Healthcare Experiences of African American Women with the Fragile X Premutation.

This study aims to understand the healthcare experiences of African American women with a fragile X premutation (PM). PM carriers are at risk for fragile X-associated conditions, including primary ovarian insufficiency (FXPOI) and neuropsychiatric disorders (FXAND). There is no racial/ethnic association with carrying a PM, but African American women historically experience barriers receiving quality healthcare in the USA. Obstacles to care may increase mental health conditions like anxiety and depression. Eight African American women with a PM were interviewed to explore disparities in receiving healthcare and to learn about psychosocial experiences during and after their diagnoses. Interviews were transcribed verbatim and independently coded by two researchers. A deductive-inductive approach was used, followed by thematic analysis to determine prominent themes. The average participant age was 52.3 ± 8.60 years, with a mean age at premutation diagnosis of 31 ± 5.95 years. Seven participants had children with FXS. Themes from interviews included healthcare experiences, family dynamics, and emotional/mental health after their diagnosis. Participants reported concerns about not being taken seriously by providers and mistrust of the medical institutions. Within families, participants reported denial, insensitivity, and isolation. Participants reported a high incidence of anxiety and depression. Both are symptoms of FXAND and stresses of systemic racism and sexism. The reported family dynamics around the news of a genetic diagnosis stand apart from other racial cohorts in fragile X research: interventions like family counseling sessions and inclusive support opportunities from national organizations could ease the impacts of a PM for African American women.

Factors to Consider to Study Preductal Oxygen Saturation Targets in Neonatal Pulmonary Hypertension.

There are potential benefits and risks to the infant with higher and lower oxygen saturation (SpO2) targets, and the ideal range for infants with pulmonary hypertension (PH) remains unknown. Targeting high SpO2 can promote pulmonary vasodilation but cause oxygen toxicity. Targeting lower SpO2 may increase pulmonary vascular resistance, especially in the presence of acidosis and hypothermia. We will conduct a randomized pilot trial to compare two ranges of target preductal SpO2 in late-preterm and term infants with hypoxic respiratory failure (HRF) and acute pulmonary hypertension (aPH) of the newborn. We will assess the reliability of a newly created HRF/PH score that could be used in larger trials. We will assess trial feasibility and obtain preliminary estimates of outcomes. Our primary hypothesis is that in neonates with PH and HRF, targeting preductal SpO2 of 95-99% (intervention) will result in lower pulmonary vascular resistance and pulmonary arterial pressures, and lower the need for pulmonary vasodilators (inhaled nitric oxide-iNO, milrinone and sildenafil) compared to targeting SpO2 at 91-95% (standard). We also speculate that a higher SpO2 target can potentially induce oxidative stress and decrease response to iNO (oxygenation and pulmonary vasodilation) for those patients that still require iNO in this range. We present considerations in planning this trial as well as some of the details of the protocol design (Clinicaltrials.gov (NCT04938167)).

Neonatal Coccidioidomycosis: A Single-center Experience and Review of the Literature.

BACKGROUND: Coccidioidomycosis is common in adult and pediatric populations living in endemic areas of the United States but has rarely been reported in neonates. We reviewed recent cases of neonatal coccidioidomycosis treated at a tertiary care children's hospital in an endemic area and compared them with previously reported cases in the literature. METHODS: We performed a retrospective chart review of infants 1 month old or less hospitalized with a diagnosis of coccidioidomycosis from January 1, 2014, to December 31, 2019. Additionally, we performed a literature review of all reported cases of neonatal coccidioidomycosis over the past 7 decades through PubMed. Infants born to mothers with confirmed or suspected active coccidioidomycosis were excluded. RESULTS: Three cases of neonatal coccidioidomycosis were identified at our institution. Each presented in a unique manner and had an alternative diagnosis at the time of initial presentation. Two patients had negative coccidioidal screening tests upon admission but later seroconverted. All patients had extrapulmonary involvement, and all recovered after appropriate treatment. A review of the literature reveals that the presentations and outcomes of neonatal coccidioidomycosis vary widely. CONCLUSIONS: There is significant variability in the presentation of coccidioidomycosis in the neonatal period, and diagnosis may be challenging. In endemic regions, healthcare providers should consider coccidioidomycosis in their differential diagnoses of ill-appearing neonates that do not respond to treatment.

