Assessments and observations of infection prevention and control practices in US outpatient hemodialysis facilities, 2015-2018: important opportunities for improvement.

Infections cause substantial morbidity and mortality among patients receiving care in outpatient hemodialysis facilities. We describe comprehensive infection prevention assessments by US public health departments using standardized interview and observation tools. Results demonstrated how facility layouts can undermine infection prevention and that clinical practices often fall short of policies.

Ventilator Weaning and Terminal Extubation: Withdrawal of Life-Sustaining Therapy in Children. Secondary Analysis of the Death One Hour After Terminal Extubation Study.

OBJECTIVE: Terminal extubation (TE) and terminal weaning (TW) during withdrawal of life-sustaining therapies (WLSTs) have been described and defined in adults. The recent Death One Hour After Terminal Extubation study aimed to validate a model developed to predict whether a child would die within 1 hour after discontinuation of mechanical ventilation for WLST. Although TW has not been described in children, pre-extubation weaning has been known to occur before WLST, though to what extent is unknown. In this preplanned secondary analysis, we aim to describe/define TE and pre-extubation weaning (PW) in children and compare characteristics of patients who had ventilatory support decreased before WLST with those who did not. DESIGN: Secondary analysis of multicenter retrospective cohort study. SETTING: Ten PICUs in the United States between 2009 and 2021. PATIENTS: Nine hundred thirteen patients 0-21 years old who died after WLST. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: 71.4% ( n = 652) had TE without decrease in ventilatory support in the 6 hours prior. TE without decrease in ventilatory support in the 6 hours prior = 71.4% ( n = 652) of our sample. Clinically relevant decrease in ventilatory support before WLST = 11% ( n = 100), and 17.6% ( n = 161) had likely incidental decrease in ventilatory support before WLST. Relevant ventilator parameters decreased were F io2 and/or ventilator set rates. There were no significant differences in any of the other evaluated patient characteristics between groups (weight, body mass index, unit type, primary diagnostic category, presence of coma, time to death after WLST, analgosedative requirements, postextubation respiratory support modality). CONCLUSIONS: Decreasing ventilatory support before WLST with extubation in children does occur. This practice was not associated with significant differences in palliative analgosedation doses or time to death after extubation.

Hormonal Therapies in Multiple Sclerosis: a Review of Clinical Data.

PURPOSE OF REVIEW: Given the potential for exogenous hormones to influence risk and course of MS, this narrative review aims to summarize current knowledge from observational and interventional studies of exogenous hormones in humans with MS. RECENT FINDINGS: Large randomized clinical trials for combined oral contraceptives and estriol both show modest effect on inflammatory activity, with the latter showing potential neuroprotective effect. After fertility treatment, large actively treated cohorts have not confirmed any elevated risk of relapse. Preclinical data suggest that androgens, selective estrogen receptor modulators (SERMs), and selective androgen receptor modulators (SARMs) may be neuroprotective but clinical data are lacking. Gender affirming treatment, particularly estrogen in trans-women, could possibly be associated with elevated risk of inflammation. For women with MS entering menopause, hormone therapy appears safe during the appropriate menopausal window, but its long-term effects on neuroprotection are unknown. Exogenous hormones, used in varied doses and for diverse indications, have variable effects on MS risk, inflammatory activity, and neuroprotection. Large randomized trials are needed before it is possible to determine the true effect of exogenous hormones in a condition as complex as MS.

Well-being at midlife: Correlates of mental health in ambulatory menopausal women with multiple sclerosis.

