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All for One and One Delivery Room Approach for All?

Multiple births are increasing in frequency related to advanced maternal age and fertility treatments, and they have an increased risk for congenital anomalies compared to singleton births. However, twins have the same congenital anomalies <15% of the time. Thus, having multiple births with discordant anomalies is a growing challenge for neonatologists. Although external anomalies can often be spotted quickly at delivery or sex differences between multiples can rapidly identify those with internal anomalies described on prenatal ultrasound, we present a case of male multiples, who would optimally receive different initial resuscitation strategies on the basis of the presence or absence of an internal anomaly. The similar size of 4 extremely preterm quadruplets raises concern for whether accurate, immediate identification of 1 neonate with a congenital diaphragmatic hernia will be reliable in the delivery room. Clinicians discuss the ethical considerations of an "all for one" approach to this resuscitation.


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Bereaved Parents, Hope, and Realism


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A Triadic Intervention for Adolescent Sexual Health: A Randomized Clinical Trial

OBJECTIVES:

In this study, we evaluate the efficacy of Families Talking Together (FTT), a triadic intervention to reduce adolescent sexual risk behavior.

METHODS:

Adolescents aged 11 to 14 and their female caregivers were recruited from a pediatric clinic; 900 families were enrolled; 84 declined. Families were randomly assigned to FTT or 1 of 2 control conditions. The FTT triadic intervention consisted of a 45-minute face-to-face session for mothers, health care provider endorsement of intervention content, printed materials for families, and a booster call for mothers. The primary outcomes were ever having had vaginal intercourse, sexual debut within the past 12 months, and condom use at last sexual intercourse. Assessments occurred at baseline, 3 months post baseline, and 12 months post baseline.

RESULTS:

Of enrolled families, 73.4% identified as Hispanic, 20.4% as African American, and 6.2% as mixed race. Mean maternal age was 38.8 years, and mean adolescent grade was seventh grade. At the 12-month follow-up, 5.2% of adolescents in the experimental group reported having had sexual intercourse, compared with 18% of adolescents in the control groups (P < .05). In the experimental group, 4.7% of adolescents reported sexual debut within the past 12 months, compared with 14.7% of adolescents in the control group (P < .05). In the experimental group, 74.2% of sexually active adolescents indicated using a condom at last sexual intercourse, compared with 49.1% of adolescents in the control group (P < .05).

CONCLUSIONS:

This research suggests that the FTT triadic intervention is efficacious in delaying sexual debut and reducing sexual risk behavior among adolescents.


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Enhancing CPR During Transition From Prehospital to Emergency Department: A QI Initiative

BACKGROUND AND OBJECTIVES:

High-quality cardiopulmonary resuscitation (CPR) increases the likelihood of survival of pediatric out-of-hospital cardiac arrest (OHCA). Maintenance of high-quality CPR during transition of care between prehospital and pediatric emergency department (PED) providers is challenging. Our objective for this initiative was to minimize pauses in compressions, in alignment with American Heart Association recommendations, for patients with OHCA during the handoffs from prehospital to PED providers. We aimed to decrease interruptions in compressions during the first 2 minutes of PED care from 17 seconds (baseline data) to 10 seconds over 12 months. Our secondary aims were to decrease the length of the longest pause in compressions to <10 seconds and eliminate encounters in which time to defibrillator pad placement was >120 seconds.

METHODS:

Our multidisciplinary team outlined our theory for improvement and designed interventions aimed at key drivers. Interventions included specific roles and responsibilities, CPR handoff choreography, and empowerment of frontline providers. Data were abstracted from video recordings of patients with OHCA receiving manual CPR on arrival.

RESULTS:

We analyzed 33 encounters between March 2018 and July 2019. We decreased total interruptions from 17 to 12 seconds during the first 2 minutes and decreased the time of the longest single pause from 14 to 7 seconds. We saw a decrease in variability of time to defibrillator pad placement.

CONCLUSIONS:

Implementation of a quality improvement initiative involving CPR transition choreography resulted in decreased interruptions in compressions and decreased variability of time to defibrillator pad placement.


