Hoppermarshall1945

Although differences in pre- and post-implementation survey scores were statistically insignificant, the program was welcomed by leadership and staff.

The program experienced higher utilization compared to similar launch studies, with eight encounters in the first month. Impact on staff morale is expected to increase; long-term peer support can improve provider well-being and patient outcomes.

The program experienced higher utilization compared to similar launch studies, with eight encounters in the first month. Impact on staff morale is expected to increase; long-term peer support can improve provider well-being and patient outcomes.The aim of the study was to establish 24-month changes in a large cohort of type II and III spinal muscular atrophy (SMA) patients assessed with the Revised Upper Limb Module (RULM), a tool specifically developed to assess upper limb function in SMA. We included 107 patients (54 type II and 53 type III) with at least 24-months follow up. The overall RULM 24-month changes showed a mean decline of -0.79 points. The difference between baseline and 24 months was significant in type II but not in type III patients. There was also a difference among functional subgroups but not in relation to age. Most patients had 24-month mean changes within 2 points, with 23% decreasing more than 2 points and 7% improving by >2 points. Our results suggest an overall progressive decline in upper limb function over 24 months. The negative changes were most notable in type II, in non-ambulant type III and with a different pattern of progression, also in non-sitter type II. In contrast, ambulant type III showed relative stability within the 24-month follow up. These findings will help in the interpretation of the real world data collected following the availability of new therapeutic approaches.Therapeutic advances in hereditary amyloid transthyretin (ATTRv) amyloidosis with polyneuropathy extended life expectancy and delayed symptom progression especially in patients with early disease. Thus, detection and monitoring of asymptomatic carriers gained importance. However, there is still limited consensus on genetic screening of ATTRv-polyneuropathy patients' family members and diagnostic tests that must be done in the follow-up. In this study, we followed prospectively five asymptomatic carriers of a family with ATTRV30M (p.Val50Met) mutation by different diagnostic tests for three years. The carriers were followed by neurological examination, nerve conduction studies, sympathetic skin response test, heart rate variability, SFN-SIQ and DN4 questionnaires, quantitative sensory testing (QST), skin biopsy and in vivo corneal confocal microscopy. Nerve conduction studies, sympathetic skin response test and heart rate variability were normal in all for three years. Baseline QST and SFN-SIQ were normal but became abnormal during follow-up of two individuals who developed small fiber neuropathy symptoms. Baseline intraepidermal nerve fiber density was low in three carriers and decreased to below normative values in all during follow-up, while corneal sub-basal nerve density was low in all carriers compared to controls during the entire follow-up. Thus, our study showed that SFN-SIQ and QST are useful diagnostic tools to detect the transition to symptomatic ATTRv-polyneuropathy.ALG2 mutations are extremely rare causes of congenital myasthenic syndromes (CMS). The clinical phenotype and treatment response is therefore not well described. We present the case of a baby who immediately after birth presented with pronounced truncal hypotonia, proximal muscle weakness and feeding difficulties. Single fibre electromyography showed neuromuscular transmission failure and salbutamol and ephedrine treatment improved both muscle weakness and neuromuscular transmission. Genetic analysis revealed a likely pathogenic variant c.1040del, p.(Gly347Valfs*27) in exon 2 and a variant of uncertain significance, c.239G>A, p.(Gly80Asp) in exon 1 of the ALG2 gene. Western blot in whole cell lysates of HEK293 cells transfected with p.Gly80Asp, or p.Gly347Valfs*27 expression constructs indicated that p.Gly347Valfs*27 is likely a null allele and p.Gly80Asp is pathogenic through marked reduction of ALG2 expression. This case highlights the utility of functional studies in clarifying variants of unknown significance, in suspected cases of CMS.Eosinophilic myositis belong to the idiopathic inflammatory myopathies and are defined by an inflammatory infiltrate composed of eosinophils within the muscle. To date, no consensus exists for diagnosis and care of such patients. The aim of this review was to describe clinical and histological presentation, treatment, and outcome of eosinophilic myositis based on a systematic review of all published histologically proven cases of eosinophilic myositis. A total of 453 records were identified in MEDLINE until November 2020. A total of 69 published cases were identified. The analysis of these allowed the distinction of the 3 previously described pathological subtypes focal eosinophilic myositis (n = 17); diffuse eosinophilic myositis (n = 36); and eosinophilic perimyositis (n = 16). We propose a simple algorithm for diagnosis and treatment strategy for the care of patient with muscular symptoms and blood eosinophilia. This work also highlights eosinophilic myositis pathogenesis and the need for careful investigations in order to rule out differential diagnoses.

The optimal timing of adjuvant chemotherapy (AC) in non-metastatic colon cancer is poorly defined. Delays in AC result in decreased survival. Effective cytotoxic treatments should be considered during the perioperative phase of care. The immediate adjuvant chemotherapy (IAC) concept intends to capitalize on the therapeutic benefits that can be achieved in the perioperative period. We aim to demonstrate that IAC is safe and tolerable.

Microsatellite stable invasive adenocarcinomas were treated with intravenous Leucovorin 20 mg/m2 and single dose of 5-Flurouracil 400mg/m2 at the time of surgery. High-risk stage II and stage III received the first dose of standard AC at 14 days after surgery. Serial measurements of blood-based biomarkers were measured. Quality of life (QOL) was measured using EORTC QLQ-C30.

