A validation study of a modified Bouchard activity record that extends the concept of 'uptime' to Rett syndrome

Aim - The aim of this study was to investigate the validity of using a Bouchard activity record (BAR) in individuals with Rett syndrome to measure physical activity, as compared with pragmatic criterion standard measures of walking status and step counts recorded using the StepWatch activity monitor (SAM). Method - During the waking hours of 1 day, 43 females (mean age 21y, SD 9y) wore a SAM whilst a proxy completed a modified BAR. Responses to the BAR were compared among participants, who were grouped according to walking status, using the Mann–Whitney two-sample rank-sum test. Relationships were sought between BAR responses and step counts using linear regression. Results - According to the proxy-reported BAR responses, those who needed assistance with walking spent more time sitting (median [interquartile range] 9h 15min [8h 8min–10h 30min] vs 6h 15min [4h 15min–8h 30min]; p<0.001) and less time standing (1h [38min–1h 30min] vs 2h 15min [45min–3h 45min]; p=0.04) than those who could walk independently. In those who could walk independently, time classified as ‘uptime’ (standing and walking) using the BAR was associated with increased step count (r2=0.58; p<0.001). Interpretation - These data support the validity of proxy-reported BAR responses. In those who could walk independently, uptime, classified using the BAR, could be used to estimate daily step count. This tool offers an inexpensive method for clinicians to gain insights into physical activity levels in individuals with Rett syndrome

Valsartan Improves Endothelial Dysfunction in Hypertension: A Randomized, Double-Blind Study

Endothelial dysfunction can predict cardiac outcomes in hypertension and reversing this abnormality has become an attractive therapeutic objective. We tested the hypothesis that blocking the angiotensin type 1 (AT1) receptor with valsartan in comparison with amlodipine would lead to an improvement in forearm resistance artery endothelial dysfunction. In total, 25 hypertensive subjects (mean age 60 years, SD 8) with a mean daytime ambulatory blood pressure (BP) of 154 (10)/97 (6) mmHg were randomized following a 3-week placebo run-in period to a double-blind, crossover trial of 16-week treatment periods with either valsartan or amlodipine, separated by a 3-week washout period. Intra-arterial infusions of acetylcholine (ACh) and NG-monomethyl-L-arginine (L-NMMA) were used to assess stimulated and basal endothelium-dependent nitric oxide (NO) release, respectively. Coinfusion of ACh and L-NMMA was employed to investigate the existence of an NO-independent vasodilatory pathway. Valsartan and amlodipine each lowered the clinical BP to the same extent (139 [7]/87 [6] and 139 [11]/89 [4] mmHg, respectively). The vasodilatory response to ACh was significantly increased with valsartan (maximal percentage change in forearm blood flow (max. ΔFBF%) 301 [47] vs. 185 [34], mean [SEM]; P < 0.05) as compared with placebo, but remained unchanged with amlodipine. Both valsartan and amlodipine similarly increased the vasoconstrictive response to L-NMMA (max. ΔFBF%–43 [5], −42 [5], respectively, vs. –26 [3] baseline; P < 0.001). The vasodilatory response after coinfusion of ACh and L-NMMA was significantly (P < 0.05) enhanced only with valsartan. Valsartan reserved peripheral endothelial dysfunction through both NO-dependent and -independent pathways, while for the same degree of BP control, amlodipine had only a partial effect on NO bioactivity

Glucocorticoid receptor alters isovolumetric contraction and restrains cardiac fibrosis