BACKGROUND: A majority of women with multiple sclerosis (MS) are diagnosed prior to menopause, yet their experiences during this transition are not well characterized. OBJECTIVES: To explore associations between mental health, sleep, and other quality of life metrics, and vasomotor symptoms (VMSs) in ambulatory, menopausal women with MS. METHODS: A secondary analysis was performed of baseline data from two trials enrolling ambulatory peri/postmenopausal women with MS: NCT02710214 (N = 24, bothersome VMS) and NCT04002934 (ongoing, N = 35, myelin repair). Measures analyzed were 36-Item Short-Form Survey (SF-36) (primary scale: general mental health), subjective sleep quality (Pittsburg Sleep Quality Index), VMS (daily diary, interference), mood (Center for Epidemiologist Studies-Depression Scale (CES-D)), walking impairment (timed 25-foot walk (T25FW)), and global disability (Expanded Disability Status Scale (EDSS)). RESULTS: Participants' characteristics (N = 59) were: mean age 51.8 years (SD = 3.4), mean disease duration 11.3 years (SD = 7.6), median EDSS 3.0 (IQR = 2.0-4.0). Mental health was associated with better sleep quality (rho = -0.41, p = 0.019) and better mood (rho = -0.75, p < 0.001), but not with EDSS or T25FW (rho < 0.20, p > 0.10). Worse sleep quality also correlated with more frequent VMS (rho = 0.41, p = 0.02) and VMS interference (rho = 0.59, p < 0.001). CONCLUSIONS: Findings suggest that optimizing sleep quality, mood, and hot flash quantity/interference could substantially improve mental health in menopausal women with MS-and highlight an important care gap in this population.

Re-WRAP (Remyelination for women at risk of axonal loss and progression): A phase II randomized placebo-controlled delayed-start trial of bazedoxifene for myelin repair in multiple sclerosis.

INTRODUCTION: Multiple sclerosis (MS) is a major cause of disability in young and middle-aged people, and myelin repair therapies are needed to slow or potentially reverse this damage. Bazedoxifene (BZA) is a selective estrogen receptor modulator identified in a novel high-throughput unbiased screen for its remyelinating potential, and its remyelinating effects were demonstrated in pre-clinical models. METHODS: This is a single-center, double blind, randomized, controlled, delayed-start Phase 2 clinical trial (NCT04002934) investigating the remyelinating effects of BZA relative to placebo. Female patients with relapsing-remitting MS, aged 45-60 years (or > 40 if post-menopausal), and ambulatory status (EDSS 0-6 inclusive), will be recruited into a clinical trial with 2 arms of identical design, except that the "Chronic Optic Neuropathy" arm requires additional inclusion criteria of electrophysiological evidence of prior visual pathway demyelination. Clinical, electrophysiological, and imaging evaluations will occur at baseline, 3 months, and 6 months. The primary outcome is change in Myelin Water Fraction (MWF) on MRI within the corpus callosum. Secondary outcomes are: visual evoked potential (VEP) P100 latency, novel digital measures of cognition and activity, and patient reported outcomes. Tertiary outcomes are: safety and tolerability. DISCUSSION: BZA has strong preclinical effects on myelin repair, and in the general population demonstrated benefits in treating postmenopausal osteoporosis. Together, these findings support the rationale for an RCT testing BZA in women with MS, evaluating established neuroimaging and neurovisual measures of myelin repair. Additionally, validating novel digital tools could increase sensitivity to change and inform the duration and design of future clinical trials.

Analgesia and Sedation at Terminal Extubation: A Secondary Analysis From Death One Hour After Terminal Extubation Study Data.

