Ambulatory Patients Research Articles

Unveiling the Respiratory Muscle Strength in Duchenne Muscular Dystrophy: The Impact of Nutrition and Thoracic Deformities, Beyond Spirometry.

Duchenne muscular dystrophy (DMD) is the most prevalent progressive muscular dystrophy, and the guidelines recommend the regular assessment of respiratory muscle function. This study aimed to assess the relationship between maximum inspiratory pressure (MIP), maximum expiratory pressure (MEP) and sniff nasal inspiratory pressure (SNIP) measurements and upright-supine spirometry parameters in children with DMD, the predictability of upright-supine spirometry in terms of diaphragm involvement, and the impact of nutrition on muscle strength. This prospective cross-sectional study examined patients with DMD by comparing upright and supine FVC, MIP, MEP, and SNIP measurements. The effects of the ambulatory status, kyphoscoliosis, chest deformity, and low BMI on respiratory parameters were investigated. Forty-four patients were included in the study. The mean patient age was 10.8 ± 2.9 years. Twenty-five patients were ambulatory. A significant decrease in FVC, FEV1, and FEF25-75 values was detected in the supine position in both ambulatory and non-ambulatory patients (p < 0.05). All patients had low MIP, MEP, and SNIP measurements (less than 60 cm H2O). MIP, MEP, and SNIP values were significantly lower in patients with a low BMI than in those without (p < 0.05). To accurately assess respiratory muscle strength, supine FVC should be combined with upright FVC, MIP, MEP, and SNIP measurements. It is crucial to regularly screen patients for nutrition, as this can significantly affect respiratory muscle function during pulmonology follow-up.

The Impact of Postural and Anthropometric Properties of Foot and Ankle on Physical Performance and Ambulation of Patients with Duchenne Muscular Dystrophy.

Abnormal foot anthropometry and posture of patients with Duchenne Muscular Dystrophy (DMD) can be considered as possible risk factors for performance and ambulation. It was aimed to examine the effects of foot posture and anthropometric characteristics, which deteriorated from the early period, on ambulation and performance of patients with DMD. The foot arch height (FAH), the metatarsal width (MW), subtalar pronation angle, and ankle limitation degree (ALD) were evaluated to determine the foot anthropometric characteristics of the patients. Foot Posture Index-6 (FPI-6) was used to evaluate foot posture. The performance of the patients was determined by the 6-minute walk test (6MWT), the 10-meter walk test (10MWT), and ascend/descend a standard 4-step test, and the ambulation was determined by the North Star Ambulatory Assessment (NSAA). Spearman's correlation coefficient was calculated to assess the relationship between foot anthropometric characteristics and posture, and performance and ambulation. The sample consisted of 48 patients with DMD aged 5.5 to 12 years. Both of the right and left foot FPI-6 scores were associated with all parameters, except descending 4-step. The left FAH was associated with 6MWT and NSAA, and the left MW was associated with 6MWT. The ALD of right foot was associated with 6MWT, ascending/descending 4 steps, and NSAA, and left ankle limitation was associated with NSAA (p<0.05). There was no relationship between other parameters. These findings suggest that postural disorders in the foot and ankle may contribute to the decrease in performance and ambulation in patients with DMD.

Understanding bacterial diversity, infection dynamics, prevention of antibiotic resistance: an integrated study in an Algerian hospital context.

Bacterial infections, particularly bacteremia, urinary tract infections (UTIs), and pus infections, remain among hospitals' most worrying medical problems. This study aimed to explore bacterial diversity, infection dynamics, and antibiotic resistance profiles of bacterial isolates. We analyzed data from 1750 outpatients and 920 inpatients, of whom 1.6% and 8.47% respectively had various bacterial infections. The analysis revealed that UTIs were the most prevalent at 41.01%, particularly affecting women. UTIs also showed a distinct distribution across admission departments, notably in emergency (23.07%) and pediatric (14.10%) units. The most frequently isolated microorganisms were Escherichia coli (E. coli), followed by Klebsiella ornithinolytica. Skin infections followed UTIs, accounting for 35.88% of cases, more prevalent in men, with Staphylococcus aureus (S. aureus) being the primary pathogen (57%). Gram-negative bacteria (GNB) like E. coli and Pseudomonas aeruginosa contributed significantly to skin infections (43%). Bacteremia cases constituted 11.52% of bacterial infections, predominantly affecting women (67%) and linked to GNB (78%). A comparative study of antibiotic susceptibility profiles revealed more pronounced resistance in GNB strains isolated from inpatients, particularly to antibiotics such as Amoxicillin/clavulanic acid, Tetracyclin, Gentamicin, Chloramphenicol, and Ampicillin. In contrast, strains from ambulatory patients showed greater resistance to Colistin. Gram-positive bacteria from hospitalized patients showed higher resistance to quinolones and cephalosporins, while ambulatory strains showed high resistance to aminoglycosides, macrolides, fluoroquinolones, and penicillin. Furthermore, these analyses identified the most effective antibiotics for the empirical treatment of both community-acquired and nosocomial infections. Ciprofloxacin, aztreonam, and amikacin exhibited low resistance rates among GNB, with gentamicin and chloramphenicol being particularly effective for community-acquired strains. For S. aureus, ciprofloxacin, rifampicin, and cefoxitin were especially effective, with vancomycin showing high efficacy against community-acquired isolates and fosfomycin and chloramphenicol being effective for hospital-acquired strains. These results are essential for guiding antibiotic therapy and improving clinical outcomes, thus contributing to precision medicine and antimicrobial stewardship efforts.

