Surviving Sepsis After Burn Campaign.

INTRODUCTION: The Surviving Sepsis Campaign was developed to improve outcomes for all patients with sepsis. Despite sepsis being the primary cause of death after thermal injury, burns have always been excluded from the Surviving Sepsis efforts. To improve sepsis outcomes in burn patients, an international group of burn experts developed the Surviving Sepsis After Burn Campaign (SSABC) as a testable guideline to improve burn sepsis outcomes. METHODS: The International Society for Burn Injuries (ISBI) reached out to regional or national burn organizations to recommend members to participate in the program. Two members of the ISBI developed specific "patient/population, intervention, comparison and outcome" (PICO) questions that paralleled the 2021 Surviving Sepsis Campaign [1]. SSABC participants were asked to search the current literature and rate its quality for each topic. At the Congress of the ISBI, in Guadalajara, Mexico, August 28, 2022, a majority of the participants met to create "statements" based on the literature. The "summary statements" were then sent to all members for comment with the hope of developing an 80% consensus. After four reviews, a consensus statement for each topic was created or "no consensus" was reported. RESULTS: The committee developed sixty statements within fourteen topics that provide guidance for the early treatment of sepsis in burn patients. These statements should be used to improve the care of sepsis in burn patients. The statements should not be considered as "static" comments but should rather be used as guidelines for future testing of the best treatments for sepsis in burn patients. They should be updated on a regular basis. CONCLUSION: Members of the burn community from the around the world have developed the Surviving Sepsis After Burn Campaign guidelines with the goal of improving the outcome of sepsis in burn patients.

Coexistence of a Heterozygous Caveolin-3 Deletion and a Novel Dystrophin Gene Mutation in a Duchenne Muscular Dystrophy Patient.

Inherited muscular abnormalities are debilitating disorders that greatly diminish the quality of life in affected individuals. Mutations in proteins such as dystrophin and caveolin, which together with other proteins form structural connections between the cytoskeleton and the extracellular matrix, are frequently the culprit of muscular dystrophies. In this case report, we describe a patient with a novel pathogenic dystrophin mutation co-existing with a caveolin-3 deletion. While genetically composed of this unique combination, the patient phenotypically presented with a primary clinical manifestation of Duchenne muscular dystrophy (DMD) in contrast to other cases of dual mutations in dystrophin and dystrophin-associated proteins.

The Spectrum of Acute Cerebrovascular Disease in Patients with COVID-19.

(1) Background: COVID-19 infection is responsible for the ongoing pandemic and acute cerebrovascular disease (CVD) has been observed in COVID-19 patients. (2) Methods: We conducted a retrospective, observational study of hospitalized adult patients admitted to our hospital with SARS-CoV-2 and acute cerebrovascular disease. All clinical data were reviewed including epidemiology, clinical features, laboratory data, neuroradiological findings, hospital management and course from 32 patients hospitalized for COVID-19 management with acute cerebrovascular disease. (3) Results: Acute CVD with COVID-19 was associated with higher NIH stroke scale on discharge compared to non-COVID-19 CVDs. Seizures complicated the hospital course in 16% of COVID-19 patients with CVD. The majority of the acute CVDs were ischemic (81%) in nature followed by hemorrhagic (22%). Acute CVD with COVID-19 resulted in average hospital stays greater than twice that of the control group (13 days in COVID-19, 5 days in control). Acute CVD with COVID-19 patients had worse clinical outcomes with 31% patient deaths and 6% discharged to hospice. In the control group, 6% of patients died. (4) Conclusions: Acute CVD associated with COVID-19 tends to be more complicated with unique and adverse clinical phenotype, longer hospital admissions, and worse clinical outcomes.

Impact assessment following implementation of a vascular access team.

BACKGROUND: A vascular access team (VAT) was created in 2018 with the aim of improving vascular access and reducing complications associated with catheters. The impact of the introduction of a VAT in the insertion and maintenance of peripheral insertion central catheters (PICCs) was assessed. The cost-benefit associated with the use of a VAT was evaluated and the satisfaction of patients and professionals interacting with the VAT was measured. METHODS: In a prospective study, 275 PICCs inserted by the VAT were assessed for their impact on complications. PICCs were implanted with maximum barrier measures using an ultrasound with IC-ECG. Also, patient and professional satisfaction have been analysed thought a questionnaire over the phone or online, and hospital financial data was used to assess the cost impact of the insertion methodology followed by the VAT versus Anaesthesia Service. RESULTS: The thrombosis rate was 2.5% (7) and the bacteraemia rate was 1.1% (3). The use of the IC-ECG was correlated with a lower complication than the RX + IC-ECG (OR = 3.28, p = 0.021). In addition, there was a high level of perceived satisfaction for the patients surveyed and for the healthcare professionals involved in the care and management of these devices. The calculated saving for the implementation of the VAT was 61.81% compared with PICCs implanted in Anaesthesia Service. CONCLUSION: Low complication rates and high overall satisfaction scores in patients and professionals were observed, showing that a specialist VAT can have a positive impact in the insertion of PICCs and which also has a clear economic benefit.