OBJECTIVES: To describe the doses of opioids and benzodiazepines administered around the time of terminal extubation (TE) to children who died within 1 hour of TE and to identify their association with the time to death (TTD). DESIGN: Secondary analysis of data collected for the Death One Hour After Terminal Extubation study. SETTING: Nine U.S. hospitals. PATIENTS: Six hundred eighty patients between 0 and 21 years who died within 1 hour after TE (2010-2021). MEASUREMENTS AND MAIN RESULTS: Medications included total doses of opioids and benzodiazepines 24 hours before and 1 hour after TE. Correlations between drug doses and TTD in minutes were calculated, and multivariable linear regression performed to determine their association with TTD after adjusting for age, sex, last recorded oxygen saturation/F io2 ratio and Glasgow Coma Scale score, inotrope requirement in the last 24 hours, and use of muscle relaxants within 1 hour of TE. Median age of the study population was 2.1 years (interquartile range [IQR], 0.4-11.0 yr). The median TTD was 15 minutes (IQR, 8-23 min). Forty percent patients (278/680) received either opioids or benzodiazepines within 1 hour after TE, with the largest proportion receiving opioids only (23%, 159/680). Among patients who received medications, the median IV morphine equivalent within 1 hour after TE was 0.75 mg/kg/hr (IQR, 0.3-1.8 mg/kg/hr) ( n = 263), and median lorazepam equivalent was 0.22 mg/kg/hr (IQR, 0.11-0.44 mg/kg/hr) ( n = 118). The median morphine equivalent and lorazepam equivalent rates after TE were 7.5-fold and 22-fold greater than the median pre-extubation rates, respectively. No significant direct correlation was observed between either opioid or benzodiazepine doses before or after TE and TTD. After adjusting for confounding variables, regression analysis also failed to show any association between drug dose and TTD. CONCLUSIONS: Children after TE are often prescribed opioids and benzodiazepines. For patients dying within 1 hour of TE, TTD is not associated with the dose of medication administered as part of comfort care.

Impact of Dose Reduction of Topical Steroids to Manage Adrenal Insufficiency in Pediatric Eosinophilic Esophagitis.

OBJECTIVE: To evaluate the impact of type and dose of swallowed topical steroids (STS) and concurrent steroid therapy on the development and resolution of adrenal insufficiency (AI) in pediatric eosinophilic esophagitis (EoE). METHODS: We performed a retrospective case-control study of pediatric EoE subjects in a single tertiary care center, who were treated with STS for at least 3 months and diagnosed with AI based on a peak stimulated cortisol level of <18 µg/dL (500 nmol/L). Steroid forms and doses, and endoscopy data were collected at the time of AI diagnosis and AI resolution or the last known evaluation. Steroid formulations were converted to a fluticasone-equivalent dose for analysis. RESULTS: Thirty-two EoE subjects with AI were identified, and 20 had AI resolution, including 12 who remained on lower dose STS. Eight of the 32 patients were also treated with extended-release budesonide (ER budesonide), which resulted in a 7-fold higher total daily steroid dose, and thus were analyzed separately. When the 24 cases that were not on ER budesonide were compared to the 81 controls, no difference was found in the STS dose nor total daily steroid dose, although the inhaled steroid dose had marginal significance. Peak eosinophil counts tended to increase when STS doses were decreased, except in subjects on ER budesonide at AI diagnosis. CONCLUSION: Altering the total daily steroid regimen can lead to resolution of AI in patients with EoE, though this may come at the expense of disease control.

Dying to retire or living to work: Challenges facing aging nurses.

ABSTRACT: Nurses who work in high-stress positions may be at risk for health problems that may result in early retirement. This article identifies health risks to aging nurses who continue to work in clinical settings and suggests methods for reducing negative health impacts to prevent them from retiring prematurely.

Extracorporeal Life Support for Respiratory Failure in Patients With Electronic Cigarette or Vaping Product Use-Associated Lung Injury.

