Rejected

In vitro study.

Focal dermal hypoplasia (FDH), or Goltz syndrome, is a rare genodermatosis affecting tissues of mesodermal and ectodermal origin. The characteristic skin changes have been reported to symptomatically flare in response to certain triggers as well as to progress over time in some cases. We present the case of a 5-year-old girl with cutaneous flaring and progression of FDH in the setting of septic shock. This case adds to the growing body of literature on both flaring and progression of the cutaneous manifestations of FDH.

We aimed to assess the feasibility and efficacy of laparoscopic extravascular stent in treatment of nutcracker syndrome by transperitoneal or retroperitoneal approach.

Intrathecal morphine (IM) is a popular adjunct for pain management in spinal deformity surgery for idiopathic scoliosis. It has not been studied in patients with early onset scoliosis (EOS). We retrospectively reviewed EOS patients undergoing growth-friendly surgery who received IM or did not receive IM (non-IM). Data from initial insertion and final fusion procedures were studied. IM was not used for lengthening procedures, short procedures (<3 h), patients with significant underlying respiratory issues, paraplegia, unsuccessful access and anesthesiologist discretion. We assessed pediatric ICU (PICU) admission and IM complications (respiratory depression, pruritus and nausea/vomiting), time to first postoperative opiate, and pain scores. There were 97 patients including 97 initial insertions (26 IM and 71 non-IM) and 74 patients with final fusions (17 IM and 57 non-IM). The first dose of opioids following insertion and final fusion occurred at 16.8 ± 3.8 and 16.8 ± 3.1 h postoperatively in the IM group compared to 5.5 ± 2.8 and 8.3 ± 3.2 h in the non-IM group, respectively (P < 0.001). Postoperative pain scores were lower in the IM groups (P = 0.001). Two patients with IM developed mild respiratory depression following initial insertion (P = 0.01) but did not require PICU admission. The rate of respiratory depression was not different between the final fusion groups. There was no difference between pruritus and nausea/vomiting at the final fusion. Preincision IM can provide well-tolerated and effective initial postoperative analgesia in select children with EOS undergoing spinal deformity surgery.

Amiodarone may be considered for patients with junctional ectopic tachycardia refractory to treatment with sedation, analgesia, cooling, and electrolyte replacements. There are currently no published pediatric data regarding the hemodynamic effects of the newer amiodarone formulation, PM101, devoid of hypotensive agents used in the original amiodarone formulation. We performed a single-center, retrospective, descriptive study from January 2012 to December 2020 in a pediatric ICU. Thirty-three patients were included (22 male and 11 female) between the ages of 1.1 and 1,460 days who developed post-operative junctional ectopic tachycardia or other tachyarrhythmias requiring PM101. Data analysis was performed on hemodynamic parameters (mean arterial pressures and heart rate) and total PM101 (mg/kg) from hour 0 of amiodarone administration to hour 72. Adverse outcomes were defined as Vasoactive-Inotropic Score >20, patients requiring ECMO or CPR, or patient death. There was no statistically significant decrease in mean arterial pressures within the 6 hours of PM101 administration (p > 0.05), but there was a statistically significant therapeutic decrease in heart rate for resolution of tachyarrhythmia (p < 0.05). Patients received up to 25 mg/kg in an 8-hour time for rate control. Average rate control was achieved within 11.91 hours and average rhythm control within 62 hours. There were four adverse events around the time of PM101 administration, with three determined to not be associated with the medication. PM101 is safe and effective in the pediatric cardiac surgical population. Our study demonstrated that PM101 can be used in a more aggressive dosing regimen than previously reported in pediatric literature with the prior formulation.

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease. SLE is treated with immunosuppressants with suboptimal efficacy and high risk of serious side effects. Patients with SLE have increased risk of mortality, organ damage and debilitating treatment-resistant fatigue. Autonomic nervous system dysfunction (AD) is present in approximately half of the patients and may promote autoimmunity by weakening the vagally mediated anti-inflammatory reflex. Recent studies suggest that transcutaneous vagus nerve stimulation (tVNS) has few side effects and beneficial effects on fatigue, pain, disease activity and organ function. This study investigates whether adjuvant tVNS improves measures of fatigue (primary end point), AD, clinical disease activity, inflammation, pain, organ function and quality of life.Hence, this study will contribute to the understanding of AD as a potentially important precursor of fatigue, disease activity, progression and complications in SLE, and how tVNS mechanistically may attenuate this. As adjuvant tVNS use may reduce the need for traditional immunosuppressive therapy, this trial may prompt a shift in the treatment of SLE and potentially other autoimmune disorders.