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{alpha}-Lipoic Acid (ALA) Improves Cystine Solubility in Cystinuria: Report of 2 Cases

Cystinuria is an autosomal recessive disorder characterized by excessive urinary excretion of cystine, resulting in recurrent cystine kidney stones, often presenting in childhood. Current treatment options for cystinuria include dietary and/or fluid measures and potassium citrate to reduce cystine excretion and/or increase solubility. Tiopronin and D-penicillamine are used in refractory cases to bind cystine in urine, albeit with serious side effects. A recent study revealed efficacy of nutritional supplement α-lipoic acid (ALA) treatment in preventing kidney stones in a mouse model of cystinuria. Here, we report 2 pediatric patients (6 and 15 years old) with cystinuria who received regular doses of ALA in addition to conventional therapy with potassium citrate. Both patients tolerated ALA without any adverse effects and had reduced frequency of symptomatic and asymptomatic kidney stones with disappearance of existing kidney stones in 1 patient after 2 months of ALA therapy. ALA treatment markedly improved laboratory markers of cystine solubility in urine with increased cystine capacity (–223 to –1 mg/L in patient 1 and +140 to +272 mg/L in patient 2) and decreased cystine supersaturation (1.7 to 0.88 in patient 1 and 0.64 to 0.48 in patient 2) without any changes in cystine excretion or urine pH. Our findings suggest that ALA improves solubility of cystine in urine and prevents stone formation in patients with cystinuria who do not respond to diet and citrate therapy.


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Teenage Use of Smartphone Applications for Menstrual Cycle Tracking


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Climate Change as a Social Determinant of Health


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ACA Medicaid Expansion and Insurance Coverage Among New Mothers Living in Poverty

BACKGROUND:

Medicaid plays a critical role during the perinatal period, but pregnancy-related Medicaid eligibility only extends for 60 days post partum. In 2014, the Affordable Care Act’s (ACA’s) Medicaid expansions increased adult Medicaid eligibility to 138% of the federal poverty level in participating states, allowing eligible new mothers to remain covered after pregnancy-related coverage expires. We investigate the impact of ACA Medicaid expansions on insurance coverage among new mothers living in poverty.

METHODS:

We define new mothers living in poverty as women ages 19 to 44 with incomes below the federal poverty level who report giving birth in the past 12 months. We use 2010–2017 American Community Survey data and a difference-in-differences approach using parental Medicaid-eligibility thresholds to estimate the effect of ACA Medicaid expansions on insurance coverage among poor new mothers.

RESULTS:

A 100-percentage-point increase in parental Medicaid-eligibility is associated with an 8.8-percentage-point decrease (P < .001) in uninsurance, a 13.2-percentage-point increase (P < .001) in Medicaid coverage, and a 4.4-percentage-point decrease in private or other coverage (P = .001) among poor new mothers. The average increase in Medicaid eligibility is associated with a 28% decrease in uninsurance, a 13% increase in Medicaid coverage, and an 18% decline in private or other insurance among poor new mothers in expansion states. However, in 2017, there were ~142 000 remaining uninsured, poor new mothers.

CONCLUSIONS:

ACA Medicaid expansions are associated with increased Medicaid coverage and reduced uninsurance among poor new mothers. Opportunities remain for expansion and nonexpansion states to increase insurance coverage among new mothers living in poverty.


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Asking for Identification and Retail Tobacco Sales to Minors

BACKGROUND AND OBJECTIVES:

A previous single-county study found that retail stores usually asked young-looking tobacco customers to show proof-of-age identification, but a large proportion of illegal tobacco sales to minors occurred after the customers had shown identification proving they were too young to purchase tobacco. We sought to investigate these findings on a larger scale.

METHODS:

We obtained state reports for federal fiscal years 2017 and 2018 from a federal agency that tracks tobacco sales to supervised minors conducting compliance checks in retail stores. We used descriptive and multivariable logistic regression methods to determine (1) how often stores in 17 states requested identifications, (2) what proportion of violations occurred after identification requests, and (3) if violation rates differed when minors were required versus forbidden to carry identification.

RESULTS:

Stores asked minors for identification in 79.6% (95% confidence interval: 79.3%–80.8%) of compliance checks (N = 17 276). Violations after identification requests constituted 22.8% (95% confidence interval: 20.0%–25.6%; interstate range, 1.7%–66.2%) of all violations and were nearly 3 times as likely when minors were required to carry identification in compliance checks. Violations were 42% more likely when minors asked for a vaping product versus cigarettes.