OBJECTIVES: Electronic cigarette or vaping product use-associated lung injury is a clinical entity that can lead to respiratory failure and death. Despite the severity of electronic cigarette or vaping product use-associated lung injury, the role of extracorporeal life support in its management remains unclear. Our objective was to describe the clinical characteristics and outcomes of patients with electronic cigarette or vaping product use-associated lung injury who received extracorporeal life support. DESIGN: We performed a retrospective review of records of electronic cigarette or vaping product use-associated lung injury patients who received extracorporeal life support. Standardized data were collected via direct contact with extracorporeal life support centers. Data regarding presentation, ventilatory management, extracorporeal life support details, and outcome were analyzed. SETTING: This was a multi-institutional, international case series with patients from 10 different institutions in three different countries. PATIENTS: Patients who met criteria for confirmed electronic cigarette or vaping product use-associated lung injury (based on previously reported diagnostic criteria) and were placed on extracorporeal life support were included. Patients were identified via literature review and by direct contact with extracorporeal life support centers. MEASUREMENTS AND MAIN RESULTS: Data were collected for 14 patients ranging from 16 to 45 years old. All had confirmed vape use within 3 months of presentation. Nicotine was the most commonly used vaping product. All patients had respiratory symptoms and radiographic evidence of bilateral pulmonary opacities. IV antibiotics and corticosteroids were universally initiated. Patients were intubated for 1.9 days (range, 0-6) prior to extracorporeal life support initiation. Poor oxygenation and ventilation were the most common indications for extracorporeal life support. Five patients showed evidence of ventricular dysfunction on echocardiography. Thirteen patients (93%) were placed on venovenous extracorporeal life support, and one patient required multiple rounds of extracorporeal life support. Total extracorporeal life support duration ranged from 2 to 37 days. Thirteen patients survived to hospital discharge; one patient died of septic shock. CONCLUSIONS: Electronic cigarette or vaping product use-associated lung injury can cause refractory respiratory failure and hypoxemia. These data suggest that venovenous extracorporeal life support can be an effective treatment option for profound, refractory respiratory failure secondary to electronic cigarette or vaping product use-associated lung injury.

Case Report of Spontaneous Bilateral Ovarian Pregnancy in a Nulliparous Lady.

Nontubal ectopic pregnancies, especially ovarian ones, are rare. Here, we report a case of spontaneous bilateral ovarian pregnancy in a 23-year-old nulliparous lady who presented with a three-day history of abdominal pain localized to the right iliac fossa. Laboratory investigations and pelvic US and transvaginal US findings were suggestive of a right ovarian ectopic pregnancy and left ovarian cyst. Following the patient's consent, the gynaecologist laparoscopically removed the right ovarian ectopic pregnancy and performed a left ovarian cystectomy. Histopathology revealed findings of trophoblastic tissue and chorionic villi with products of conception in both ovaries leading to the diagnosis of bilateral spontaneous ectopic pregnancy. Physicians must be mindful in cases that have a similar clinical presentation because an early diagnosis leads to a reduction in the morbidity and mortality of this specific patient population and helps to improve their overall prognosis.

Discharge against medical advice from the emergency department in a university hospital.

BACKGROUND: Patients discharged against medical advice (DAMA) act as a high-risk population for the Emergency Department (ED), regardless of their presentations, and can pose a serious burden for the hospital. This study examines the prevalence, demographic and clinical characteristics, reasons, and clinical outcomes of a small sample of DAMA patients in a teaching university hospital, including readmission, morbidity, and mortality. METHODS: A prospective, descriptive cross-sectional study was conducted in the ED of King Hamad University Hospital (KHUH) with 98,992 patient visits during a 1-year period from June 2018 to June 2019. Consenting DAMA patients were asked to complete a data collection form. RESULTS: Patients (n = 413) had a mean age of 44.1 years with a female majority (57.1%). The majority were categorized as triage level-3 (87.7%). The main reasons for DAMA included refusal of the procedure/operation (23.2%), long ED waiting time (22.2%), subjective improvement with treatment (17.7%), and children at home (14.8%), whereas the least selected reason was dissatisfaction with medical care (1.2%). Follow-up of DAMA patients revealed that 86 cases (20.8%) were readmitted to the ED within 72 h of which 41 (47.7%) cases were morbidity and 2 (2.3%) were mortality. Marital status was a predictor of DAMA patients who revisit the ED within 72 h. CONCLUSION: The results act as a pilot study to examine a small sample of DAMA patients' characteristics, diagnosis, and ED revisits. Hospitals should investigate further the DAMA population on a larger scale, reasons for refusing procedures, and utilize this knowledge to improve the healthcare process.