Low back joint compression forces have been linked to the development of chronic back pain. Back-support exoskeletons controllers based on low back compression force estimates could potentially reduce the incidence of chronic pain. However, progress has been hampered by the lack of robust and accurate methods for compression force estimation. Electromyography (EMG)-driven musculoskeletal models have been proposed to estimate lumbar compression forces. Nonetheless, they commonly underrepresented trunk musculoskeletal geometries or activation-contraction dynamics, preventing validation across large sets of conditions. Here, we develop and validate a subject-specific large-scale (238 muscle-tendon units) EMG-driven musculoskeletal model for the estimation of lumbosacral moments and compression forces, under eight box-lifting conditions. Ten participants performed symmetric and asymmetric box liftings under 5 and 15 kg weight conditions. EMG-driven model-based estimates of L5/S1 flexion-extension moments displayed high correlation, R (mean range: 0.88-0.94), and root mean squared errors between 0.21 and 0.38 Nm/kg, with respect to reference inverse dynamics moments. Model-derived muscle forces were utilized to compute lumbosacral compression forces, which reached eight times participants body weight in 15 kg liftings. For conditions involving stooped postures, model-based analyses revealed a predominant decrease in peak lumbar EMG amplitude during the lowering phase of liftings, which did not translate into a decrease in muscle-tendon forces. During eccentric contraction (box-lowering), our model employed the muscle force-velocity relationship to preserve muscle force despite significant EMG reduction. Our modeling methodology can inherently account for EMG-to-force non-linearities across subjects and lifting conditions, a crucial requirement for robust real-time control of back-support exoskeletons.

Knowledge of the pre-rehabilitation generic status of functioning in individuals with low back pain is necessary to understand the clinical utility of rehabilitation care. We conducted a scoping review to describe the pre-rehabilitation functioning status of persons with nonspecific low back pain using the World Health Organization Disability Assessment Schedule (WHODAS)-36 or WHODAS-12. We searched multiple databases from 2010 to 2021 for studies reporting pre-rehabilitation scores using WHODAS in persons with low back pain. Reviewers independently screened articles and extracted data, and we descriptively summarized results by the duration of low back pain (acute/subacute <3 months; chronic ≥3 months), and the WHODAS version. Of 1770 citations screened, eight citations were relevant. Five studies were conducted in Europe, two in America, and one in the African Region (mostly high-income countries). In persons with acute low back pain, the mean WHODAS-36 pre-rehabilitation summary score (complex scoring) was 22.8/100 (SD = 15.4) (one study). In persons with chronic low back pain, the mean WHODAS-36 summary score (complex scoring) ranged from 22.8/100 (SD = 5.7) to 41.5/100 (SD = 13.8) (two studies). For WHODAS-12 in persons with chronic low back pain, the mean summary score was 11.4/48 (SD = 8.7) or 14.4/48 (SD = 9.4) using simple scoring (two studies), and 25.8/100 (SD = 2.2) using complex scoring (one study). No floor or ceiling effects were observed in WHODAS-36 summary scores for chronic low back pain. Our scoping review comprehensively summarizes available studies reporting pre-rehabilitation levels of functioning using WHODAS in persons with low back pain. Persons with low back pain seeking rehabilitation have moderate limitations in functioning, and limitations level tends to be worse with chronic low back pain.

Ultrasound guided nerve blocks have been shown to be an effective analgesia option for patients with hip fracture. An education program was developed to train Emergency Department doctors and it received positive feedback from partici-pants who demonstrated competency after the training. We aimed to evaluate the education program at the behavioral level of the Kirkpatrick Model by determining the translation of training to practice of ultrasound guided nerve blocks in patients with a hip fracture at the Emergency Department.

Arrhythmogenic cardiomyopathy (ACM) is a genetically determined myocardial disease, characterized by myocytes necrosis with fibrofatty substitution and ventricular arrhythmias that can even lead to sudden cardiac death. The presence of inflammatory cell infiltrates in endomyocardial biopsies or in autoptic specimens of ACM patients has been reported, suggesting a possible role of inflammation in the pathophysiology of the disease. Furthermore, chest pain episodes accompanied by electrocardiographic changes and troponin release have been observed and defined as the "hot-phase" phenomenon. The aim of this critical systematic review was to assess the clinical features of ACM patients presenting with "hot-phase" episodes. According to PRISMA guidelines, a search was run in the PubMed, Scopus and Web of Science electronic databases using the following keywords: "arrhythmogenic cardiomyopathy"; "myocarditis" or "arrhythmogenic cardiomyopathy"; "troponin" or "arrhythmogenic cardiomyopathy"; and "hot-phase". A total of 1433 titles were retrieved, of which 65 studies were potentially relevant to the topic. Through the application of inclusion and exclusion criteria, 9 papers reporting 103 ACM patients who had experienced hot-phase episodes were selected for this review. Age at time of episodes was available in 76% of cases, with the mean age reported being 26 years ± 14 years (min 2-max 71 years). Overall, 86% of patients showed left ventricular epicardial LGE. At the time of hot-phase episodes, 49% received a diagnosis of ACM (Arrhythmogenic left ventricular cardiomyopathy in the majority of cases), 19% of dilated cardiomyopathy and 26% of acute myocarditis. At the genetic study, was the more represented disease-gene (69%), followed by (9%) and (6%). In conclusion, ACM patients showing hot-phase episodes are usually young, and is the most common disease gene, accounting for 69% of cases. Currently, the role of "hot-phase" episodes in disease progression and arrhythmic risk stratification remains to be clarified.