Critical Care Delivery: The Importance of Process of Care and ICU Structure to Improved Outcomes: An Update From the American College of Critical Care Medicine Task Force on Models of Critical Care.

In 2001, the Society of Critical Care Medicine published practice model guidelines that focused on the delivery of critical care and the roles of different ICU team members. An exhaustive review of the additional literature published since the last guideline has demonstrated that both the structure and process of care in the ICU are important for achieving optimal patient outcomes. Since the publication of the original guideline, several authorities have recognized that improvements in the processes of care, ICU structure, and the use of quality improvement science methodologies can beneficially impact patient outcomes and reduce costs.

Emerging therapies in traumatic brain injury.

Despite decades of basic and clinical research, treatments to improve outcomes after traumatic brain injury (TBI) are limited. However, based on the recent recognition of the prevalence of mild TBI, and its potential link to neurodegenerative disease, many new and exciting secondary injury mechanisms have been identified and several new therapies are being evaluated targeting both classic and novel paradigms. This includes a robust increase in both preclinical and clinical investigations.

De novo mutations in NALCN cause a syndrome characterized by congenital contractures of the limbs and face, hypotonia, and developmental delay.

Freeman-Sheldon syndrome, or distal arthrogryposis type 2A (DA2A), is an autosomal-dominant condition caused by mutations in MYH3 and characterized by multiple congenital contractures of the face and limbs and normal cognitive development. We identified a subset of five individuals who had been putatively diagnosed with "DA2A with severe neurological abnormalities" and for whom congenital contractures of the limbs and face, hypotonia, and global developmental delay had resulted in early death in three cases; this is a unique condition that we now refer to as CLIFAHDD syndrome. Exome sequencing identified missense mutations in the sodium leak channel, non-selective (NALCN) in four families affected by CLIFAHDD syndrome.

De novo heterozygous mutations in SMC3 cause a range of Cornelia de Lange syndrome-overlapping phenotypes.

Cornelia de Lange syndrome (CdLS) is characterized by facial dysmorphism, growth failure, intellectual disability, limb malformations, and multiple organ involvement. Mutations in five genes, encoding subunits of the cohesin complex (SMC1A, SMC3, RAD21) and its regulators (NIPBL, HDAC8), account for at least 70% of patients with CdLS or CdLS-like phenotypes. To date, only the clinical features from a single CdLS patient with SMC3 mutation has been published.

Disseminate and recurrent infundibulofolliculitis in childhood: a rarely reported entity.

Disseminate and recurrent infundibulofolliculitis (DRIF) is a term used to describe a micropapular skin disorder, most commonly occurring in young men with Fitzpatrick skin type V and VI. We present the case of two young Sudanese brothers with a clinical and histologic picture typical of this condition, which has been infrequently reported in children and has not previously been reported in related individuals. The etiology remains uncertain and there is no clear consensus on treatment.

Found in translation: Understanding the biology and behavior of experimental traumatic brain injury.

The aim of this review is to discuss in greater detail the topics covered in the recent symposium entitled "Traumatic brain injury: laboratory and clinical perspectives," presented at the 2014 International Behavioral Neuroscience Society annual meeting. Herein, we review contemporary laboratory models of traumatic brain injury (TBI) including common assays for sensorimotor and cognitive behavior. New modalities to evaluate social behavior after injury to the developing brain, as well as the attentional set-shifting test (AST) as a measure of executive function in TBI, will be highlighted.

Deciphering of mitochondrial cardiolipin oxidative signaling in cerebral ischemia-reperfusion.

It is believed that biosynthesis of lipid mediators in the central nervous system after cerebral ischemia-reperfusion starts with phospholipid hydrolysis by calcium-dependent phospholipases and is followed by oxygenation of released fatty acids (FAs). Here, we report an alternative pathway whereby cereberal ischemia-reperfusion triggered oxygenation of a mitochondria-specific phospholipid, cardiolipin (CL), is followed by its hydrolysis to yield monolyso-CLs and oxygenated derivatives of fatty (linoleic) acids. We used a model of global cerebral ischemia-reperfusion characterized by 9 minutes of asphyxia leading to asystole followed by cardiopulmonary resuscitation in postnatal day 17 rats.

