A Patient With Schimmelpenning Syndrome and Mosaic KRAS Mutation.

Schimmelpenning syndrome is a neurocutaneous disorder characterized by craniofacial nevus sebaceus that fall along embryonic cutaneous lines and tend to be associated with neurological, ocular, skeletal, and vascular abnormalities. We report a child with extensive nevus sebaceus of the scalp, face, and thorax and other unusual physical findings who was found to have a mosaic mutation of KRAS c.35G>A p.12D pathognomonic of Schimmelpenning syndrome.

Factors Predicting Hospital Length of Stay Following Pharyngeal Flap for Velopharyngeal Insufficiency.

OBJECTIVE: Routine hospital admission following pharyngeal flap (PF) to correct velopharyngeal insufficiency (VPI) is the standard at most hospitals. Nevertheless, there is increasing resistance from third-party providers to approve stays longer than a "short stay" (23-hour) observation period. The purpose of the current study was to evaluate length of stay (LOS) and document potential influencing factors following PF. DESIGN: Retrospective chart review. Demographic and perioperative data were collected, and statistical analyses were performed to determine associations with hospital length of stay (LOS). Readiness for discharge was determined by oral intake, analgesic requirement, and respiratory status. SETTING: Tertiary care children's hospital Participants: All patients undergoing PF for VPI between 1990 and 2014. OUTCOME MEASURES: (1) LOS, (2) % satisfying all discharge criteria within a 23-hour observational time frame. RESULTS: Seventy-five patients were studied, with an average age of 6.8 years. Mean LOS was 65.4 hours. Only 11 patients (14.9%) met all discharge criteria by 23 hours. Multivariate predictors of shorter LOS were increasing patient age, male gender, lack of syndromic association, administration of an intraoperative antiemetic and steroids, and shorter anesthetic duration. Time to first oral intake correlated positively with LOS. Administration of intraoperative antiemetics increased the odds of meeting all discharge criteria within 23 hours by a factor of 12. CONCLUSIONS: Identification of factors associated with LOS after PF may allow providers to predict and potentially mitigate hospital stays. Nevertheless, most patients undergoing PF are not clinically ready for discharge within a short-stay (23-hour) observation period.

Conjoined Twin Separation: Integration of Three-Dimensional Modeling for Optimization of Surgical Planning.

Conjoined twinning is a rare anomaly, with an incidence of approximately 1 in 100,000 live births. There is a high perinatal mortality rate, but twins who survive pose reconstructive challenges that require meticulous preoperative planning. The authors describe the senior surgeon's career experience with conjoined twin separation, and the evolution of medical modeling and 3-dimensional imaging as a critical component in presurgical planning.The authors performed a retrospective review of all consecutive patients of conjoined twin separation at a single institution from January 2004 to December 2013. Data were collected related to patient demographics, comorbidities, operative technique, perioperative complications, survival, long-term outcomes, and the type of medical modeling system used for preoperative planning.Five sets of conjoined twins underwent separation during the 10-year study period. There were 3 sets of thoraco-omphalopagus twins, 1 set of pyopagus twins, and 1 set of ischiopagus tetrapus twins. The mean age at separation was 70 days, with a mean of 3.5 surgical procedures performed per patient during the first year of life. One set of twins experienced postseparation complications that warranted immediate return to the operating room. The overall survival rate after separation was 70%. The imaging methods used were computed tomography scan with 3-dimensional reconstruction, plaster molds, medical modeling with composite printing, and virtual surgical planning.The use of imaging and medical modeling in presurgical planning has proven to be an important element in optimizing the outcomes for patients with this rare anomaly.

State of the Science: Disgust and the Anxiety Disorders.

Anxiety disorders have long been conceptualized as disorders of fear, while other emotions have largely been overlooked. However, an emerging literature has increasingly implicated disgust in certain anxiety-related disorders, including obsessive-compulsive disorder, specific phobias (e.g., spider phobia), health anxiety, and posttraumatic stress disorder. Roughly two decades of research has accumulated evidence identifying various mechanisms linking disgust-related phenomena to these disorders. In the present "State of the Science" review, we sought to summarize the current state of the literature with respect to disgust-related mechanisms in anxiety disorders, including trait-level vulnerabilities (e.g., disgust proneness), cognitive processes (e.g., biases of attention and memory), and associated learning mechanisms (e.g., evaluative conditioning). Research in these areas has revealed important ways in which disgust differs from fear-related phenomena, which have important treatment implications. From there, we sought to summarize research on laboratory interventions that attempt to target and attenuate disgust, as well as the early research on formal cognitive-behavioral treatments that integrate disgust-related interventions for anxiety disorders. Although the past two decades of research have revealed important insights related to the role of disgust in psychopathology, much remains to be learned in this area. We propose some future directions, emphasizing the importance of a guiding framework that highlights studying disgust-related mechanisms across different levels of analysis.