Clinical Nurse Leader: Evolution of the Role in Oncology Care.

The clinical nurse leader (CNL) role has evolved since the American Association of Colleges of Nursing published a white paper on the role in 2007. Since then, various publications have evaluated the role of CNLs in enhancing quality outcomes for patients. The introduction of the CNL role in the oncology setting, although occurring with variability across the United States, provides a unique opportunity to explore the benefits of this role in cancer care outcomes.

Needle-guided ultrasound technique for axillary artery catheter placement in critically ill patients: A case series and technique description.

PURPOSE: Axillary arterial cannulation for blood pressure monitoring has been reported in adults since 1973. Reported failure rates using palpation landmarks are high. This report describes a needle-guided ultrasound technique for axillary arterial line placement in critically ill patients. METHODS: A retrospective review of all patients requiring axillary arterial cannulation attempts with ultrasound-assisted needle guidance for hemodynamic monitoring was performed from July 2010 to June 2016 at a single institution. RESULTS: One hundred fifty nine (159) cannulation attempts were performed in 155 patients. The overall success rate was 97%, with a first pass success rate of 84%. Inexperienced operators performed 49% of procedures under direct faculty supervision, and had a 99% success rate, which was not different from experienced operators. Almost 20% of patients had moderate-to-severe coagulopathy (platelets<50k/uL, INR>2.0 or PTT>60s). Complications reported included the following: nonfunctioning of catheter (6%) and hematoma (6%). Ischemia was noted in 2 patients (1%), but only one was attributed to the arterial catheter. CONCLUSIONS: Use of the needle-guided ultrasound assisted approach for axillary arterial line placement is easily teachable and can be used to promote safe and successful placement of axillary arterial lines for novice learners.

A three-year reflective writing program as part of introductory pharmacy practice experiences.

OBJECTIVES: To implement and evaluate a 3-year reflective writing program incorporated into introductory pharmacy practice experiences (IPPEs) in the first- through third-year of a doctor of pharmacy (PharmD) program. DESIGN: Reflective writing was integrated into 6 IPPE courses to develop students' lifelong learning skills. In their writing, students were required to self-assess their performance in patient care activities, identify and describe how they would incorporate learning opportunities, and then evaluate their progress. Practitioners, faculty members, and fourth-year PharmD students served as writing preceptors. ASSESSMENT: The success of the writing program was assessed by reviewing class performance and surveying writing preceptor's opinions regarding the student's achievement of program objectives. Class pass rates averaged greater than 99% over the 8 years of the program and the large majority of the writing preceptors reported that student learning objectives were met. A support pool of 99 writing preceptors was created. CONCLUSIONS: A 3-year reflective writing program improved pharmacy students' reflection and reflective writing skills.

Small-dose dexamethasone improves quality of recovery scores after elective cardiac surgery: a randomized, double-blind, placebo-controlled study.