SARS-CoV-2 surveillance strategy in essential workers of the Madrid City Council during the first epidemic wave in Spain, March-July 2020.

OBJECTIVES: To study prevalence of infection in essential workers of Madrid City Council by occupation, related characteristics, use of protective devices, risk perception, and main concerns about COVID-19 during lockdown. METHODS: A total of 30 231 workers were PCR tested for SARS-CoV-2 infection. Information was collected on COVID-19-related symptoms, risk factors, preventive equipment, and risk perception. The crude prevalence was calculated for infection, use of protective devices, perceived risk and main concerns. Additionally, adjusted prevalence and prevalence ratios (PR) were estimated for these variables using logistic regression models with age, gender, occupation, epidemiological week and laboratory as confounding factors. RESULTS: Overall prevalence of infection was 3.2% (95% CI 3.0% to 3.4%), being higher among policemen (4.4%) and bus drivers (4.2%), but lower among emergency healthcare personnel, firefighters, food market workers and burial services (<2%). Lower excess risk was observed in workers reporting occupational contact with COVID-19 cases only (PR=1.42; 95% CI 1.18 to 1.71) compared with household exposure only (PR=2.75; 95% CI 2.32 to 3.25). Infection was more frequent in symptomatic workers (PR=1.28; 95% CI 1.11 to 1.48), although 42% of detected infections were asymptomatic. Use of facial masks (78.7%) and disinfectants (86.3%) was common and associated with lower infection prevalence (PRmasks=0.68; 95% CI 0.58 to 0.79; PRdisinfectants=0.75; 95% CI 0.61 to 0.91). Over 50% of workers felt being at high risk of infection and worried about infecting others, yet only 2% considered quitting their work. CONCLUSIONS: This surveillance system allowed for detecting and isolating SARS-CoV-2 cases among essential workers, identifying characteristics related to infection and use of protective devices, and revealing specific needs for work-safety information and psychological support.

Results of Nurse Case Management in Primary Heath Care: Bibliographic Review.

BACKGROUND: The new characteristics of today's population, together with the presence of chronic diseases in the elderly, require a new approach to care, promoting coordination between different levels of care. In this sense, we find the figure of the nurse case manager (NCM) in primary health care mainly responsible for ensuring continuity of care in complex patients with chronic diseases. OBJECTIVE: to describe the role of the NCM in care management, determining its effectiveness in addressing chronic disease (health outcomes and quality of life) and its efficiency in the health system. METHODS: Bibliographic review of scientific evidence on case management applied to nursing. Between March and April 2020 a bibliographic search was carried out in the Dialnet, Scielo, Scopus and Pubmed databases. INCLUSION CRITERIA: articles written in the last 5 years, which analyze how this nursing rol influences the care and health of patients. RESULTS: A total of 16 articles were selected. The NCM reduced the use of the emergency department, hospital admissions, readmissions, and the duration of these in the patients studied. CONCLUSION: The NCM is effective and efficient for both patients and health institutions, and a common practice model is needed that includes standardized protocols and evidence-based practices.

Focal epilepsy caused by single cerebral cavernous malformation (CCM) is associated with regional and global resting state functional connectivity (FC) disruption.

Epilepsy, including the type with focal onset, is increasingly viewed as a disorder of the brain network. Here we employed the functional connectivity (FC) metrics estimated from the resting state functional MRI (rsfMRI) to investigate the changes of brain network associated with focal epilepsy caused by single cerebral cavernous malformation (CCM). Eight CCM subjects and 21 age and gender matched controls were enrolled in the study. Seven of 8 CCM subjects underwent surgical resection of the CCM and became seizure free and 4 of the surgical subjects underwent a repeat rsfMRI study. We showed that there was both regional and global disruption of the FC values among the CCM subjects including decreased in homotopic FC (HFC) and global FC (GFC) in the regions of interest (ROIs) where the CCMs were located. There was also the disruption of the default mode network (DMN) especially the FC between the middle prefrontal cortex (MPFC) and the right lateral parietal cortex (LPR) among these individuals. We observed the trend of alleviation of these disruptions after the individual has become seizure free from the surgical resection of the CCM. Using a voxel-based approach, we found the disruption of the HFC and GFC in the brain tissue immediately adjacent to the CCM and the severity of the disruption appeared inversely proportional to the distance of the brain tissue to the lesion. Our findings confirm the disruption of normal brain networks from focal epilepsy, a process that may be reversible with successful surgical treatments rendering patients seizure free. Some voxel-based metrics may help identify the epileptogenic zone and guide the surgical resection.