Is disgust more resistant to extinction than fear? A meta-analytic review of laboratory paradigms.

Disgust can be acquired via evaluative conditioning; a process by which a neutral stimulus (conditioned stimulus; CS) comes to be evaluated as disgusting due to its pairing with an inherently disgusting stimulus (unconditioned stimulus; US). Research has shown that conditioned disgust responses are resistant to extinction which may have implications for disorders (i.e., contamination-based obsessive-compulsive disorder, specific phobias, and post-traumatic stress disorder) in which heightened disgust has been implicated. Importantly, extinction is the primary mechanism by which exposure therapies are thought to achieve symptom reduction for these disorders. Exposure therapies were originally modeled on fear extinction, whereas disgust extinction was largely overlooked until recently. Accordingly, differences in the degree to which learned disgust and fear can be attenuated via extinction learning remains unclear. The present investigation was a meta-analysis directly comparing the degree of extinction of conditioned disgust (n = 14) and conditioned fear (n = 14) in laboratory paradigms. Extinction was operationalized as the standardized mean difference (SMD) in evaluative ratings between the CS+ (the CS paired with the US) and CS- (the unpaired CS) after extinction training. Results of a subgroup analysis indicated that disgust (SMD = 0.52) was significantly more resistant to extinction than fear (SMD = 0.37). Additionally, a series of meta-regression analyses indicated that extinction was not influenced by important study characteristics (e.g., sex, age, number of conditioning and extinction trials). The findings suggest that extinction-based approaches may be less effective at attenuating learned disgust and research is needed to better optimize treatments for disgust-related disorders.

Personality or pathology? Predictors of early substance use in first-year college students.

OBJECTIVE: The aim of the current study was to examine the dual role of personality and psychopathology in predicting substance use among first-year students. PARTICIPANTS: 103 first-semester undergraduate students were recruited via the university subject pool. METHODS: Participants completed personality questionnaires, structured clinical interviews, followed by the completion of diary entries each week reporting on substance use throughout their first semester. RESULTS: Results indicated that a past diagnosis of an affective (mood/anxiety/stress) disorder was the most significant predictor of substance use. Personality and current psychopathology had no association to substance use. CONCLUSION: This finding is consistent with developmental models of substance use relating to emotion-related disease and suggests that greater nuance is needed in understanding substance use risk in college students.

Does past military experience impact fear reactivity in active-duty firefighters? Well, it is complicated.

OBJECTIVE: A significant proportion of military veterans successfully transition out of the military into civilian careers as first responders, such as firefighters. Like military service, being a firefighter is a high-risk profession involving exposure to aversive environments. Thus, it is possible that military experience might serve to buffer or exacerbate risk for negative psychological outcomes in firefighters. However, both occupations are associated with increased risk for psychopathology, such as PTSD, and little research has examined the effect of military service on processes that underlie stress in veterans serving as active-duty firefighters. The current study explores whether military service confers an adaptive advantage or an additional risk. METHOD: Using a case-control design, we examined differences in fear reactivity through electrodermal activity (EDA) and recording of fearful facial expressions, between 32 firefighters with and 32 firefighters without military veteran status (MVS; all men). Participants completed a semistructured, emotionally evocative interview with multiple contexts eliciting varying levels of emotion. RESULTS: MVS firefighters had relatively elevated EDA across contexts. However, lower baseline levels indicated calmer resting state in MVS firefighters. There was greater incidence of lifetime PTSD in MVS compared with non-MVS firefighters (40.6% vs. 15.6%). Overall, firefighters with past PTSD had less EDA reactivity. Finally, number of military deployments was associated with higher fear expressions throughout the interview. CONCLUSIONS: These findings highlight the need to consider interactions between military experience and psychiatric history in future investigations examining risk and resilience in first responders. (PsycInfo Database Record (c) 2022 APA, all rights reserved).

Were there losses in social support during the pandemic? Testing the impact of COVID-19 on psychological adjustment to trauma in United States adults.

