), así como identificar la capacidad de esfuerzo de los pacientes (prueba de marcha de 6 min y cicloergometría). Se describen también las posibles estrategias terapéuticas vigentes que incluyen las diversas modalidades de entrenamiento y de soporte farmacológico y nutricional

In patients with chronic obstructive pulmonary disease (COPD), skeletal muscle dysfunction is a major comorbidity that negatively impacts their exercise capacity and quality of life. In the current guidelines, the most recent literature on the various aspects of COPD muscle dysfunction has been included. The Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) scale has been used to make evidence-based recommendations on the different features. Compared to a control population, one third of COPD patients exhibited a 25% decline in quadriceps muscle strength, even at early stages of their disease. Although both respiratory and limb muscles are altered, the latter are usually more severely affected. Numerous factors and biological mechanisms are involved in the etiology of COPD muscle dysfunction. Several tests are proposed in order to diagnose and evaluate the degree of muscle dysfunction of both respiratory and limb muscles (peripheral), as well as to identify the patients’ exercise capacity (six-minute walking test and cycloergometry). Currently available therapeutic strategies including the different training modalities and pharmacological and nutritional support are also described

Frailty in childhood cancer survivors.

Young adult childhood cancer survivors are at an increased risk of frailty, a physiologic phenotype typically found among older adults. This phenotype is associated with new-onset chronic health conditions and mortality among both older adults and childhood cancer survivors. Mounting evidence suggests that poor fitness, muscular weakness, and cognitive decline are common among adults treated for childhood malignancies, and that risk factors for these outcomes are not limited to those treated with cranial radiation. Although the pathobiology of this phenotype is not known, early cellular senescence, sterile inflammation, and mitochondrial dysfunction in response to initial cancer or treatment-related insults are hypothesized to play a role. To the authors' knowledge, interventions to prevent or remediate frailty among childhood cancer survivors have not been tested to date. Pharmaceutical, nutraceutical, and lifestyle interventions have demonstrated some promise.

Nitrogen-bisphosphonate therapy is linked to compromised coenzyme Q10 and vitamin E status in postmenopausal women.

BACKGROUND: Nitrogen-bisphosphonates (N-BPs) are the most widely used drugs for bone fragility disorders. Long-term or high-dose N-BP use is associated with unusual serious side effects such as osteonecrosis of the jaw, musculoskeletal pain, and atypical fractures of long bones. It has escaped notice that the pathway N-BPs block is central for the endogenous synthesis of coenzyme Q10, an integral enzyme of the mitochondrial respiratory chain and an important lipid-soluble antioxidant. Our objective was to assess the coenzyme Q10 and antioxidant status in relation to N-BP exposure in women with postmenopausal osteoporosis. METHODS: Seventy-one postmenopausal women (age, 73.5 ± 5.5 y) with osteoporosis and no other malignancy were included in this cross-sectional study. Seventeen were treatment naive, 27 were on oral N-BP, and 27 were on i.v. N-BP. RESULTS: Vitamin E γ-tocopherol levels (µmol/mL) were significantly reduced in N-BP users [oral, H(2) = 18.5, P = .02; i.v., H(2) = 25.2, P < .001; mean rank comparisons after Kruskal-Wallis test). Length of time (days) of N-BP exposure, but not age, was inversely associated with the coenzyme Q10/cholesterol ratio (µmol/mol) (ß = -0.27; P = .025), which was particularly low for those on i.v. N-BP (mean difference = -35.0 ± 16.9; 95% confidence interval, -65.2 to -4.9; P = .02). CONCLUSION: The degree of N-BP exposure appears related to compromised coenzyme Q10 status and vitamin E γ-tocopherol levels in postmenopausal women with osteoporosis. This phenomenon may link to certain adverse N-BP-associated effects. Confirmation of this would suggest that therapeutic supplementation could prevent or reverse certain complications of long-term N-BP therapy for at-risk individuals.

Neuromuscular electrical stimulation for preventing skeletal-muscle weakness and wasting in critically ill patients: a systematic review.