Revisiting racial disparities in access to surgical management of drug-resistant temporal lobe epilepsy post implementation of Affordable Care Act.

OBJECTIVES: Prior to enactment of the Affordable Care Act(ACA), several reports demonstrated remarkable racial disparities in access to surgical care for epileptic patients. Implementation of ACA provided healthcare access to 7-16 million uninsured Americans. The current study investigates racial disparity post ACA era in (1) access to surgical management of drug-resistant temporal lobe epilepsy (DRTLE); (2) short-term outcomes in the surgical cohort. PATIENT AND METHODS: Adult patients with DRTLE registered in the National Inpatient Sample (2012-2013) were identified. Association of race (African Americans and other minorities with respect to Caucasians) with access to surgical management of TLE, and short-term outcomes [discharge disposition, length of stay (LOS) and hospital charges] in the surgical cohort were investigated using multivariable regression techniques. RESULTS: Of the 4062 patients with DRTLE, 3.6%(n=148) underwent lobectomy. Overall, the mean age of the cohort was 42.35±16.33years, and 54% were female. Regression models adjusted for patient demographics, clinical and hospital characteristics demonstrated no racial disparities in access to surgical care for DRTLE. Likewise, no racial disparity was noted in outcomes in the surgical cohort. CONCLUSION: Our study reflects no racial disparity in access to surgical care in patients with DRTLE post 2010 amendment of the ACA. The seismic changes to the US healthcare system may plausibly have accounted for addressing the gap in racial disparity for epilepsy surgery.

American Clinical Neurophysiology Society Guideline 5: Minimum Technical Standards for Pediatric Electroencephalography.

This revision to the EEG Guidelines is an update incorporating the current electroencephalography technology and practice. It was previously published as Guideline 2. Similar to the prior guideline, it delineates the aspects of Guideline 1 that should be modified for neonates and young children. Recording conditions for photic stimulation and hyperventilation are revised to enhance the provocation of epileptiform discharges. Revisions recognize the difficulties involved in performing an EEG under sedation in young children. Recommended neonatal EEG montages are displayed for the reduced set of electrodes only since the montages in Guideline 3 should be used for a 21-electrode 10-20 system array. Neonatal documentation is updated to use current American Academy of Pediatrics term "postmenstrual age" rather than "conceptional age." Finally, because therapeutic hypothermia alters the prognostic value of neonatal EEG, the necessity of documenting the patient's temperature at the time of recording is emphasized.

Psammomatous Cavernous Malformation Presenting as Drug-Resistant Epilepsy: Case Illustration and Review of Literature.

BACKGROUND: Psammoma bodies (PBs) are whorled, laminated hyaline spherules containing calcium deposits. Intracranially, the presence of PBs is associated with variants of meningioma and pituitary lesions, as well as aging choroid plexus. Limited information exists on their presence in vascular malformation. RESULTS: In this report, we describe a case of an adolescent male with drug-resistant epilepsy that was surgically managed at our regional epilepsy center. The epileptogenic focus was determined to be emanating from an indolent right insular lesion. Histopathologic evaluation showed the abundance of intravascular and perivascular PBs. Immunohistochemical evaluation confirmed the vascular origin using vascular markers. The unusual presence of PBs in a vascular lesion was unanticipated. CONCLUSIONS: Based on our case, we present the clinicoradiologic characteristics, supplemented with intraoperative findings, for this unusual lesion. In addition, because of the unusual presence of PBs in vascular lesions, we provide the findings of a systematic literature review to show the association of PBs with intracranial vascular lesions.

American Clinical Neurophysiology Society Guideline 5: Minimum Technical Standards for Pediatric Electroencephalography.