CONCLUSIONS:

Stores that sell tobacco to underage customers are more likely to be detected and penalized when youth inspectors carry identification during undercover tobacco sales compliance checks. The new age-21 tobacco sales requirement presents an opportunity to require identifications be carried and address other long-standing weaknesses in compliance-check protocols to help combat the current adolescent vaping epidemic.


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Racial and Ethnic Differences in Emergency Department Pain Management of Children With Fractures

OBJECTIVES:

To test the hypotheses that minority children with long-bone fractures are less likely to (1) receive analgesics, (2) receive opioid analgesics, and (3) achieve pain reduction.

METHODS:

We performed a 3-year retrospective cross-sectional study of children <18 years old with long-bone fractures using the Pediatric Emergency Care Applied Research Network Registry (7 emergency departments). We performed bivariable and multivariable logistic regression to measure the association between patient race and ethnicity and (1) any analgesic, (2) opioid analgesic, (3) ≥2-point pain score reduction, and (4) optimal pain reduction (ie, to mild or no pain).

RESULTS:

In 21 069 visits with moderate-to-severe pain, 86.1% received an analgesic and 45.4% received opioids. Of 8533 patients with reassessment of pain, 89.2% experienced ≥2-point reduction in pain score and 62.2% experienced optimal pain reduction. In multivariable analyses, minority children, compared with non-Hispanic (NH) white children, were more likely to receive any analgesics (NH African American: adjusted odds ratio [aOR] 1.72 [95% confidence interval 1.51–1.95]; Hispanic: 1.32 [1.16–1.51]) and achieve ≥2-point reduction in pain (NH African American: 1.42 [1.14–1.76]; Hispanic: 1.38 [1.04–1.83]) but were less likely to receive opioids (NH African American: aOR 0.86 [0.77–0.95]; Hispanic: aOR 0.86 [0.76–0.96]) or achieve optimal pain reduction (NH African American: aOR 0.78 [0.67–0.90]; Hispanic: aOR 0.80 [0.67–0.95]).

CONCLUSIONS:

There are differences in process and outcome measures by race and ethnicity in the emergency department management of pain among children with long-bone fractures. Although minority children are more likely to receive analgesics and achieve ≥2-point reduction in pain, they are less likely to receive opioids and achieve optimal pain reduction.


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E-cigarette or Vaping Product Use-Associated Lung Injury (EVALI) Without Respiratory Symptoms

Electronic cigarette or vaping product use–associated lung injury (EVALI) is a newly emerging diagnosis in the United States, yet the incidence has surged greatly in the past year. With the trend of using electronic cigarettes (e-cigarettes) and vaping rising at an alarming rate among teenagers, many are resorting to friends, illicit drug dealers, and other informal sources to obtain their e-cigarettes, which is greatly contributing to the national outbreak of EVALI. The incidence of adolescents presenting with the constellation of respiratory, gastrointestinal, and constitutional symptoms characteristic of EVALI has been widely reported within the nation. We present one such case of an adolescent boy with a 2-year history of daily vaping who presented with nausea, vomiting, weight loss, and fever but lacked the respiratory symptoms that have been reported in the majority of EVALI cases reported thus far. Computed tomography scan of the abdomen and pelvis revealed an incidental finding of lung pathology characteristic of EVALI, prompting further workup and diagnosis of EVALI. In this case, it is demonstrated that the presentation of EVALI can be variable and is still poorly defined. The rising morbidity and mortality from EVALI reveal the importance of considering EVALI in all patients with a history of vaping or e-cigarette use, regardless of the presence or absence of respiratory symptoms.


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Parental Considerations Regarding Cure and Late Effects for Children With Cancer

BACKGROUND:

More than 80% of children with cancer become long-term survivors, yet most survivors experience late effects of treatment. Little is known about how parents and physicians consider late-effects risks against a potential survival benefit when making treatment decisions.

METHODS:

We used a discrete choice experiment to assess the importance of late effects on treatment decision-making and acceptable trade-offs between late-effects risks and survival benefit. We surveyed 95 parents of children with cancer and 41 physicians at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center to assess preferences for 5 late effects of treatment: neurocognitive impairment, infertility, cardiac toxicity, second malignancies, and impaired growth and development.

RESULTS:

Each late effect had a statistically significant association with treatment choice, as did survival benefit (P < .001). Avoidance of severe cognitive impairment was the most important treatment consideration to parents and physicians. Parents also valued cure and decreased risk of second malignancies; physician decision-making was driven by avoidance of second malignancies and infertility. Both parents and physicians accepted a high risk of infertility (parents, a 137% increased risk; physicians, an 80% increased risk) in exchange for a 10% greater chance of cure.