Corticosteroids directly affect the heart and vasculature and are implicated in the pathogenesis of heart failure. Attention is focussed upon the role of the mineralocorticoid receptor (MR) in mediating pro-fibrotic and other adverse effects of corticosteroids upon the heart. In contrast, the role of the glucocorticoid receptor (GR) in the heart and vasculature is less well understood. We addressed this in mice with cardiomyocyte and vascular smooth muscle deletion of GR (SMGRKO mice). Survival of SMGRKO mice to weaning was reduced compared with that of littermate controls. Doppler measurements of blood flow across the mitral valve showed an elongated isovolumetric contraction time in surviving adult SMGRKO mice, indicating impairment of the initial left ventricular contractile phase. Although heart weight was elevated in both genders, only male SMGRKO mice showed evidence of pathological cardiomyocyte hypertrophy, associated with increased myosin heavy chain-β expression. Left ventricular fibrosis, evident in both genders, was associated with elevated levels of mRNA encoding MR as well as proteins involved in cardiac remodelling and fibrosis. However, MR antagonism with spironolactone from birth only modestly attenuated the increase in pro-fibrotic gene expression in SMGRKO mice, suggesting that elevated MR signalling is not the primary driver of cardiac fibrosis in SMGRKO mice, and cardiac fibrosis can be dissociated from MR activation. Thus, GR contributes to systolic function and restrains normal cardiac growth, the latter through gender-specific mechanisms. Our findings suggest the GR:MR balance is critical in corticosteroid signalling in specific cardiac cell types

Development of spasticity with age in a total population of children with cerebral palsy

<p>Abstract</p> <p>Background</p> <p>The development of spasticity with age in children with cerebral palsy (CP) has, to our knowledge, not been studied before. In 1994, a register and a health care program for children with CP in southern Sweden were initiated. In the programme the child's muscle tone according to the modified Ashworth scale is measured twice a year until six years of age, then once a year. We have used this data to analyse the development of spasticity with age in a total population of children with cerebral palsy.</p> <p>Methods</p> <p>All measurements of muscle tone in the gastrocnemius-soleus muscle in all children with CP from 0 to 15 years during the period 1995–2006 were analysed. The CP subtypes were classified according to the Surveillance of Cerebral Palsy in Europe network system. Using these criteria, the study was based on 6218 examinations in 547 children. For the statistical analysis the Ashworth scale was dichotomized. The levels 0–1 were gathered in one category and levels 2–4 in the other. The pattern of development with age was evaluated using piecewise logistic regression in combination with Akaike's An Information Criterion.</p> <p>Results</p> <p>In the total sample the degree of muscle tone increased up to 4 years of age. After 4 years of age the muscle tone decreased each year up to 12 years of age. A similar development was seen when excluding the children operated with selective dorsal rhizotomy, intrathecal baclofen pump or tendo Achilles lengthening. At 4 years of age about 47% of the children had spasticity in their gastro-soleus muscle graded as Ashworth 2–4. After 12 years of age 23% of the children had that level of spasticity. The CP subtypes spastic bilateral and spastic unilateral CP showed the same pattern as the total sample. Children with dyskinetic type of CP showed an increasing muscle tone up to age 6, followed by a decreasing pattern up to age 15.</p> <p>Conclusion</p> <p>In children with CP, the muscle tone as measured with the Ashworth scale increases up to 4 years of age and then decreases up to 12 years of age. The same tendency is seen in all spastic subtypes. The findings may have implications both for clinical judgement and for research studies on spasticity treatment.</p

Survey on worldwide trauma team activation requirement

PURPOSE : trauma team activation (TTA) is thought to be essential for advanced and specialized care of very severely injured patients. However, non-specific TTA criteria may result in overtriage that consumes valuable resources or endanger patients in need of TTA secondary to undertriage. Consequently, criterion standard definitions to calculate the accuracy of the various TTA protocols are required for research and quality assurance purposes. Recently, several groups suggested a list of conditions when a trauma team is considered to be essential in the initial care in the emergency room. The objective of the survey was to post hoc identify trauma-related conditions that are thought to require a specialized trauma team that may be widely accepted, independent from the country’s income level. METHODS : A set of questions was developed, centered around the level of agreement with the proposed post hoc criteria to define adequate trauma team activation. The participants gave feedback before they answered the survey to improve the quality of the questions. The finalized survey was conducted using an online tool and a word form. The income per capita of a country was rated according to the World Bank Country and Lending groups. RESULTS : The return rate was 76% with a total of 37 countries participating. The agreement with the proposed criteria to define post hoc correct requirements for trauma team activation was more than 75% for 12 of the 20 criteria. The rate of disagreement was low and varied between zero and 13%. The level of agreement was independent from the country’s level of income. CONCLUSIONS : The agreement on criteria to post hoc define correct requirements for trauma team activation appears high and it may be concluded that the proposed criteria could be useful for most countries, independent from their level of income. Nevertheless, more discussions on an international level appear to be warranted to achieve a full consensus to define a universal set of criteria that will allow for quality assessment of over- and undertriage of trauma team activation as well as for the validation of field triage criteria for the most severely injured patients worldwide.http://link.springer.com/journal/68am2022Surger