Divergent long-term consequences of chronic treatment with haloperidol, risperidone, and bromocriptine on traumatic brain injury-induced cognitive deficits.

Antipsychotic drugs (APDs) are provided in the clinic to manage traumatic brain injury (TBI)-induced agitation and aggression. Experimental TBI studies consistently show that daily administration of the APDs, haloperidol (HAL) and risperidone (RISP), hinder recovery. However, it is unknown how long the adverse effects remain after cessation of treatment.

A combined therapeutic regimen of buspirone and environmental enrichment is more efficacious than either alone in enhancing spatial learning in brain-injured pediatric rats.

Buspirone, a 5-HT1A receptor agonist, and environmental enrichment (EE) enhance cognition and reduce histopathology after traumatic brain injury (TBI) in adult rats, but have not been fully evaluated after pediatric TBI, which is the leading cause of death in children. Hence, the aims of this study were to assess the efficacy of buspirone alone (Experiment 1) and in combination with EE (Experiment 2) in TBI postnatal day-17 male rats. The hypothesis was that both therapies would confer cognitive and histological benefits when provided singly, but their combination would be more efficacious.

Autism traits in children and adolescents with Cornelia de Lange syndrome.

Cornelia de Lange syndrome (CdLS) is a cohesinopathy causing delayed growth and limb deficits. Individuals with CdLS have mild to profound intellectual disability and autistic features. This study characterizes the behavioral phenotype of children with CdLS, focusing on autistic features, maladaptive behaviors, and impact of age.

Environmental enrichment as a viable neurorehabilitation strategy for experimental traumatic brain injury.

Environmental enrichment (EE) emerged as a robust independent variable capable of influencing behavioral outcome in experimental studies after the fortuitous observation by renowned neuropsychologist Donald O. Hebb that rats raised as pets in his home performed markedly better on problem-solving tasks than those kept in the laboratory. In the subsequent years, numerous studies ensued demonstrating that EE was also capable of inducing neuroplasticity in normal (i.

Cornelia de Lange syndrome: further delineation of phenotype, cohesin biology and educational focus, 5th Biennial Scientific and Educational Symposium abstracts.

Cornelia de Lange syndrome (CdLS) is the prototype for the cohesinopathy disorders that have mutations in genes associated with the cohesin subunit in all cells. Roberts syndrome is the next most common cohesinopathy. In addition to the developmental implications of cohesin biology, there is much translational and basic research, with progress towards potential treatment for these conditions.

Multiple deployments and combat trauma: do homefront stressors increase the risk for posttraumatic stress symptoms?

Multiple deployments are common among military personnel who served in Operation Enduring Freedom and Operation Iraqi Freedom and are associated with greater posttraumatic stress symptoms (PTSS). Homefront stressors (i.e.

Loss-of-function HDAC8 mutations cause a phenotypic spectrum of Cornelia de Lange syndrome-like features, ocular hypertelorism, large fontanelle and X-linked inheritance.

Cornelia de Lange syndrome (CdLS) is a multisystem genetic disorder with distinct facies, growth failure, intellectual disability, distal limb anomalies, gastrointestinal and neurological disease. Mutations in NIPBL, encoding a cohesin regulatory protein, account for >80% of cases with typical facies. Mutations in the core cohesin complex proteins, encoded by the SMC1A, SMC3 and RAD21 genes, together account for ∼5% of subjects, often with atypical CdLS features.

Old dog, new tricks: the attentional set-shifting test as a novel cognitive behavioral task after controlled cortical impact injury.