Vitamin D Levels and Their Relationship with Ambulation in Patients with Amyotrophic Lateral Sclerosis

Vitamin D Levels and Their Relationship with Ambulation in Patients with Amyotrophic Lateral Sclerosis

Dengue severity by serotype and immune status in 19 years of pediatric clinical studies in Nicaragua.

Dengue virus, a major global health threat, consists of four serotypes (DENV1-4) that cause a range of clinical manifestations from mild to severe and potentially fatal disease. This study, based on 19 years of data from the Pediatric Dengue Cohort Study and Pediatric Dengue Hospital-based Study in Managua, Nicaragua, investigates the relationship of serotype and immune status with dengue severity. Dengue cases were confirmed by molecular, serological, and/or virological methods, and sudy participants 6 months to 17 years old were followed during their hospital stay or as ambulatory patients. We enrolled a total of 15,266 participants, of whom 3,227 (21%) were positive for DENV infection. Of 2,630 cases with serotype result by RT-PCR, 557 corresponded to DENV1, 992 to DENV2, 759 to DENV3 and 322 to DENV4. Severe disease was more prevalent among secondary DENV2 and DENV4 cases, while similar disease severity was observed in both primary and secondary DENV1 and DENV3 cases. According to the 1997 World Health Organization (WHO) severity classification, both DENV2 and DENV3 caused a higher proportion of severe disease compared to other serotypes, whereas DENV3 caused the greatest percentage of severity according to the WHO-2009 classification. DENV2 was associated with increased odds of pleural effusion and low platelet count, while DENV3 was associated with both hypotensive and compensated shock. These findings demonstrate differences in dengue severity by serotype and immune status and emphasize the critical need for a dengue vaccine with balanced effectiveness against all four serotypes, particularly as existing vaccines show variable efficacy by serotype and serostatus.

"If you cannot measure it, you cannot improve it". Outcome measures in Duchenne Muscular Dystrophy: current and future perspectives.

Duchenne Muscular Dystrophy (DMD) is an X-linked recessive neuromuscular disorder primarily affecting males, caused by mutations in the dystrophin gene. The absence of dystrophin protein leads to progressive skeletal muscle degeneration. Recent advances in the therapeutic landscape underscore the need to identify appropriate outcome measures to assess treatment efficacy in ambulant and non-ambulant DMD patients, across clinical and research settings. This is essential for accurately evaluating new treatments and attributing therapeutic benefits.It is crucial to establish a robust correlation between outcome scores and disease progression patterns. This task is challenging since functional test performance may be influenced by different patient's characteristics, including the physiological evolution of the neurodevelopment together with the disease progression. While widely used DMD outcomes such as the North Star Ambulatory Assessment, the 6-Minute Walking Test, the 4 stairs climbed, and the Performance of the Upper Limb exhibit reliability and validity, their clinical significance is influenced by the wide phenotype and progression variability of the disease.We present and discuss the features (relevance, quantifiability, validity, objectivity, reliability, sensitivity, specificity, precision) of available DMD outcome measures, including new potential measures that may be provided by digital tools and artificial intelligence.

Opportunities to improve the diagnosis and treatment of primary hyperparathyroidism: retrospective cohort study.

Although primary hyperparathyroidism (PHPT) is readily diagnosed biochemically and can be cured with low-risk surgery, it is often underrecognized and undertreated. Our objectives were to characterize, within our health system, how often patients with hypercalcemia were evaluated for PHPT and how often patients with PHPT underwent definitive treatment with parathyroidectomy. Ambulatory patients aged 18 years or older seen at our health system between January 2018 and June 2023 with chronic hypercalcemia were identified from the medical record. After excluding causes of secondary hyperparathyroidism, the proportion of patients with parathyroid hormone (PTH) tests was calculated. Among patients with biochemical evidence of PHPT, the proportion of patients who underwent parathyroidectomy was calculated. Multivariable logistic regression was used to identify factors associated with an evaluation for PHPT and, separately, with parathyroidectomy. Of 7,675 patients with chronic hypercalcemia, 3,323 (43.3%) had a PTH test obtained within 6 months. An age between 40-49 vs. <30 years [(odds ratio (OR) =3.2; 95% confidence interval (CI): 1.8-5.6; P<0.001], a serum calcium level between 11.6-12.0 vs. <11.0 mg/dL (OR =3.9; 95% CI: 3.2-4.7; P<0.001), and osteoporosis (OR =3.1; 95% CI: 2.7-3.5; P<0.001) were associated with an evaluation for PHPT. Among those with PTH levels, 1,327 (39.9%) had PHPT but only 916 (69.0%) were recognized. Three hundred and forty-five (26.0%) patients with PHPT underwent parathyroidectomy. An increasing number of surgical indications was associated with parathyroidectomy (P<0.001), though overall rates remained less than 40%. Among indications for surgery, including age and serum total calcium level, only osteoporosis was associated with parathyroidectomy (OR =2.0; 95% CI: 1.4-2.8; P<0.001). In this study, more than half of patients with chronic hypercalcemia were not evaluated for PHPT. Among patients with biochemical evidence of PHPT, one-third were unrecognized and only one-in-four received curative treatment. Opportunities to improve the management of PHPT exist within our large integrated health system.