BACKGROUND: Neuromuscular electrical stimulation (NMES) therapy may be useful in early musculoskeletal rehabilitation during acute critical illness. The objective of this systematic review was to evaluate the effectiveness of NMES for preventing skeletal-muscle weakness and wasting in critically ill patients, in comparison with usual care. METHODS: We searched PubMed, CENTRAL, CINAHL, Web of Science, and PEDro to identify randomized controlled trials exploring the effect of NMES in critically ill patients, which had a well-defined NMES protocol, provided outcomes related to skeletal-muscle strength and/or mass, and for which full text was available. Two independent reviewers extracted data on muscle-related outcomes (strength and mass), and participant and intervention characteristics, and assessed the methodological quality of the studies. Owing to the lack of means and standard deviations (SDs) in some studies, as well as the lack of baseline measurements in two studies, it was impossible to conduct a full meta-analysis. When means and SDs were provided, the effect sizes of individual outcomes were calculated, and otherwise, a qualitative analysis was performed. RESULTS: The search yielded 8 eligible studies involving 172 patients. The methodological quality of the studies was moderate to high. Five studies reported an increase in strength or better preservation of strength with NMES, with one study having a large effect size. Two studies found better preservation of muscle mass with NMES, with small to moderate effect sizes, while no significant benefits were found in two other studies. CONCLUSIONS: NMES added to usual care proved to be more effective than usual care alone for preventing skeletal-muscle weakness in critically ill patients. However, there is inconclusive evidence for its benefit in prevention of muscle wasting.

Coenzyme Q10 deficiency in mitochondrial DNA depletion syndromes.

We evaluated coenzyme Q10 (CoQ) levels in patients studied under suspicion of mitochondrial DNA depletion syndromes (MDS) (n=39). CoQ levels were quantified by HPLC, and the percentage of mtDNA depletion by quantitative real-time PCR. A high percentage of MDS patients presented with CoQ deficiency as compared to other mitochondrial patients (Mann-Whitney-U test: p=0.001). Our findings suggest that MDS are frequently associated with CoQ deficiency, as a possible secondary consequence of disease pathophysiology. Assessment of muscle CoQ status seems advisable in MDS patients since the possibility of CoQ supplementation may then be considered as a candidate therapy.

The clinical and biochemical presentation of vitamin D deficiency and insufficiency in children and adolescents.

AIM: To evaluate the clinical and biochemical findings of the children and adolescents with vitamin D deficiency and insufficiency in order to determine the clinical and biochemical presentation differences between age groups. METHODS: This retrospective study included a review of medical reports of 543 patients (aged between 1-17 years) who were referred to our hospital between October 2011 and May 2012 with symptoms related to vitamin D deficiency or insufficiency. The patients were divided into four groups by age: 1-3 years (Group 1), 4-6 years (Group 2), 7-11 years (Group 3) and 12-17 years (Group 4). Patients diagnosed with vitamin D deficiency or insufficiency were evaluated as to their clinical and biochemical findings. RESULTS: Gender distribution were not statistically different between the four groups. The mean ages of Groups 1-4 were 1.9±0.7, 5.1±0.9, 8.9±1.3, 13.1±1.1, respectively. Major complaints on admission were muscle weakness (91%), low weight gain (failure to thrive) (89%), head deformity (frontal bossing) (35.6%), bone deformity (enlargement of wrist and ankles) (29.7%) for Group 1. Muscle weakness (76%) and low weight gain (failure to thrive) (68%) for Group 2. Leg and chest pain were the major symptoms in Group 3 (57% and 28%, respectively) and in Group 4 (26% and 55%, respectively) as well as high rates of obesity (31% and 63%). The biochemical findings of vitamin D deficiency mostly appeared in the first group who developed vitamin D deficiency due to the lack of vitamin D supplementation. However, in older children, the majority of the patients had low 25 hydroxyvitamin D (25 OHD) values without evidence of biochemical findings of osteomalacia. CONCLUSION: Depending on the degree of deficiency and insufficiency, and the age of the patients, the clinical and biochemical findings varied widely. Children under the age of 3 who either never received vitamin D supplementation or who had been receiving supplementation that was stopped too early were at a greater risk for developing clinically and biochemically proved vitamin D deficiency. In older children, low vitamin D levels mostly resulted in subtle complaints without abnormal biochemical findings.

Protocol for diaphragm pacing in patients with respiratory muscle weakness due to motor neurone disease (DiPALS): a randomised controlled trial.