This revision to the EEG Guidelines is an update incorporating the current electroencephalography technology and practice. It was previously published as Guideline 2. Similar to the prior guideline, it delineates the aspects of Guideline 1 that should be modified for neonates and young children. Recording conditions for photic stimulation and hyperventilation are revised to enhance the provocation of epileptiform discharges. Revisions recognize the difficulties involved in performing an EEG under sedation in young children. Recommended neonatal EEG montages are displayed for the reduced set of electrodes only since the montages in Guideline 3 should be used for a 21-electrode 10-20 system array. Neonatal documentation is updated to use current American Academy of Pediatrics term "postmenstrual age" rather than "conceptional age." Finally, because therapeutic hypothermia alters the prognostic value of neonatal EEG, the necessity of documenting the patient's temperature at the time of recording is emphasized.

ATMIN is a transcriptional regulator of both lung morphogenesis and ciliogenesis.

Initially identified in DNA damage repair, ATM-interactor (ATMIN) further functions as a transcriptional regulator of lung morphogenesis. Here we analyse three mouse mutants, Atmin(gpg6/gpg6), Atmin(H210Q/H210Q) and Dynll1(GT/GT), revealing how ATMIN and its transcriptional target dynein light chain LC8-type 1 (DYNLL1) are required for normal lung morphogenesis and ciliogenesis. Expression screening of ciliogenic genes confirmed Dynll1 to be controlled by ATMIN and further revealed moderately altered expression of known intraflagellar transport (IFT) protein-encoding loci in Atmin mutant embryos. Significantly, Dynll1(GT/GT) embryonic cilia exhibited shortening and bulging, highly similar to the characterised retrograde IFT phenotype of Dync2h1. Depletion of ATMIN or DYNLL1 in cultured cells recapitulated the in vivo ciliogenesis phenotypes and expression of DYNLL1 or the related DYNLL2 rescued the effects of loss of ATMIN, demonstrating that ATMIN primarily promotes ciliogenesis by regulating Dynll1 expression. Furthermore, DYNLL1 as well as DYNLL2 localised to cilia in puncta, consistent with IFT particles, and physically interacted with WDR34, a mammalian homologue of the Chlamydomonas cytoplasmic dynein 2 intermediate chain that also localised to the cilium. This study extends the established Atmin-Dynll1 relationship into a developmental and a ciliary context, uncovering a novel series of interactions between DYNLL1, WDR34 and ATMIN. This identifies potential novel components of cytoplasmic dynein 2 and furthermore provides fresh insights into the molecular pathogenesis of human skeletal ciliopathies.

Epstein-Barr virus induced hemophagocytic lymphohistiocytosis in X-linked lymphoproliferative disease.

X-linked lymphoproliferative disease (XLP) is a rare, often fatal genetic disorder characterized by extreme vulnerability to Epstein-Barr virus (EBV). EBV-induced hemophagocytic lymphohistiocytosis (HLH) is a known presentation in XLP. In EBV-induced HLH in XLP, the brain imaging findings in the acute phase include a non specific pattern. In this report, we highlight the magnetic resonance imaging and magnetic resonance spectroscopy findings in a child with EBV induced HLH in XLP.

Dominant ß-catenin mutations cause intellectual disability with recognizable syndromic features.

The recent identification of multiple dominant mutations in the gene encoding ß-catenin in both humans and mice has enabled exploration of the molecular and cellular basis of ß-catenin function in cognitive impairment. In humans, ß-catenin mutations that cause a spectrum of neurodevelopmental disorders have been identified. We identified de novo ß-catenin mutations in patients with intellectual disability, carefully characterized their phenotypes, and were able to define a recognizable intellectual disability syndrome. In parallel, characterization of a chemically mutagenized mouse line that displays features similar to those of human patients with ß-catenin mutations enabled us to investigate the consequences of ß-catenin dysfunction through development and into adulthood. The mouse mutant, designated batface (Bfc), carries a Thr653Lys substitution in the C-terminal armadillo repeat of ß-catenin and displayed a reduced affinity for membrane-associated cadherins. In association with this decreased cadherin interaction, we found that the mutation results in decreased intrahemispheric connections, with deficits in dendritic branching, long-term potentiation, and cognitive function. Our study provides in vivo evidence that dominant mutations in ß-catenin underlie losses in its adhesion-related functions, which leads to severe consequences, including intellectual disability, childhood hypotonia, progressive spasticity of lower limbs, and abnormal craniofacial features in adults.

[Effectiveness of a stretching program on anxiety levels of workers in a logistic platform: a randomized controlled study]. / Efectividad de un programa de estiramientos sobre los niveles de ansiedad de los trabajadores de una plataforma logística: un estudio controlado aleatorizado.