Of the 20 patients recruited, 40% had final pathology of stage III, 40% stage II and 20% stage I. All patients received intra-operative chemotherapy with no associated morbidity. MLN8054 Median length of stay was 2 days (range of 2-4). There was no intraoperative morbidity with 5% (N=1) grade 3 complication. AC was administered to 65% of patients. The median time to AC was 14 days (range 14-36). Overall quality of life and health scores were similar before surgery and at 30-day postoperatively (P < .05).

A protocol based on IAC starting at the time of surgical resection was found to be safe and feasible with no adverse effects on surgical morbidity or quality of life. Further prospective studies are needed to explore the oncologic benefit of this novel systemic treatment approach.

A protocol based on IAC starting at the time of surgical resection was found to be safe and feasible with no adverse effects on surgical morbidity or quality of life. Further prospective studies are needed to explore the oncologic benefit of this novel systemic treatment approach.The aim of this study was to measure the inter- and intraobserver variations as well as integrality of the Zwipp, Crosby-Fitzgibbons, Sanders, and Eastwood-Atkins classification systems based on more accurate CT scans. Five hundred and forty-nine patients with intra-articular calcaneal fractures from January 2018 to December 2019 taken from a database in our level-I trauma center (3 affiliated hospitals) were included. For each case, normative CT (1 mm slices) scans were available. Four different observers reviewed all CT scans 2 times according to these 4 most prevalent fracture classification systems (FCSs) within a 2-month interval. For these 4 FCSs, the kappa [κ] coefficient was used to evaluate interobserver reliability and intraobserver reproducibility, and the percentage that can be classified was used to indicate integrality. The κ values were measured for Zwipp (κ = 0.38 interobserver, κ = 0.61 intraobserver), Crosby-Fitzgibbons (κ = 0.48 interobserver, κ = 0.79 intraobserver), Sanders (κ = 0.40 interobserver, κ = 0.57 intraobserver), and Eastwood-Atkins (κ = 0.44 interobserver, κ = 0.72 intraobserver). Furthermore, the integralities were calculated for Zwipp (100%), Crosby-Fitzgibbons (100%), Sanders (92%) as well as Eastwood-Atkins (89.6%). Compared with previous literatures, CT scanning with higher accuracy can significantly improve intraobserver reproducibility of Zwipp and Eastwood-Atkins FCSs, but it has no positive effect on variability of Sanders FCS and interobserver reliability of Crosby-Fitzgibbons FCS. In terms of integrality, Zwipp and Crosby-Fitzgibbons FCSs appear to be superior to the other 2 FCSs.The Foot and Ankle Ability Measure (FAAM) is a patient-reported outcome measure that is available in several languages. We aim to assess the structural and construct validity of the FAAM with an emphasis on pain and functionality after foot surgery. The activities of daily living (ADL) and Sports subscales of the Finnish version of the FAAM were completed by 182 patients who underwent operative treatment for disorders of the foot. Convergent validity was assessed by principal component analysis using Spearman's correlation coefficient between the FAAM subscales and the principal components (Function-PC and Pain-PC) derived from validated patient-reported outcome measures. Subscales were studied for floor and ceiling effects, internal consistency and unidimensionality. Internal consistency was examined with Cronbach's alpha and the subscale structure with exploratory factor analysis. FAAM-ADL had high correlation with the Function-PC (r = 0.87, 95% confidence interval [CI] 0.81-0.91) and the Pain-PC (r = 0.75, 95% CI 0.65-0.83). FAAM-Sports had moderate correlation (r = 0.64, 95% CI 0.50-0.74) with the Function-PC and high correlation (r = 0.74, 95% CI 0.64-0.82) with the Pain-PC. No floor or ceiling effects were observed. Cronbach's alpha was 0.97 (95% CI 0.96-0.98) for the ADL and 0.93 (95% CI 0.91-0.95) for the Sports subscales. The results supported the unidimensionality of the FAAM-Sports. Within the ADL subscale, 3 factors were identified, suggesting a 3-factor model for the FAAM overall. Results highlighted the inter-relationship of pain and physical function. Further research on longitudinal validity is needed.

Trapeziometacarpal joint osteoarthritis (OA) produces significant functional impairment due to pain and loss of strength in both power and precision grips, but few studies have related radiographic scores to functional and pain-based measures.

To investigate the association between markers of radiographic disease and outcomes for symptomatic and functional disease.

This study in an exploratory analysis of baseline data from the first 100 participants in a clinical trial evaluating the efficacy of combined conservative therapies for base of thumb OA (COMBO).

Functional Index for Hand Osteoarthritis (FIHOA) scores and Visual Analogue Scale (VAS) scores for pain were recorded for the index hand. Bilateral isometric grip and tip-pinch strength measurements were taken, as well as posteroanterior and Eaton stress-view hand radiographs. Generalized estimating equations (GEEs), univariate, and multivariate analyses were used according to whether the data were bilateral or unilateral.

A total of 79 females and 21 males were included, with a median Kellgren-Lawrence (KL) grade of 3 in the index hand.