Early ambulation after total knee arthroplasty: a retrospective single-center study

PurposeEarly ambulation is an important step in accelerating post-joint replacement surgery recovery. However, there is limited research on populations who are unable to walk immediately after the operation. The purpose of this study was to determine the factors influencing postoperative ambulation in total knee arthroplasty (TKA) patients.MethodsPrimary TKA patients were included in this retrospective study. All patients were divided into two groups. Patients who began walking within 24 h were categorized as the early ambulation group, while patients who began walking after 24 h were classified as the late ambulation group. Recorded demographic data included age, gender, body mass index (BMI), clinical diagnosis, and comorbidities. Hematological parameters potentially affecting patients’ preoperative physical condition were also documented. Additionally, intraoperative metrics such as surgical time, surgical side, tourniquet time, intraoperative blood loss, the placement of drains, and prosthetic model were recorded.ResultsA total of 453 patients (79.0% female, 21.0% male) were included in this study. The average age of all patients was 68.5±7.9 years, ranging from 36 to 87 years, with an average BMI of 27.2±9.9 kg/m2\\documentclass[12pt]{minimal} \\usepackage{amsmath} \\usepackage{wasysym} \\usepackage{amsfonts} \\usepackage{amssymb} \\usepackage{amsbsy} \\usepackage{mathrsfs} \\usepackage{upgreek} \\setlength{\\oddsidemargin}{-69pt} \\begin{document}$$\\mathrm {m^{2}}$$\\end{document}. The mean postoperative ambulation time was 1.6 days, with a range of 0–4 days. In univariate group comparisons, an increase in postoperative time to ambulation was significantly associated with a history of heart disease (P<0.001\\documentclass[12pt]{minimal} \\usepackage{amsmath} \\usepackage{wasysym} \\usepackage{amsfonts} \\usepackage{amssymb} \\usepackage{amsbsy} \\usepackage{mathrsfs} \\usepackage{upgreek} \\setlength{\\oddsidemargin}{-69pt} \\begin{document}$$P<0.001$$\\end{document}), stroke history (P=0.003\\documentclass[12pt]{minimal} \\usepackage{amsmath} \\usepackage{wasysym} \\usepackage{amsfonts} \\usepackage{amssymb} \\usepackage{amsbsy} \\usepackage{mathrsfs} \\usepackage{upgreek} \\setlength{\\oddsidemargin}{-69pt} \\begin{document}$$P=0.003$$\\end{document}), and prior surgeries (P=0.003\\documentclass[12pt]{minimal} \\usepackage{amsmath} \\usepackage{wasysym} \\usepackage{amsfonts} \\usepackage{amssymb} \\usepackage{amsbsy} \\usepackage{mathrsfs} \\usepackage{upgreek} \\setlength{\\oddsidemargin}{-69pt} \\begin{document}$$P=0.003$$\\end{document}). Patients who delayed ambulation also exhibited significantly higher coagulation-related parameters including PT (P<0.001\\documentclass[12pt]{minimal} \\usepackage{amsmath} \\usepackage{wasysym} \\usepackage{amsfonts} \\usepackage{amssymb} \\usepackage{amsbsy} \\usepackage{mathrsfs} \\usepackage{upgreek} \\setlength{\\oddsidemargin}{-69pt} \\begin{document}$$P<0.001$$\\end{document}), APTT (P=0.002\\documentclass[12pt]{minimal} \\usepackage{amsmath} \\usepackage{wasysym} \\usepackage{amsfonts} \\usepackage{amssymb} \\usepackage{amsbsy} \\usepackage{mathrsfs} \\usepackage{upgreek} \\setlength{\\oddsidemargin}{-69pt} \\begin{document}$$P=0.002$$\\end{document}), TT (P=0.039\\documentclass[12pt]{minimal} \\usepackage{amsmath} \\usepackage{wasysym} \\usepackage{amsfonts} \\usepackage{amssymb} \\usepackage{amsbsy} \\usepackage{mathrsfs} \\usepackage{upgreek} \\setlength{\\oddsidemargin}{-69pt} \\begin{document}$$P=0.039$$\\end{document}) before surgery compared to those who mobilized early. Furthermore, prolonged surgical time (P=0.030\\documentclass[12pt]{minimal} \\usepackage{amsmath} \\usepackage{wasysym} \\usepackage{amsfonts} \\usepackage{amssymb} \\usepackage{amsbsy} \\usepackage{mathrsfs} \\usepackage{upgreek} \\setlength{\\oddsidemargin}{-69pt} \\begin{document}$$P= 0.030$$\\end{document}), increased intraoperative blood loss (P<0.001\\documentclass[12pt]{minimal} \\usepackage{amsmath} \\usepackage{wasysym} \\usepackage{amsfonts} \\usepackage{amssymb} \\usepackage{amsbsy} \\usepackage{mathrsfs} \\usepackage{upgreek} \\setlength{\\oddsidemargin}{-69pt} \\begin{document}$$P<0.001$$\\end{document}), and the placement of intraoperative drains (P<0.001\\documentclass[12pt]{minimal} \\usepackage{amsmath} \\usepackage{wasysym} \\usepackage{amsfonts} \\usepackage{amssymb} \\usepackage{amsbsy} \\usepackage{mathrsfs} \\usepackage{upgreek} \\setlength{\\oddsidemargin}{-69pt} \\begin{document}$$P<0.001$$\\end{document}) also significantly extended the time to postoperative ambulation. However, after multivariate logistic regression analysis, only PT (OR 1.86, 95% CI 1.32-\\documentclass[12pt]{minimal} \\usepackage{amsmath} \\usepackage{wasysym} \\usepackage{amsfonts} \\usepackage{amssymb} \\usepackage{amsbsy} \\usepackage{mathrsfs} \\usepackage{upgreek} \\setlength{\\oddsidemargin}{-69pt} \\begin{document}$$-$$\\end{document}2.61, P<0.001\\documentclass[12pt]{minimal} \\usepackage{amsmath} \\usepackage{wasysym} \\usepackage{amsfonts} \\usepackage{amssymb} \\usepackage{amsbsy} \\usepackage{mathrsfs} \\usepackage{upgreek} \\setlength{\\oddsidemargin}{-69pt} \\begin{document}$$P<0.001$$\\end{document}), TT (OR 1.30, 95% CI 1.09-\\documentclass[12pt]{minimal} \\usepackage{amsmath} \\usepackage{wasysym} \\usepackage{amsfonts} \\usepackage{amssymb} \\usepackage{amsbsy} \\usepackage{mathrsfs} \\usepackage{upgreek} \\setlength{\\oddsidemargin}{-69pt} \\begin{document}$$-$$\\end{document}1.55, P=0.004\\documentclass[12pt]{minimal} \\usepackage{amsmath} \\usepackage{wasysym} \\usepackage{amsfonts} \\usepackage{amssymb} \\usepackage{amsbsy} \\usepackage{mathrsfs} \\usepackage{upgreek} \\setlength{\\oddsidemargin}{-69pt} \\begin{document}$$P=0.004$$\\end{document}) intraoperative blood loss (OR 1.01, 95% CI 1.00-\\documentclass[12pt]{minimal} \\usepackage{amsmath} \\usepackage{wasysym} \\usepackage{amsfonts} \\usepackage{amssymb} \\usepackage{amsbsy} \\usepackage{mathrsfs} \\usepackage{upgreek} \\setlength{\\oddsidemargin}{-69pt} \\begin{document}$$-$$\\end{document}1.01, P=0.008\\documentclass[12pt]{minimal} \\usepackage{amsmath} \\usepackage{wasysym} \\usepackage{amsfonts} \\usepackage{amssymb} \\usepackage{amsbsy} \\usepackage{mathrsfs} \\usepackage{upgreek} \\setlength{\\oddsidemargin}{-69pt} \\begin{document}$$P=0.008$$\\end{document}) and the placement of intraoperative drains (OR 11.39, 95% CI 6.59-\\documentclass[12pt]{minimal} \\usepackage{amsmath} \\usepackage{wasysym} \\usepackage{amsfonts} \\usepackage{amssymb} \\usepackage{amsbsy} \\usepackage{mathrsfs} \\usepackage{upgreek} \\setlength{\\oddsidemargin}{-69pt} \\begin{document}$$-$$\\end{document}19.69, P<0.001\\documentclass[12pt]{minimal} \\usepackage{amsmath} \\usepackage{wasysym} \\usepackage{amsfonts} \\usepackage{amssymb} \\usepackage{amsbsy} \\usepackage{mathrsfs} \\usepackage{upgreek} \\setlength{\\oddsidemargin}{-69pt} \\begin{document}$$P<0.001$$\\end{document}) were identified as predictive factors for late ambulation in patients after TKA.ConclusionIn this study, preoperative coagulation function, intraoperative blood loss and the placement of intraoperative drains were factors contributing to delay ambulation time. Therefore, it is believed that properly improving preoperative coagulation function, effective intraoperative hemostasis, and reducing the placement of drains have a positive impact on early postoperative ambulation in patients undergoing TKA.