Gaps in infection prevention practices for catheter-associated urinary tract infections and central line-associated bloodstream infections as identified by the Targeted Assessment for Prevention Strategy.

BACKGROUND: Catheter-associated urinary tract infections (CAUTI) and central line-associated bloodstream infections (CLABSI) represent a substantial portion of health care-associated infections (HAIs) reported in the United States. The Targeted Assessment for Prevention Strategy is a quality improvement framework to reduce health care-associated infections. Data from the Targeted Assessment for Prevention Facility Assessments were used to determine common infection prevention gaps for CAUTI and CLABSI. METHODS: Data from 2,044 CAUTI and 1,680 CLABSI assessments were included in the analysis. Items were defined as potential gaps if ≥33% respondents answered Unknown, ≥33% No, or ≥50% No or Unknown or Never, Rarely, Sometimes, or Unknown to questions pertaining to those areas. Review of response frequencies and stratification by respondent role were performed to highlight opportunities for improvement. RESULTS: Across CAUTI and CLABSI assessments, lack of physician champions (<35% Yes) and nurse champions (<55% Yes), along with lack of awareness of competency assessments, audits, and feedback were reported. Lack of practices to facilitate timely removal of urinary catheters were identified for CAUTI and issues with select device insertion practices, such as maintaining aseptic technique, were perceived as areas for improvement for CLABSI. CONCLUSIONS: These data suggest common gaps in critical components of infection prevention and control programs. The identification of these gaps has the potential to inform targeted CAUTI and CLABSI prevention efforts.

Application of a Standard Cross-Specialty Workup for Diagnosis and Metabolic Screening of Obese Adolescents With Polycystic Ovary Syndrome.

PURPOSE: Polycystic ovary syndrome (PCOS) is a common disorder. The used diagnostic criteria and screening for related metabolic disease in adolescent girls with PCOS vary with medical specialty, causing discrepancies in care. We sought to improve and standardize care to guidelines for adolescents with PCOS and obesity. METHODS: This is a quality improvement project in a multispecialty, tertiary care children's hospital. Providers in Adolescent Medicine, Gynecology, Pediatric Endocrinology, and Endocrine-Metabolic clinics participated. Diagnostic testing and metabolic screening data were collected for new patient encounters with PCOS and obesity, with two improvement cycles from December 2012 to March 2017. Providers received education on diagnostics tests and metabolic screening recommendations, and electronic medical record tools were created. The number of diagnostic and metabolic screening tests ordered per cycle and per clinic were analyzed and compared with baseline. RESULTS: The preintervention cycle included 74 encounters-44 in Cycle 1 and 58 in Cycle 2. Diagnostic test completeness improved by Cycle 2 (46%-68%) for all clinics. Screening for metabolic disease only improved in Gynecology (27%-71%). Adolescent Medicine, Endocrinology, and Endocrine-Metabolic used note templates, and Adolescent Medicine and Gynecology used order sets. Note templates were associated with more diagnostic tests ordered in Endocrinology (30%-88%; p = .002). CONCLUSIONS: Implementation of quality improvement measures improved the number of diagnostic and metabolic screening tests performed in new patient encounters for PCOS, although the most effective strategy varied by clinic type and electronic medical record habits. Similar efforts should be implemented to standardize care at other institutions.

Healthcare providers' perspectives on family presence during resuscitation in the emergency departments of the Kingdom of Bahrain.