OBJECTIVES: The use of steroid therapy in cardiac surgical patients remains controversial. The aim of this clinical investigation was to determine the effect of small-dose dexamethasone therapy on patient-perceived quality of recovery (QoR) scores in elective cardiac surgical patients. In addition, the authors assessed the impact of dexamethasone on the incidence of common adverse events after cardiopulmonary bypass (CPB). DESIGN: A prospective, randomized study. SETTING: University hospitals. PARTICIPANTS: One hundred seventeen patients undergoing cardiac surgery with CPB and anticipated early tracheal extubation. INTERVENTIONS: Subjects were randomized to receive either dexamethasone (dexamethasone group, 8 mg at the induction of anesthesia and at the initiation of CPB) or placebo (control group, saline). MEASUREMENTS AND MAIN RESULTS: The QoR was assessed using the QoR-40 scoring system preoperatively and on postoperative days (PODs) 1 and 2. Secondary outcome measures assessed in the postoperative period included nausea, vomiting, fatigue, febrile responses, shivering, pulmonary gas exchange, and analgesic requirements. Global QoR-40 scores (median [range]) were higher in the dexamethasone group compared with the control group on POD 1 (167 [133-192] v 157 [108-195]; p < 0.0001) and POD 2 (173 [140-196] v 166 [122-196]; p = 0.001). In the dexamethasone group, improved QoR was observed in the QoR-40 dimensions of emotional state (p = 0.002), physical comfort (p = 0.0001-0.006), and pain (p < 0.0001). The incidences or severity of postoperative fatigue (p < 0.0001), febrile responses (p < 0.0001), and shivering (p = 0.001) were reduced in the dexamethasone group. CONCLUSIONS: Patient-perceived postoperative QoR in cardiac surgical patients is enhanced significantly by small-dose dexamethasone treatment.

Preoperative dexamethasone enhances quality of recovery after laparoscopic cholecystectomy: effect on in-hospital and postdischarge recovery outcomes.

BACKGROUND: The effect of dexamethasone on quality of recovery after discharge from the hospital after laparoscopic surgery has not been examined rigorously in previous investigations. We hypothesized that preoperative dexamethasone would enhance patient-perceived quality of recovery on postoperative day 1 in subjects undergoing laparoscopic cholecystectomy. METHODS: One hundred twenty patients undergoing outpatient laparoscopic cholecystectomy were randomized to receive either dexamethasone (8 mg) or placebo-saline. A 40-item quality-of-recovery scoring system (QoR-40) was administered preoperatively and on postoperative day 1 to all subjects. Nausea, vomiting, fatigue, and pain scores were recorded at the time of discharge from the postanesthesia care unit and ambulatory surgical unit. Hospital length of stay was also assessed. RESULTS: Global QoR-40 scores on postoperative day 1 were higher in the dexamethasone group (median [range], 178 [130-195]) compared with the control group (161 [113-194]) (median difference [99% CI], -18 [-26 to -8]; P < 0.0001). Postoperative QoR-40 scores in the dimensions of emotional state, physical comfort, and pain were all improved in the dexamethasone group compared with the control group (P < 0.001). Nausea, fatigue, and pain scores were all reduced in the dexamethasone group during the hospitalization, as were postoperative analgesic requirements (P < 0.05). Total hospital length of stay was also reduced in subjects administered steroids (P = 0.003). CONCLUSIONS: Among patients undergoing outpatient laparoscopic cholecystectomy surgery, the use of preoperative dexamethasone enhanced postdischarge quality of recovery and reduced nausea, pain, and fatigue in the early postoperative period.

Cerebral oxygen desaturation events assessed by near-infrared spectroscopy during shoulder arthroscopy in the beach chair and lateral decubitus positions.

BACKGROUND: Patients undergoing shoulder surgery in the beach chair position (BCP) may be at risk for adverse neurologic events due to cerebral ischemia. In this investigation, we sought to determine the incidence of cerebral desaturation events (CDEs) during shoulder arthroscopy in the BCP or lateral decubitus position (LDP). METHODS: Data were collected on 124 patients undergoing elective shoulder arthroscopy in the BCP (61 subjects) or LDP (63 subjects). Anesthetic management was standardized in all patients. Regional cerebral tissue oxygen saturation (Scto(2)) was quantified using near-infrared spectroscopy. Baseline heart rate, mean arterial blood pressure, arterial oxygen saturation, and Scto(2) were measured before patient positioning and then every 3 minutes for the duration of the surgical procedure. Scto(2) values below a critical threshold (> or = 20% decrease from baseline or absolute value < or = 55% for >15 seconds) were defined as a CDE and treated using a predetermined protocol. The number of CDEs and types of intervention used to treat low Scto(2) values were recorded. The association between intraoperative CDEs and impaired postoperative recovery was also assessed. RESULTS: Anesthetic management was similar in the BCP and LDP groups, with the exception of more interscalene blocks in the LDP group. Intraoperative hemodynamic variables did not differ between groups. Scto(2) values were lower in the BCP group throughout the intraoperative period (P < 0.0001). The incidence of CDEs was higher in the BCP group (80.3% vs 0% LDP group), as was the median number of CDEs per subject (4, range 0-38 vs 0, range 0-0 LDP group, all P < 0.0001). Among all study patients without interscalene blocks, a higher incidence of nausea (50.0% vs 6.7%, P = 0.0001) and vomiting (27.3% vs 3.3%, P = 0.011) was observed in subjects with intraoperative CDEs compared with subjects without CDEs. CONCLUSIONS: Shoulder surgery in the BCP is associated with significant reductions in cerebral oxygenation compared with values obtained in the LDP.