Opioid free analgesia after return home in ambulatory colonic surgery patients: a single-center observational study

BackgroundBecause of the adverse effects of morphine and its derivatives, non-opioid analgesia procedures are proposed after outpatient surgery. Without opioids, the ability to provide quality analgesia after the patient returns home may be questioned. We examined whether an opioid-free strategy could ensure satisfactory analgesia after ambulatory laparoscopic colectomy.MethodsWe performed a retrospective observational single-center study (of prospective collected database) including all patients eligible for scheduled outpatient colectomy. Postoperative analgesia was provided by paracetamol and nefopam. Postoperative follow-up included pain at mobilization (assessed by a numerical rating scale, NRS), hemodynamic variables, temperature, resumption of transit and biological markers of postoperative inflammation. The primary outcome was the proportion of patients with moderate to severe pain (NRS > 4) the day after surgery.ResultsData from 144 patients were analyzed. The majority were men aged 59 ± 12 years with a mean BMI of 27 [25-30] kg/m2. ASA scores were 1 for 14%, 2 for 59% and 3 for 27% of patients. Forty-seven patients (33%) underwent surgery for cancer, 94 for sigmoiditis (65%) and 3 (2%) for another colonic pathology. Postoperative pain was affected by time since surgery (Q3 = 52.4,p < 0.001) and decreased significantly from day to day. The incidence of moderate to severe pain at mobilization (NRS > 4) on the first day after surgery was (0.19; 95% CI, 0.13–0.27).ConclusionNon-opioid analgesia after ambulatory laparoscopic colectomy seems efficient to ensure adequate analgesia. This therapeutic strategy makes it possible to avoid the adverse effects of opioids.Trial registrationThe study was retrospectively registered and approved by the relevant institutional review board (CERAR) reference IRB 00010254–2018 – 188). All patients gave written informed consent for analysis of their data. The anonymous database was declared to the French Data Protection Authority (CNIL) (reference 221 2976 v0 of April 12, 2019).

Efficacy and safety of hypertonic saline therapy in ambulatory patients with heart failure: The SALT-HF trial.