OBJECTIVE: To study the influence of a short programme of stretching exercises on anxiety levels of workers in a Spanish logistic company. METHOD: A controlled clinical trial was carried out by means of an inter-subject design of random homogeneous blocks. Participants were assigned to the experimental group (n=67), treated with a programme of stretching exercises of 10-minute duration after working hours for a period of 3 months, or to the untreated control group (n=67). The primary result variable was anxiety, and the secondary variables were burnout syndrome, quality of life and flexibility. An analysis of covariance (ANCOVA) by intention to treat was performed on each of the result variables by controlling the baseline scores, the age and the practice of introjective activities outside the program, with the size effect calculated by means of the partial eta-squared value (η(2)). RESULTS: The results of the ANCOVA showed a moderate effect of the stretching exercise programme on the levels of anxiety (η(2)=0,06; P=.004). Other effects found were substantial for flexibility (η(2)=0,13; P<.001); moderately high for bodily pain (η(2)=0,08; P=.001), and moderate for vitality (η(2)=0,05; P=.016); mental health (η(2)=0,05; P=.017); general health (η(2)=0,04; P=.028) and exhaustion (η(2)=0,04; P=.025). CONCLUSIONS: The implementation of a short programme of stretching exercises in the work place was effective for reducing levels of anxiety, bodily pain and exhaustion, and for raising levels of vitality, mental health, general health and flexibility. This type of intervention could be seen as a low-cost strategy for improving the well-being of workers.

Brain abscess from a peritonsillar abscess in an immunocompetent child: a case report and review of the literature.

A brain abscess is uncommon but potentially lethal. Common predisposing risk factors include congenital cyanotic heart disease, immunocompromised status, and the presence of septic foci. We describe a left frontal brain abscess accompanied by fever, headache, and weight loss for a 3-month period. The presumptive source of the brain abscess involved a left peritonsillar abscess. To the best of our knowledge, one similar case was reported in the literature in 1929. The specific signs of peritonsillar abscess in our patient included trismus, decreased phonation, and a muffled voice. The peritonsillar abscess was not clinically diagnosed, but incidentally detected on lower axial sections of cranial magnetic resonance imaging. Fever and trismus improved after surgical drainage of the peritonsillar abscess. The cerebral abscess was conservatively treated with intravenous antibiotics. The patient developed hydrocephalus as a sequela to the involvement of the basal meninges.

Lymphocytic hypophysitis in children: a novel presentation and literature review.

Lymphocytic hypophysitis, an autoimmune mediated chronic inflammation of the pituitary gland, is uncommon in children.(1) The usual presentation is with symptoms secondary to pressure from the enlargement of the gland itself and or impairment of pituitary hormones of varying degrees, in conjunction with a sellar mass.( 2,3) In 80% of cases, partial or panhypopituitarism is present with approximately 75% of cases having multiple hormonal deficiencies.(2) We report the case of a prepubertal female who presented with cyclical vomiting, as her initial symptom of lymphocytic hypophysitis which resolved promptly after pituitary biopsy. This case suggests that cyclical vomiting can be a symptom associated with lymphocytic hypophysitis. Therefore, it should be considered in the differential diagnosis of any child presenting with cyclical vomiting.

Intraventricular fat from retrograde flow through a lumboperitoneal shunt.

We present a case of intraventricular fat deposits discovered on magnetic resonance imaging and computed tomography in the absence of a ruptured dermoid cyst. The patient is a 25-year-old right-handed African-American female who previously had lumboperitoneal (LP) shunt placement for pseudotumor cerebri. She had multiple shunt-related complications that required two revisions as well as an incisional hernia repair. We suggest that the presence of fat in her ventricles is due to retrograde flow from abdominal fat during the time when the shunt tip was located in the anterior abdominal wall as opposed to her peritoneum. Unlike ventriculoperitoneal shunts, LP shunts do not contain valves, making retrograde passage of fat possible when abdominal pressures exceed lumbar subarachnoid pressures. The presence of these small amounts of fat in the ventricles is unlikely to cause any further symptoms in this patient, but monitoring for any blockage of cerebrospinal fluid flow or neurologic deterioration would be advisable.

Infantile spasms associated with 5q14.3 deletion.

West syndrome consists of the triad of infantile spasms, characteristic electroencephalogram (EEG) pattern of hypsarrythmia and developmental delay. Infantile spasms generally occur in infants during the first year of life. There are different genetic mutations and syndromes associated with infantile spasms. We report the case of a white 14-month-old boy who developed infantile spasms and was found to have a 5q14.3 deletion.