Fetal oxygen tension promotes tenascin-C-dependent lung branching morphogenesis.

Tenascin-C (TN-C) is a mesenchyme-derived extracellular matrix (ECM) glycoprotein required for fetal lung branching morphogenesis. Given that the low oxygen (O(2)) environment of the fetus is also essential for normal lung branching morphogenesis, we determined whether fetal O(2) tension supports this process by promoting TN-C expression. Initial studies showed that 15-day fetal rat lung explants cultured for 2 days at 3% O(2) not only branched well, but they also expressed higher levels of TN-C when compared to lungs maintained at 21% O(2), which branched poorly. Antisense oligonucleotide studies demonstrated that TN-C produced in response to 3% O(2) was essential for lung branching morphogenesis. As well, exogenous TN-C protein was shown to promote branching of lung epithelial rudiments cultured at 21% O(2). Because ECM-degrading proteinases are capable of catabolizing TN-C protein, we reasoned that 3% O(2) might promote TN-C deposition by limiting the activity of these enzymes within the fetal lung. Consistent with this idea, gelatin zymography showed that the activity of a 72-kDa gelatinase, identified as matrix metalloproteinase-2 (MMP-2), was lower at 3% O(2) vs. 21% O(2). Furthermore, pharmacologic inhibition of MMP-2 activity in fetal lung explants cultured at 21% O(2) resulted in increased TN-C deposition within the mesenchyme, as well as enhanced branching morphogenesis. Collectively, these studies indicate that fetal O(2) tension promotes TN-C-dependent lung epithelial branching morphogenesis by limiting the proteolytic turnover of this ECM component within the adjacent mesenchyme.

Paired-related homeobox gene Prx1 is required for pulmonary vascular development.

Herein, we show that the paired-related homeobox gene, Prx1, is required for lung vascularization. Initial studies revealed that Prx1 localizes to differentiating endothelial cells (ECs) within the fetal lung mesenchyme, and later within ECs forming vascular networks. To begin to determine whether Prx1 promotes EC differentiation, fetal lung mesodermal cells were transfected with full-length Prx1 cDNA, resulting in their morphological transformation to an endothelial-like phenotype. In addition, Prx1-transformed cells acquired the ability to form vascular networks on Matrigel. Thus, Prx1 might function by promoting pulmonary EC differentiation within the fetal lung mesoderm, as well as their subsequent incorporation into vascular networks. To understand how Prx1 participates in network formation, we focused on tenascin-C (TN-C), an extracellular matrix (ECM) protein induced by Prx1. Immunocytochemistry/histochemistry showed that a TN-C-rich ECM surrounds Prx1-positive pulmonary vascular networks both in vivo and in tissue culture. Furthermore, antibody-blocking studies showed that TN-C is required for Prx1-dependent vascular network formation on Matrigel. Finally, to determine whether these results were relevant in vivo, we examined newborn Prx1-wild-type (+/+) and Prx1-null (-/-) mice and showed that Prx1 is critical for expression of TN-C and lung vascularization. These studies provide a framework to understand how Prx1 controls EC differentiation and their subsequent incorporation into functional pulmonary vascular networks.