Combination of hypertonic saline solution (HSS) with intravenous loop diuretics has been suggested to improve diuretic response in patients hospitalized for heart failure (HF). The efficacy and safety of this approach in the ambulatory setting remain unexplored. In this multicentre, double-blind, randomized study, we allocated ambulatory patients with worsening heart failure (WHF) to a 1-h infusion of intravenous furosemide (ivFurosemide)-HSS versus ivFurosemide. The primary endpoint was the volume of diuresis at 3 h. Secondary endpoints included 3-h natriuresis and weight variation, 7-day congestion data, kidney function and electrolytes, and 30-day clinical events. Overall, 167 participants (median age: 81 years, 30.5% female) were randomized across 13 sites between December 2020 and March 2023. There were no differences in 3-h diuresis between treatments (ivFurosemide-HSS: 1099 ml vs. ivFurosemide: 1103 ml, p = 0.963), 3-h natriuresis (∆ +2.642 mEq/L, p = 0.559), or 3-h weight (∆ +0.012 kg, p = 0.920). Patients in the ivFurosemide-HSS arm experienced significant weight decrease at 7 days (Δ -0.586 kg, p = 0.048). There were no between-treatment differences in clinical congestion score, biomarkers, inferior vena cava diameter, or the presence of lung ultrasound B-lines. At 30 days, 26.5% of the patients in the ivFurosemide-HSS group versus 33.3% in the ivFurosemide group experienced WHF (hazard ratio 0.76, p = 0.330). The incidence of death from any cause or HF hospitalization was 6% of patients in the ivFurosemide-HSS group and 8.3% of patients in the ivFurosemide group (hazard ratio 0.69, p = 0.521). The incidence of worsening kidney function or metabolic derangements was not significantly different in the two arms. A single infusion of ivFurosemide-HSS did not improve 3-h diuresis or congestion parameters in patients with ambulatory WHF. This therapy showed an appropriate safety profile.

Cross-sectional survey study of the natural history of LAMA2-related dystrophy

BackgroundLAMA2-related dystrophies (LAMA2-RD) are a rare group of neuromuscular disorders with a broad spectrum of phenotype severity, ranging from mild to severe. We performed a cross-sectional study of LAMA2-RD through motor function and pulmonary tests to establish the disease's natural history. MethodsForty-four individuals with LAMA2-RD were included and evaluated once through functional outcome measures including Motor Function Measure 32 (MFM32), Revised Upper Limb Module (RULM), goniometry, and Forced Vital Capacity (FVC). Fixed Effect Regression Model (ERM) and Kaplan-Meier curve were used for calculating the rate of the disease progression ResultsPatients were between 2 and 25 years old (mean 11.4), the most frequent phenotype presentation was non-ambulant (N=36, 81.8%) while eight patients (18,2 %) were ambulant. The non-ambulant group presented a more severe progression of the disease. Non-ambulant patients had a 1.85 % decrease in FVC/year against 1.32 %/year among ambulant patients. In the non-ambulant group, there was a 4.2 % drop/year in the MFM32-D2 domain (p<0.00001), a 2.6 % drop/year in the D3 domain (p<0.0001), and a 2.7 % drop/year in the MFM32 global assessment (p<0.0001). However, the non-ambulant group's evaluation of upper limb function through the RULM scale did not show a statistically significant reduction. In the non-ambulant group, elbow and knee retractions worsened 3.22 degrees/year (p=0.00087) and 1.92 degrees/year, respectively. While in those patients who acquired gait, elbow and knee retractions worsened 2.45 degrees/year (p=0.0003) and 1.73 degrees/year (p=0.01), respectively. ConclusionThis study confirmed the progressive nature of LAMA2-RD, both in ambulant and non-ambulant patients. MFM32, FVC, and goniometry were identified as promising outcome measures for natural history studies and clinical trials in LAMA2-RD.

Home-video EEG monitoring in a pediatric setting

IntroductionPediatric video-EEG monitoring is a standard procedure in epilepsy clinics, typically conducted in in-hospital settings.However, hospitalizationis sometimesunnecessary and imposes a burden on children and their families. In response to the rise of telehealth, home video-EEG monitoring has emerged, utilizing portable EEG equipment and video-cameras. ObjectiveThe aim of this study was to assess the feasibility of home video-EEGin a pediatric population. MethodsWe conducteda prospective pilot study of twentyhome video-EEG tests in children. We evaluated the quality of EEG and video recordings using a 5-point scale.Demographic, clinical and quality data were comparedto a similar group undergoing in-hospital video-EEG monitoring. ResultsTwenty children aged 2.1–17.2 years (mean 9.57 ± 1.01), 12 females (60 %), underwent home video-EEG. A higher proportion of children with intellectual disability/autism were observed in the home-EEG group compared to the in-hospital group: 12 patients (60 %) vs. 5 (25 %) (p < 0.05*, Fisher exact test). In the ambulatory group patients with developmental and epileptic encephalopathy were overrepresented (7 i.e., 35 % vs. 0), while those withself-limited childhood epilepsy were more prevalent in the in-hospital group (5 i.e., 25 % vs 0) (p < 0.05*, Chi square). In the ambulatory group the reasons for referral were seizure localization/classification in 11 patients (55 %), paroxysmal event classification in 5 (25 %) and quantification of sleep epileptic activity in 4(20 %),similar to the in-hospital group (40 %, 40 % and 20 % respectively, p > 0.05, Chi square). The quality of the EEG recording was higher compared to in-hospital tests: median 5 [IQR 3.25–5] vs 4[IQR 3–4] (p < 0.05*, Mann-Whitney U test), while the quality of video recording was lower compared to in-hospital recordings: median 3[IQR 2.25–4] vs 5[IQR4-5] (p < 0.01**, Mann-Whitney U test). ConclusionsHome video-EEG monitoring is apromising option forlong-termpediatric EEG monitoring, particularlyfor children with special needs.