Introduction: Social support is a key protective factor in the psychological adjustment of individuals to traumatic events. However, since March 2020, extant research has revealed evidence of increased loneliness, social isolation, and disconnection, likely due to COVID-19 pandemic-related recommendations that restricted day-to-day contact with others. Methods: In this investigation, we applied a case-control design to test the direct impacts of the pandemic on social support in United States adults recovering from a significant injury caused by PTSD-qualifying, traumatic events (e.g., motor vehicle crashes, violence, etc.). We compared individuals who experienced trauma during the pandemic, the "cases" recruited and evaluated between December 2020 to April 2022, to trauma-exposed "controls," recruited and evaluated pre-pandemic, from August 2018 through March 9, 2020 (prior to changes in public health recommendations in the region). Cohorts were matched on key demographics (age, sex, education, race/ethnicity, income) and injury severity variables. We tested to see if there were differences in reported social support over the first 5 months of adjustment, considering variable operationalizations of social support from social network size to social constraints in disclosure. Next, we tested to see if the protective role of social support in psychological adjustment to trauma was moderated by cohort status to determine if the impacts of the pandemic extended to changes in the process of adjustment. Results: The results of our analyses suggested that there were no significant cohort differences, meaning that whether prior to or during the pandemic, individuals reported similar levels of social support that were generally protective, and similar levels of psychological symptoms. However, there was some evidence of moderation by cohort status when examining the process of adjustment. Specifically, when examining symptoms of post-traumatic stress over time, individuals adjusting to traumatic events during COVID-19 received less benefit from social support. Discussion: Although negative mental health implications of the pandemic are increasingly evident, it has not been clear how the pandemic impacted normative psychological adjustment processes. These results are one of the first direct tests of the impact of COVID-19 on longitudinal adjustment to trauma and suggest some minimal impacts.

Recent advances in austere combat surgery: Use of aortic balloon occlusion as well as blood challenges by special operations medical forces in recent combat operations.

BACKGROUND: Resuscitative endovascular balloon occlusion of the aorta (REBOA) for control of noncompressible torso hemorrhage is a technology that is increasingly being utilized in the combat casualty setting. Its use in the resource restricted environment holds potential to improve hemorrhage control, decrease blood product utilization, decrease morbidity, and improve combat mortality. The objective of this report is to present the single largest series of REBOA use on severely injured combat casualties. METHODS: Over an 18-month period, austere surgical teams comprised of coalition partners provided initial damage control resuscitation (DCR) and surgical stabilization for over 2,300 combat casualties prior to transferring patients to the next level of trauma care. RESULTS: Twenty patients presented with injuries from explosion and gunshot wounds with mean initial heart rate of 129 bpm and mean initial systolic blood pressure of 71 mm Hg. Femoral cutdowns were used in six patients. Aortic occlusion was achieved with REBOA catheter placement in Zone 1 (n = 17) and Zone 3 (n = 2). Systolic blood pressure increased an average of 56 mm Hg with aortic occlusion. There were no access related site complications. All patients survived transport to the next level of care. The majority of blood products transfused in this cohort were whole blood, largely supported by emergent blood drives. CONCLUSION: This series demonstrates the potential for REBOA as a lifesaving technique for the patient who presents with hemodynamic instability and noncompressible torso hemorrhage. Resuscitative endovascular balloon occlusion of the aorta allows austere surgical teams to rapidly stabilize severely injured combat casualties, expand capability, and provide enhanced DCR while minimizing personnel, resources, and blood product utilization. The use of "whole blood only" strategy for DCR shows potential to be superior to traditional component therapy, and when combined with "proactive" REBOA utilization, provides significant improvements in hemodynamics and hemorrhage control. LEVEL OF EVIDENCE: Case series, level V.

A Modern Case Series of Resuscitative Endovascular Balloon Occlusion of the Aorta (REBOA) in an Out-of-Hospital, Combat Casualty Care Setting.

BACKGROUND: Resuscitative endovascular balloon occlusion of the aorta (REBOA) is used to mitigate bleeding and sustain central aortic pressure in the setting of shock. The ER-REBOA™ catheter is a new REBOA technology, previously reported only in the setting of civilian trauma and injury care. The use of REBOA in an out-of-hospital setting has not been reported, to our knowledge. METHODS: We present a case series of wartime injured patients cared for by a US Air Force Special Operations Surgical Team at an austere location fewer than 3km (5-10 minutes' transport) from point of injury and 2 hours from the next highest environment of care-a Role 2 equivalent. RESULTS: In a 2-month period, four patients presented with torso gunshot or fragmentation wounds, hemoperitoneum, and class IV shock. Hand-held ultrasound was used to diagnose hemoperitoneum and facilitate 7Fr femoral sheath access. ER-REBOA balloons were positioned and inflated in the aorta (zone 1 [n = 3] and zone 3 [n = 1]) without radiography. In all cases, REBOA resulted in immediate normalization of blood pressure and allowed induction of anesthesia, initiation of whole-blood transfusion, damage control laparotomy, and attainment of surgical hemostasis (range of inflation time, 18-65 minutes). There were no access- or REBOArelated complications and all patients survived to achieve transport to the next echelon of care in stable condition. CONCLUSION: To our knowledge, this is the first series to demonstrate the feasibility and effectiveness of REBOA in modern combat casualty care and the first to describe use of the ER-REBOA catheter. Use of this device by nonsurgeons and surgeons not specially trained in vascular surgery in the out-of-hospital setting is useful as a stabilizing and damage control adjunct, allowing time for resuscitation, laparotomy, and surgical hemostasis.