Factors Associated with Revision Surgery after Internal Fixation of Hip Fractures

Background: Femoral neck fractures are associated with high rates of revision surgery after management with internal fixation. Using data from the Fixation using Alternative Implants for the Treatment of Hip fractures (FAITH) trial evaluating methods of internal fixation in patients with femoral neck fractures, we investigated associations between baseline and surgical factors and the need for revision surgery to promote healing, relieve pain, treat infection or improve function over 24 months postsurgery. Additionally, we investigated factors associated with (1) hardware removal and (2) implant exchange from cancellous screws (CS) or sliding hip screw (SHS) to total hip arthroplasty, hemiarthroplasty, or another internal fixation device. Methods: We identified 15 potential factors a priori that may be associated with revision surgery, 7 with hardware removal, and 14 with implant exchange. We used multivariable Cox proportional hazards analyses in our investigation. Results: Factors associated with increased risk of revision surgery included: female sex, [hazard ratio (HR) 1.79, 95% confidence interval (CI) 1.25-2.50; P = 0.001], higher body mass index (fo

Looking in the mirror: second generation, Greek women reflecting on their identity

Society is constructed of - and - by a multiplicity of individuals, each of whom maintain their own values, beliefs, traditions and ambitions. Individual identity therefore is socially created and operates on two levels - collective and individual. Time, place, the family and one's parents, patterns of interaction between the individual and her environment, and an individual's sense of communal and self worth, all work to re-define one's identity. Individual identity functions in a similar fashion to a chameleon, constantly changing its colour, readjusting itself to its surroundings. Similarly, the individual him/herself is a prism of different colours and faces, images of reality. S/he is an amalgam of intercepting multiple identities. Thus, to achieve a more limpid reflection of the women studied in this dissertation, one must examine the 'points of intersection', where the individual interacts with, and responds to, collective images of ethnic, Greek, gender, and bi-cultural identities

Exploring Essential Orthopaedics

Orthopaedic surgeons have been noted to have the power of a bull and, unfairly, the brains to match. In fact orthopaedics is the most logical and rewarding of the medical specialties. The surgeons involved are craftsmen (and now women) who may be involved in a degree of carpentry, but the result is something to be admired and preserved for prosperity in pictures. It is a true art. There is a hint of jealousy in these scandalous comments on the orthopaedic intellect. Students introduced to orthopaedics generally find it enjoyable, especially when it is opposed to a specialty that mixes potions, forces them upon unwitting victims and then awaits the side-effects. It is black and white versus shades of grey, but students have great difficulty learning degrees rather than stark contrasts.Orthopaedics has been divided into ‘medicine’ and ‘surgery’ but generally, orthopaedic medicine is handled by a rheumatologist. Sports physicians also treat a number of conditions related to orthopaedic medicine. The orthopaedic surgeon is more concerned with the surgical conditions – not surgery. He or she operates on only a small percentage of patients they see. Those that would benefit from surgery (and not be made worse) must be carefully selected – another part of the art. This makes the bovine comparison even more unfair. But don’t get between the orthopod and a gate. This program explores the aspects of orthopaedic surgery that are essential to hopeful or current clinicians