Fecal incontinence is not associated with UTI: A contemporary case-control study.

Urinary tract infections (UTIs) are a leading cause of infection in adults. The most common cause is gastrointestinal bacteria ascending the urethra into the bladder. Studies showing fecal incontinence (FI) is a risk factor for UTI have been limited to nursing home populations. Healthy patients with recurrent UTI, especially women, often receive counseling, suggesting improper personal hygiene contributes to UTIs. This advice can be stigmatizing. Given UTI prevalence, it is important to elucidate risk factors for improved diagnosis, treatment, and patient education. Our objective was to perform a hospital-centered, retrospective case-control analysis to assess the effect of FI on UTI development in ambulatory patients. Patients (n = 3035) with a diagnosis of FI were identified from a single institution and propensity score-matched with screening colonoscopy patients (n = 3035) from 2018 to 2021. Patients were matched on age, sex, race, ethnicity, body mass index, and comorbidities, for example, diabetes, vesicoureteral reflux, and urinary incontinence. The association between FI and UTI was tested using Pearson's χ2 test. Median age was 64 years with more females than males (73.81% vs. 71.20% female for case/control, p = 0.02). Patients with FI were more often to have concurrent urinary incontinence (18.62% vs. 10.25% for case/control, p < 0.001), as well as specifically urgency incontinence (13.28% vs. 11.57% for case/control, p = 0.04). There was no significant difference in the incidence of UTI between patients with FI and those presenting for screening colonoscopy (p = 0.44). FI was not associated with an increased number of UTIs. Based on our results, current stigmatizing beliefs regarding the association between FI and UTI should be reevaluated.

Liver transplantation provides survival benefit at all levels of frailty: From the Multicenter Functional Assessment in Liver Transplantation Study.

Offering LT to frail patients may reduce waitlist mortality but may increase post-LT mortality. LT survival benefit is the concept of balancing these risks. We sought to quantify net survival benefit with LT by liver frailty index (LFI). We analyzed data in the multi-center Functional Assessment in LT (FrAILT) Study from 2012-2021. Pre-LT cohort included ambulatory patients with cirrhosis awaiting LT, without hepatocellular carcinoma; post-LT cohort included those who underwent LT. Primary outcomes were pre-LT and post-LT mortality. We computed 1-, 3-, and 5-year restricted mean survival times (RMST) from adjusted Cox models. Survival benefit was calculated as net gain in life-years with LT. Pre-LT cohort included 2628 patients: median MELDNa was 18 (IQR 14-22); 731 (28%) were frail; 440 (17%) died pre-LT. Post-LT cohort included 1335 patients: median MELDNa was 20 (IQR 14-24); 325 (24%) were frail; 103 (8%) died post-LT. Pre-LT RMST decreased substantially as LFI increased. Post-LT RMST also decreased as LFI increased but only modestly. There was no LFI threshold at which pre-LT and post-LT RMST intersected-patients had net survival benefit at all LFI values. Pre-LT and, to a lesser degree, post-LT mortality increased as LFI increased. Transplant offered a survival benefit at all LFI values, driven by a reduction in pre-LT mortality. No threshold of LFI was identified at which the risk of post-LT mortality exceeded pre-LT mortality. LT offers net survival benefit even in the presence of advanced frailty among those selected for LT.

Anesthetic recovery outcomes after 2 methods of nasal mucosal preparation for endoscopic sinus surgery

PurposeOur institution uses two approaches for nasal mucosal preparation during endoscopic sinus surgery (ESS) to improve surgical field visualization: topical epinephrine (TE) versus topical cocaine with injection of lidocaine containing epinephrine (TCLE). We aimed to compare anesthetic outcomes after ESS using these techniques. Methods and materialsWe retrospectively identified adult patients at our institution who underwent ESS from May 2018 through January 2023 under general anesthesia with propofol and remifentanil infusions. Postoperative anesthetic outcomes, including pain and recovery time, were compared between patients who had mucosal preparation with TE versus TCLE using inverse probability of treatment weighting (IPTW) to adjust for potential confounders. ResultsAmong 1449 patients who underwent ESS, 585 had TE, and 864 had TCLE. Compared with TE, during anesthetic recovery, the TCLE group had fewer episodes of severe pain (numeric pain score ≥ 7) (IPTW-adjusted odds ratio, 0.65; 95 % CI, 0.49–0.85; P = .002), less opioid analgesic administration (IPTW-adjusted odds ratio, 0.55; 95 % CI, 0.44–0.69; P < .001), and shorter recovery room stay (IPTW-adjusted ratio of the geometric mean, 0.90; 95 % CI, 0.85–0.96; P = .002). Postoperative nausea and vomiting and postoperative sedation were similar between groups. ConclusionsPatients who received preparation of the nasal mucosa with TCLE, compared with TE, were less likely to report severe pain or receive an opioid analgesic in the postanesthesia recovery room and had faster anesthetic recovery. This observation from our large clinical practice indicates that use topical and local anesthetic during endoscopic sinus surgery may have benefit for ambulatory ESS patients.