BACKGROUND: Worldwide, policies exist on family presence during resuscitation (FPDR), however, this is still lacking in the Gulf Corporation Countries (GCC) in general and in the Kingdom of Bahrain in particular. The aim of this study is to assess the perspectives of healthcare providers (HP) on FPDR among those working in the emergency departments (EDs) in the Kingdom. METHODS: A self-administered anonymous electronic survey was collected from 146 HPs (emergency physicians and nurses) working in the three major EDs in the Kingdom of Bahrain. Besides demographic data, 18 items measuring HPs' perceptions of FPDR were generated using the 5-point Likert scale. RESULTS: Surveys (n = 146) from physicians and nurses were analysed (45.9% vs. 54.1%, respectively). There were significant differences between physicians and nurses in terms of personal beliefs, FPDR enhancing professional satisfaction and behaviour, and the importance of a support person and saying goodbye (p < 0.001). However, general responses demonstrated that the majority of HPs encouraged and supported FPDR, but with greater support from physicians than nurses. CONCLUSION: The study reflects that many HPs in EDs participated in and are familiar with FPDR, with the majority of ED physicians supporting it. Further studies should investigate the reasons for the lack of support from nurses. Results may contribute to the development of hospital ED policies that allow FPDR in the region.

Clinical Features of E-cigarette, or Vaping, Product Use-Associated Lung Injury in Teenagers.

BACKGROUND: In the United States in 2019, there was an outbreak of electronic cigarette, or vaping, product use-associated lung injury (EVALI). The manifestations of EVALI in adolescents are not well characterized. We describe the diagnosis, evaluation, and management of EVALI in adolescents hospitalized at a tertiary care, university-affiliated children's hospital. METHODS: A multidisciplinary committee developed an EVALI algorithm on the basis of guidelines from the Centers for Disease Control and Prevention. A retrospective chart review was conducted on patients diagnosed with EVALI. Descriptive analyses included sociodemographic characteristics, clinical presentation, laboratory and imaging results, pulmonary function testing, oxygen requirements, and clinic follow-up. RESULTS: Thirteen hospitalized adolescents were diagnosed with confirmed or probable EVALI. The majority were female (54%) with a mean age of 15.9 years. Sixty-nine percent of patients presented with respiratory symptoms, whereas gastrointestinal symptoms were prominent in 85% of patients. Vaping Δ-9-tetrahydrocannabinol was reported in 92% of patients, and vaping nicotine was reported in 62% of patients. All had bilateral ground-glass opacities on the chest computed tomography (CT) scan. Treatment with glucocorticoids led to clinical improvement in 11 of 12 patients. Treatment with glucocorticoids led to improvement in both forced expiratory volume in 1 second and forced vital capacity (P < .05). Four patients required home oxygen on the basis of 6-minute walk test results. CONCLUSIONS: Diagnosis of EVALI should be suspected on the basis of vaping history and clinical presentation. Glucocorticoid treatment led to an improvement in symptoms and lung function. The 6-minute walk test may help determine oxygen needs at discharge.

Review of Health Consequences of Electronic Cigarettes and the Outbreak of Electronic Cigarette, or Vaping, Product Use-Associated Lung Injury.

Electronic cigarettes (e-cigarettes) are battery-operated devices to insufflate nicotine or other psychoactive e-liquid aerosols. Despite initial claims of e-cigarettes as a nicotine-cessation device, aggressive marketing of e-cigarettes has led to an explosion in adolescents' and young adults' use over the last few years. Coupled with a lack of adequate investigation and regulation of e-cigarettes, the USA is facing an outbreak of e-cigarette, or vaping, product use-associated lung injury (EVALI) starting in mid-2019. While little long-term health hazard data are available, the components and constituents of e-cigarettes may adversely impact health. Propylene glycol and glycerin are humectants (water-retaining excipients) that generate pulmonary irritants and carcinogenic carbonyl compounds (e.g., formaldehyde, acetaldehyde, and acrolein) when heated in e-cigarettes. Metals contained in heating coils and cartridge casings may leach metals such as aluminum, chromium, iron, lead, manganese, nickel, and tin. Flavoring agents are considered safe for ingestion but lack safety data for inhalational exposures. Diacetyl, a common buttery flavoring agent, has known pulmonary toxicity with inhalational exposures leading to bronchiolitis obliterans. In 2019, clusters of lung injury associated with e-cigarette use were identified in Wisconsin and Illinois. Patients with EVALI present with a constellation of respiratory, gastrointestinal, and constitutional symptoms. Radiographically, patients have bilateral ground glass opacifications. As of February 18, 2020, the Centers for Disease Control has identified 2807 hospitalized patients diagnosed with either "confirmed" or "probable" EVALI in the US. Currently, vitamin E acetate (VEA) used as a diluent in tetrahydrocannabinol vape cartridges is implicated in EVALI. VEA cuts tetrahydrocannabinol oil without changing the appearance or viscosity. When inhaled, pulmonary tissue lacks the mechanism to metabolize and absorb VEA, which may lead to its accumulation. While most EVALI patients were hospitalized, treatment remains largely supportive, and use of corticosteroids has been associated with clinical improvement. The outbreak of EVALI highlights the need for regulation of e-cigarette devices and e-liquids. Clinicians need to be aware of the health hazards of e-cigarettes and be vigilant in asking about vaping.