BACKGROUND: Motor neurone disease (MND) is a devastating illness which leads to muscle weakness and death, usually within 2-3 years of symptom onset. Respiratory insufficiency is a common cause of morbidity, particularly in later stages of MND and respiratory complications are the leading cause of mortality in MND patients. Non Invasive Ventilation (NIV) is the current standard therapy to manage respiratory insufficiency. Some MND patients however do not tolerate NIV due to a number of issues including mask interface problems and claustrophobia. In those that do tolerate NIV, eventually respiratory muscle weakness will progress to a point at which intermittent/overnight NIV is ineffective. The NeuRx RA/4 Diaphragm Pacing System was originally developed for patients with respiratory insufficiency and diaphragm paralysis secondary to stable high spinal cord injuries. The DiPALS study will assess the effect of diaphragm pacing (DP) when used to treat patients with MND and respiratory insufficiency. METHOD/DESIGN: 108 patients will be recruited to the study at 5 sites in the UK. Patients will be randomised to either receive NIV (current standard care) or receive DP in addition to NIV. Study participants will be required to complete outcome measures at 5 follow up time points (2, 3, 6, 9 and 12 months) plus an additional surgery and 1 week post operative visit for those in the DP group. 12 patients (and their carers) from the DP group will also be asked to complete 2 qualitative interviews. DISCUSSION: The primary objective of this trial will be to evaluate the effect of Diaphragm Pacing (DP) on survival over the study duration in patients with MND with respiratory muscle weakness. The project is funded by the National Institute for Health Research, Health Technology Assessment (HTA) Programme (project number 09/55/33) and the Motor Neurone Disease Association and the Henry Smith Charity. TRIAL REGISTRATION: Current controlled trials ISRCTN53817913. The views and opinions expressed therein are those of the authors and do not necessarily reflect those of the HTA programme, NIHR, NHS or the Department of Health.

Upper-body morbidity after breast cancer: incidence and evidence for evaluation, prevention, and management within a prospective surveillance model of care.

The purpose of this paper is to review the incidence of upper-body morbidity (arm and breast symptoms, impairments, and lymphedema), methods for diagnosis, and prevention and treatment strategies. It was also the purpose to highlight the evidence base for integration of prospective surveillance for upper-body morbidity within standard clinical care of women with breast cancer. Between 10% and 64% of women report upper-body symptoms between 6 months and 3 years after breast cancer, and approximately 20% develop lymphedema. Symptoms remain common into longer-term survivorship, and although lymphedema may be transient for some, those who present with mild lymphedema are at increased risk of developing moderate to severe lymphedema. The etiology of morbidity seems to be multifactorial, with the most consistent risk factors being those associated with extent of treatment. However, known risk factors cannot reliably distinguish between those who will and will not develop upper-body morbidity. Upper-body morbidity may be treatable with physical therapy. There is also evidence in support of integrating regular surveillance for upper-body morbidity into the routine care provided to women with breast cancer, with early diagnosis potentially contributing to more effective management and prevention of progression of these conditions.

Los suplementos micronutricionales en la DMAE / Micronutritional supplementation in the ARMD

No disponible

Prevalence of clinically significant functional fatigue or weakness in specialty outpatient clinics of Pune, India.

Disorders of fatigue are important in clinical practice but inadequately studied in developing countries. Questions about their consistency and variation across cultures also require attention. The standard professional diagnostic formulations of these disorders, namely, chronic fatigue syndrome and neurasthenia, are not used widely in India, perhaps due to lack of research and poor appreciation of their clinical significance. Recognising patients with clinically significant functional fatigue or weakness often seek help from various care-givers, prevalence of this condition was studied in four specialty clinics of Sassoon Hospital, Pune. An operationally defined set of criteria was used to create a screening instrument. Trained research assistants surveyed 1,874 consecutive patients from psychiatry, medicine, dermatology, and ayurved clinics. Data were entered and analysed to compute the rates of this condition, compare them across clinics and between sexes, and to compute rates adjusted for age, sex, and the clinic attended. Overall prevalence was 5.02% with higher rates in the dermatology and ayurved clinics than in psychiatry and medicine clinics. The female preponderance (63.83%) was notable (p < 0.001). Mean age of patients with this condition was similar across clinics. Logistic regression showed female sex (OR 2.19, 95% CI 1.41 to 3.40) and dermatology clinic (OR 1.70, 1.02 to 2.85) to be significant predictors of CS-FoW. Female preponderance indicates the need for studies with gender focus. Clinical and cultural epidemiological studies informing psychiatrists as well as other physicians are necessary. Need for counselling for majority of these patients calls for appropriate changes in healthcare delivery.