COMPLIANCE WITH THE SUSPECTED CAUDA EQUINA SYNDROME (CES) PATHWAY IN BETSI CADWALADR UNIVERSITY HEALTH BOARD (BCUHB) WEST: A RETROSPECTIVE AUDIT AGAINST NATIONAL STANDARDS

ObjectiveGuidelines published by the British Association of Spine Surgeons (BASS) and Society of British Neurological Surgeons (SBNS) recommend urgent MRI imaging and intervention in individuals suspected of having CES. The need for an evidence based protocol is driven by a lack of 24/7 MRI services and centralisation of neurosurgery to tertiary centres, compounded by CES's significant medico-legal implications. We conducted an audit to evaluate the pathway for suspected CES in BCUHB West between 2018 and 2021.MethodsA retrospective audit of patients managed for suspected CES between 01/11/2018 and 01/05/2021 was performed, using the SBNS/BASS guidelines as the standard.ResultsA total of 252 patients received an emergency MRI for suspected CES between 2018 and 2021. 99% of patients were scanned in compliance with SBNS/BASS standards. Radiological evidence of CES was found in 18% of patients. 33% of emergency scans were performed by out-of-hours services. 4% of patients had repeated scans within the same 6-month period. The majority of referrals originated from Orthopaedics surgeons (78%), or staff in the Emergency Department (8%). 92% of ambulatory patients were not admitted to hospital. During the peak of the COVID-19 pandemic, referrals increased from 2.5 to 3.5 per week.ConclusionSBNS/BASS standards were largely met, avoiding life changing disability and medico-legal consequences. The department should continue to follow SBNS/BASS guidance on the management of individuals with suspected CES. Challenges regarding the use of repeated scans should be addressed to avoid unnecessary costs. Introduction of new early recognition guidelines and Same Day Emergency Care (SDEC) has likely driven an increase in suspected CES referrals, and subsequent MRI demand. This audit should be utilised as an ongoing tool to ensure best practice continues, and to implement simple measures which may improve compliance with the pathway.

Influence of different electrode placement patterns on electromagnetic interference generated by Left Ventricular Assist Devices (LVADs) during electrocardiogram performance

Abstract Background Heart failure (HF), with an incidence of approximately 3/1000 person-years, often progresses to advanced stages where symptoms persist despite treatment. In such cases, mechanical circulatory assist devices, particularly left ventricular assist devices (LVADs) in ambulatory patients, enhance survival and quality of life. These LVADs generate electromagnetic interference, resulting in noise artefacts in conventional electrocardiograms (ECGs). The conventional ECG should be configured with a low-pass filter of 150 Hz and a high-pass filter of 0.05 Hz, as recommended by the American Heart Association/American College of Cardiology/Heart Rhythm Society. LVADs, such as the HeartMate 3 (Abbott®), typically operate at a rotational speed of 5000 to 6000 rpm, corresponding to oscillatory frequencies of 83.3 to 100 Hz. Objective To assess the impact of various surface electrode placement strategies and modifications to the low-pass filter on the electromagnetic interference produced by LVADs. Methods We conducted an observational study involving seven patients with HeartMate 3 adjusted to various rotational speeds (refer to Table 1). Three distinct electrode placement schemes (AHA, Mason Likar, and modified Torso) were employed for the frontal plane leads. The horizontal plane leads remained unmodified. Each patient underwent a total of 6 ECGs, corresponding to each of the aforementioned schemes. ECG recordings were performed using a high-pass filter set at 0.05 Hz and a low-pass filter set at 150 Hz. Additionally, a set of ECGs was obtained by modifying the low-pass filter to 40 Hz for further analysis. Results Figure 1 displays ECGs obtained from a male with a HeartMate 3 operating at 4600 rpm or 76.6 Hz. This device employs an algorithm to generate an "artificial pulse" by cyclically accelerating and decelerating the pump speed by 2000 rpm every 2 seconds, resulting in rotational speeds of 2600 rpm and 6600 rpm (43.3 Hz and 110 Hz, respectively). No specific placement scheme demonstrated consistent noise reduction across all cases, likely due to inter-individual variability resulting from factors such as the final position of the LVAD the rpm in each case, and the thoracic impedance of each patient. However, the modification of the low-pass filter to 40 Hz yielded a noteworthy improvement in noise reduction across all cases. In our limited sample, this adjustment did not pose issues for ECG interpretation. Nevertheless, it is essential to acknowledge that excessively low filter settings may not only eliminate noise but also potentially discard clinically relevant high-frequency signals (e.g., pacemaker spikes, large amplitude Rs, QRS notches). Conclusions Individualizing the electrode placement scheme is crucial for minimizing noise generated by LVADs. The adjustment of the low-pass filter to 40 Hz could generally prove beneficial in reducing artifact generation, despite the clinical limitations it may entail.

Heart failure with improved ejection fraction: patient characteristics, clinical outcomes and predictors for improvement.