Severe E-Cigarette, or Vaping, Product Use Associated Lung Injury Requiring Venovenous Extracorporeal Membrane Oxygenation.

OBJECTIVES: To report a severe case of e-cigarette or vaping product use-associated lung injury with complex course requiring venovenous extracorporeal membrane oxygenation. DESIGN: Case report. SETTING: PICU in an academic medical center. PATIENTS: A 16-year-old girl presenting with gastrointestinal and respiratory symptoms was admitted to our PICU after having progressive respiratory failure and bilateral pulmonary ground-glass opacities on chest CT. INTERVENTIONS: Venovenous extracorporeal membrane oxygenation MEASUREMENTS AND MAIN RESULTS:: After extensive infectious workup was unrevealing, she reported a history of vaping e-cigarette containing either nicotine or delta-9-tetrahydrocannabinol oil prior to symptom onset. She was given a presumptive diagnosis of e-cigarette or vaping product use-associated lung injury. The PICU team in consultation with pulmonology and medical toxicology started high-dose IV methylprednisolone 1 mg/kg bid. Despite initial improvements, she continued to require positive pressure ventilation and developed pneumomediastinum with progression to tension pneumothoraces and a persistent air leak. Unable to maintain her oxygenation, she was placed on venovenous extracorporeal membrane oxygenation for a prolonged course and had a tracheostomy placement. The clinical course, severity, and range of interventions in affected patients around the country have varied widely. Respiratory symptoms have been the most severe, but the constellation of symptoms in e-cigarette or vaping product use-associated lung injury include constitutional symptoms (fevers, weight-loss) and gastrointestinal symptoms (nausea, vomiting, diarrhea). In many cases, steroid use led to rapid clinical improvements. However, other cases with severe illness, like our patient, necessitated high-dose IV steroids, intubation, and venovenous extracorporeal membrane oxygenation. The underlying etiology and pathophysiology of e-cigarette or vaping product use-associated lung injury remains unknown. The Centers for Disease Control and Prevention in conjunction with state/local health departments and the Food and Drug Administration is actively investigating the outbreak. CONCLUSIONS: Clinicians need to be aware of the current outbreak of e-cigarette or vaping product use-associated lung injury and ask about vaping in patients presenting with gastrointestinal and respiratory symptoms. Treatment options are anecdotal and necessitate a multidisciplinary approach.

Evidence of Systemic Absorption of Enteral Budesonide in Patients with Fontan-Associated Protein-Losing Enteropathy.