Cognitive impairment associated with prefrontal cortical dysfunction is a major component of disability in traumatic brain injury (TBI) survivors. Specifically, deficits of cognitive flexibility and attentional set-shifting are present across all levels of injury severity. Though alterations in spatial learning have been extensively described in experimental models of TBI, studies investigating more complex cognitive deficits are relatively scarce.

Increased risk of alcohol dependency in a cohort of National Guard troops with PTSD: a longitudinal study.

Studies show high rates of co-morbid post-traumatic stress disorder (PTSD) and alcohol use disorder (AUD) but there is no consensus on the causal direction of the relationship. Some theories suggest AUD develops as a coping mechanism to manage PTSD symptoms and others that AUD is a vulnerability factor for PTSD. A third hypothesis posits independent developmental pathways stemming from a shared etiology, such as the trauma exposure itself.

Newborn screening for X-linked adrenoleukodystrophy: further evidence high throughput screening is feasible.

X-linked adrenoleukodystrophy (ALD) is characterized by adrenal insufficiency and neurologic involvement with onset at variable ages. Plasma very long chain fatty acids are elevated in ALD; even in asymptomatic patients. We demonstrated previously that liquid chromatography tandem mass spectrometry measuring C26:0 lysophosphatidylcholine reliably identifies affected males.

Mapping subcortical brain maturation during adolescence: evidence of hemisphere- and sex-specific longitudinal changes.

Early to mid-adolescence is an important developmental period for subcortical brain maturation, but longitudinal studies of these neurodevelopmental changes are lacking. The present study acquired repeated magnetic resonance images from 60 adolescent subjects (28 female) at ages 12.5 and 16.

Gender differences in the risk and protective factors associated with PTSD: a prospective study of National Guard troops deployed to Iraq.

This study examines gender differences in post-traumatic stress symptoms (PTSS) and PTSS risk/protective factors among soldiers deployed to Iraq. We pay special attention to two potentially modifiable military factors, military preparedness and unit cohesion, which may buffer the deleterious psychological effects of combat. Longitudinal data were collected on 922 New Jersey National Guard soldiers (91 women) deployed to Iraq in 2008.

Minimally invasive technique versus an extensile lateral approach for intra-articular calcaneal fractures.

Background: The optimal method for treating intra-articular fractures of the calcaneus remains controversial. Extensile approaches allow excellent fracture exposure, but high rates of wound complications are seen. Newer minimally invasive techniques for calcaneus fracture fixation offer a potentially lower wound complication rate, but long-term clinical results are not available.

Metatarsal head resurfacing for advanced hallux rigidus.

Background: Advanced stages of first metatarsophalangeal (MTP) arthritis have traditionally been treated with various arthroplasties or arthrodesis. Studies suggest the outcomes of arthrodesis are superior to those of metallic joint replacement; however, complications and suboptimal outcomes in active patients still remain with arthrodesis of the first MTP joint. This study reports results of patients with advanced MTP arthritis who underwent metallic resurfacing of the metatarsal side of the MTP joint.

Environmental enrichment promotes robust functional and histological benefits in female rats after controlled cortical impact injury.

Environmental enrichment (EE) consistently induces marked benefits in male rats after traumatic brain injury (TBI), but whether similar efficacy extends to females is not well established. Hence, the aim of this study was to reassess the effect of EE on functional and histological outcome in female rats after brain trauma. Twenty-four normal cycling adult female rats underwent verification of estrous stage prior to controlled cortical impact (CCI) or sham injury and then were assigned to EE or standard (STD) housing.

Respirator and hearing protection use in the certified safe farm program.

An evaluation was conducted on the results of the Certified Safe Farm controlled intervention program on a wide range of occupational health and safety outcomes. This report focuses on the outcomes of personal protective equipment (PPE) usage among one cohort of 438 Iowa (owner-operator) farmers in the Certified Safe Farm study during a 5-year period from 2004 to 2008. Intervention farmers reported an 11% increase in regular respirator usage and a 23% increase in regular use of hearing protection relative to comparison groups.