Heart failure with improved ejection fraction (HFimpEF) is a recently recognized entity presenting a diagnostic and therapeutic challenge. Our aim was to characterize the profile of HFimpEF patients and evaluate predictors for EF lack of improvement among heart failure with reduced ejection fraction (HFrEF) patients. We included ambulatory HFrEF patients (EF≤40%) between January 1, 2015, and September 1, 2022, with two consecutive echocardiography exams at least 6 months apart. HFimpEF was defined as improved EF from ≤40%->40% and by ≥10%. A total of 567 HFrEF patients (72% male, 54.3 ± 14.4 years old) were analyzed. Patients without EF improvement were more likely to be male, had more comorbidities, ischemic cardiomyopathy (ICMP), markers of adverse cardiac remodeling (lower EF and higher left and right ventricular diameters) and presence of late gadolinium enhancement (LGE) in MRI (P < 0.05 for all). In a multivariate analysis, male sex, ICMP, lower EF, larger ventricular size and LGE remained independent predictors for lack of EF improvement. A prediction model for lack of EF improvement including LVEF, LV diameter, diastolic blood pressure and ischemic etiology exhibited an area under the ROC curve of 0.77 (95% CI 0.73-0.81; P < 0.001). HFimpEF patients had better prognosis with lower hospitalizations and mortality rates. Guideline directed medical therapy (GDMT) were associated with improved outcomes in both groups regardless of EF improvement. Lack of improvement in EF among HFrEF patients may be predicted by HF etiology and imaging parameters of adverse cardiac remodeling, and is associated with worse prognosis. GDMT were associated with improved outcomes in both HFimpEF and HFrEF patients.

Discrepancy between estimated glomerular filtration rate by creatinine versus cystatin C in different patient care settings

ObjectiveEstimated glomerular filtration rate (eGFR) calculated by cystatin C (cysC) has been recommended for broader adoption. This study assessed the discrepancy between eGFR calculated by cysC (eGFRcys) and creatinine (eGFRcr) in different patient care settings and explored potential contributing factors to such discrepancies. MethodsThis retrospective study included 2072 patients with paired cysC and creatinine results in different patient care settings. Delta eGFRcr-cys (eGFRcr − eGFRcys) was analyzed in relationship to patient care settings and the Elixhauser Comorbidity index. The 90-day survival in patients with different delta eGFR was assessed by Kaplan-Meier analysis, univariate and multivariate Cox proportional hazard models. In addition, discrepancy between eGFRcys and eGFRcr was analyzed in 50 ambulatory patients with systemic inflammation but normal kidney function. ResultsInpatients had higher cysC (median 1.91 mg/L), lower eGFRcys (median 31 mL/min/1.73 m2), and larger delta eGFRcr-cys (median 18 mL/min/1.73 m2) than outpatients (cysC median 1.53 mg/L, p < 0.0001, eGFRcys median 41 mL/min/1.73 m2, p < 0.0001, delta eGFRcr-cys median 4 mL/min/1.73 m2, p < 0.0001). Higher Elixhauser Comorbidity index correlated with lower eGFRcys and larger delta eGFRcr-cys, with median delta eGFRcr-cys 11 and 6 mL/min/1.73 m2 in patients with a Comorbidity index > 15 and ≤ 15, respectively (p < 0.0001). Increased delta eGFRcr-cys was associated with worse 90-day survival. Patients with systemic inflammation but normal kidney function had lower eGFRcys (median 77.5 mL/min/1.73 m2) than eGFRcr (median 97 mL/min/1.73 m2, p < 0.001), with red blood cell abnormalities as associated factors. ConclusionInflammation and comorbidities are associated with decreased eGFRcys and large discrepancies between eGFRcr and eGFRcys independent of kidney function and are most apparent in inpatients. Creatinine-cysC combined eGFR reduces this discrepancy and should be broadly adopted.

American registry of ambulatory and acute decompensated heart failure (AMERICCAASS registry): Rationale and design.

Heart failure (HF) is a highly prevalent and progressive condition associated with significant morbidity and mortality rates. Acute decompensated HF precipitates millions of hospitalizations each year. Despite therapeutic advances, the overall prognosis of HF is poor. The varying clinical courses and outcomes of patients with this disease may be due to region-specific gaps and since most HF studies are conducted in developed countries, the participation of Latin American and Caribbean countries is low. Considering this, the American Registry of Ambulatory and Acute Decompensated Heart Failure (AMERICCAASS) aims to characterize the population with ambulatory and acute decompensated HF in the American continent and to determine rehospitalization and survival outcomes during the 12months of follow-up. AMERICCAASS Registry is an observational, prospective, and hospital-based registry recruiting patients with ambulatory or acute decompensated HF. The registry plans to include between two and four institutions per country from at least 20 countries in the Americas, and at least 60 patients recruited from each participant institution regardless of their ambulatory or acutely decompensated condition. Ambulatory patients with confirmed HF diagnosis or inpatients presenting with acute decompensated HF will be included. Follow-up will be performed at 12months in ambulatory patients or 1, 6, and 12months after hospital discharge in acutely decompensated HF patients. This ongoing study began on 1 April 2022, with recruitment scheduled to end on 30 November 2023, and follow-up on 31 January 2025. Ethics approval was obtained from the Biomedical Research Ethics Committee of Fundación Valle del Lili. Data collected in the AMERICCAASS registry is being stored on the electronic platform REDCap (Research Electronic Data Capture), which allows different forms for patient groups to enable unbiased analyses. For quantitative variables comparison, we will use the Student's t-test or non-parametric tests accordingly. Categorical variables will be presented as proportions, and groups will be compared with Fisher's exact test. The significance level will be <0.05 for comparisons. Readmissions and post-discharge mortality will be calculated as proportions at 1, 6, and 12months, with a survival analysis by conditional probability and the Kaplan-Meier method. AMERICCAASS Registry is intended to be the most important registry of the continent for obtaining important information about demographics, aetiology, co-morbidities, and treatment received, either ambulatory or hospitalized. This registry may contribute to the optimization of national and regional evidence and public policies for the diagnosis and treatment of HF disease.