To evaluate for evidence of systemic glucocorticoid absorption in cases of Fontan-associated protein-losing enteropathy (PLE) treated with enteral budesonide, we reviewed the charts of 27 patients with Fontan-associated PLE followed at Children's Hospital Colorado from 2005 to 2018. Cases were excluded for lack of budesonide thserapy or a treatment duration of less than 6 months. Charts were examined by two endocrinologists for review of prior biochemical endocrine evaluations, alterations in linear growth, and physical exam findings consistent with steroid excess. Twelve patients met inclusion criteria. Eight had prior documented cortisol screening. Three patients were tested while on treatment with a median fasting AM cortisol of 0.9 mcg/dL; two of these had a concomitantly measured ACTH, both below the detectable limit. Five patients were tested while weaning or having discontinued budesonide, with a median fasting AM cortisol of 9.1 mcg/dL. Eleven patients had decreases in height velocity associated with starting budesonide. Six patients had documentation of cushingoid features by an endocrinologist. In this cohort of children treated with budesonide for PLE following Fontan, clinical signs of systemic glucocorticoid absorption were frequent. Cortisol secretion was suppressed while on therapy, with adrenal recovery noted once budesonide was discontinued. Growth failure and cushingoid features were common findings. While these findings should be confirmed in larger cohorts, we recommend that the evaluation for systemic absorption of exogenous steroids be considered in patients treated with long-term enteral budesonide given the potential risk for adrenal crisis in times of physiologic stressors.

Growth hormone deficiency in megalencephaly-capillary malformation syndrome: An association with activating mutations in PIK3CA.

Megalencephaly-capillary malformation syndrome (MCAP) is a brain overgrowth disorder characterized by cortical malformations (specifically polymicrogyria), vascular anomalies, and segmental overgrowth secondary to somatic activating mutations in the PI3K-AKT-MTOR pathway (PIK3CA). Cases of growth failure and hypoglycemia have been reported in patients with MCAP, raising the suspicion for unappreciated growth hormone (GH) deficiency. Here we report an observational multicenter study of children with MCAP and GH deficiency. Eleven participants were confirmed to have GH deficiency, all with very low or undetectable circulating concentrations of insulin-like growth factor-1 and insulin-like growth factor binding protein-3. Seven underwent GH stimulation testing and all had insufficient responses with a median GH peak of 3.7 ng/ml (range 1.1-8.6). Growth patterns revealed a drastic decline in length z-scores within the first year of life but then stabilized afterward. Five were treated with GH; one discontinued due to inconsolability. The other four participants continued on GH with improvement in linear growth velocity. Other endocrinopathies were identified in 7 of the 11 participants in this cohort. This study indicates that GH deficiency is associated with MCAP and that children with MCAP and hypoglycemia and/or postnatal growth failure should be evaluated for GH deficiency and other endocrinopathies.

Subscapularis tenotomy versus lesser tuberosity osteotomy during total shoulder arthroplasty for primary osteoarthritis: a prospective, randomized controlled trial.

BACKGROUND: There is no current consensus on subscapularis mobilization during total shoulder arthroplasty. The purpose of this prospective, randomized controlled trial was to compare functional and radiographic outcomes of the more traditional subscapularis tenotomy (ST) versus lesser tuberosity osteotomy (LTO). METHODS: This study enrolled 60 shoulders in 59 patients with primary osteoarthritis. Thirty shoulders were preoperatively randomized to each group. Preoperative and 6-week, 3-month, 6-month, and 1-year postoperative data were collected. Ultrasound was performed at 3 months to evaluate subscapularis healing in tenotomy subjects, whereas radiographs were used to evaluate osteotomy healing. Intraoperative data included operative time, tenotomy or osteotomy repair time, and osteotomy thickness. RESULTS: No significant differences in range of motion or clinical outcomes occurred at baseline or 1 year postoperatively between the 2 groups. The mean total case duration for ST was significantly less than that for LTO (129.3 minutes vs 152.7 minutes), along with a significantly shorter subscapularis repair time for ST (34.3 minutes vs 39.3 minutes, P = .024). At final follow-up, 27 of 29 LTO shoulders (93.1%) showed bone-to-bone healing on radiographs, whereas 26 of 30 ST shoulders (86.7%) had no full-thickness tear of the subscapularis on ultrasound at 3 months. CONCLUSIONS: Both techniques produced successful objective and subjective clinical outcomes. LTO heals more reliably than ST. Mean total case and subscapularis repair times were significantly greater for LTO than for ST.