CONCLUSIONS:

Avoidance of severe neurocognitive impairment was the predominant driver of parent and physician treatment preferences, even over an increased chance of cure. This highlights the importance of exploring parental late-effects priorities when discussing treatment options.


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Trends in Outpatient Procedural Sedation: 2007-2018

BACKGROUND:

Pediatric subspecialists routinely provide procedural sedation outside the operating room. No large study has reported trends in outpatient pediatric procedural sedation. Our purpose in this study was to identify significant trends in outpatient procedural sedation using the Pediatric Sedation Research Consortium.

METHODS:

Prospectively collected data from 2007 to 2018 were used for trending procedural sedation. Patient characteristics, medications, type of providers, serious adverse events, and interventions were reported. The Cochran–Armitage test for trend was used to explore the association between the year and a given characteristic.

RESULTS:

A total of 432 842 sedation encounters were identified and divided into 3 4-year epochs (2007–2011, 2011–2014, and 2014–2018). There was a significant decrease in infants <3 months of age receiving procedural sedation (odds ratio = 0.97; 95% confidence interval, 0.96–0.98). A large increase was noticed in pediatric hospitalists providing procedural sedation (0.6%–9.5%; P < .001); there was a decreasing trend in sedation by other providers who were not in emergency medicine, critical care, or anesthesiology (13.9%–3.9%; P < .001). There was an increasing trend in the use of dexmedetomidine (6.3%–9.3%; P < .001) and a decreasing trend in the use of chloral hydrate (6.3%–0.01%; P < .001) and pentobarbital (7.3%–0.5%; P < .001). Serious adverse events showed a nonsignificant increase overall (1.35%–1.75%).

CONCLUSIONS:

We report an increase in pediatric hospitalists providing sedation and a significant decrease in the use of chloral hydrate and pentobarbital by providers. Further studies are required to see if sedation services decrease costs and optimize resource use.


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Providing the Evidence for Managing Depression in Pregnancy


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Optimizing Human Papillomavirus Immunization: The Role of Centralized Reminder and Recall Systems


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Every Child Counts: The Importance of the 2020 Census for Pediatric Health Equity


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Exploring Early Childhood Factors as an Avenue to Address Chronic Peer Victimization


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Adolescent Sexual Health Interventions: Innovation, Efficacy, Cost, and the Urgent Need to Scale


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Family Values Means Covering Families: Parents Need to Focus on Parenting, Not Access to Care


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The Time Is Now: Standardized Sedation Training for Pediatric Hospitalists


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Ahmed A, Fend PI, Gaensbauer JT, Reves RR, Khurana R, Salcedo K, Punnoose R, Katz DJ, for the TUBERCULOSIS EPIDEMIOLOGIC STUDIES CONSORTIUM. Interferon-{gamma} Release Assays in Children <15 Years of Age. Pediatrics. 2020:145(1):e20191930


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Early and Often: The Need for Comprehensive Discussion of Treatment-Induced Cancer Late Effects


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Implicit Bias in Pediatrics: An Emerging Focus in Health Equity Research


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Ames SG, Davis BS, Marin JR, L. Fink EL, Olson LM, Gausche-Hill M, Kahn JM. Emergency Department Pediatric Readiness and Mortality in Critically Ill Children. Pediatrics. 2019;144(3):e20190568


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Children With Intellectual and Developmental Disabilities as Organ Transplantation Recipients

The demand for transplantable solid organs far exceeds the supply of deceased donor organs. Patient selection criteria are determined by individual transplant programs; given the scarcity of solid organs for transplant, allocation to those most likely to benefit takes into consideration both medical and psychosocial factors. Children with intellectual and developmental disabilities have historically been excluded as potential recipients of organ transplants. When a transplant is likely to provide significant health benefits, denying a transplant to otherwise eligible children with disabilities may constitute illegal and unjustified discrimination. Children with intellectual and developmental disabilities should not be excluded from the potential pool of recipients and should be referred for evaluation as recipients of solid organ transplants.


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Providing Care for Infants Born at Home

The American Academy of Pediatrics (AAP) believes that current data show that hospitals and accredited birth centers are the safest settings for birth in the United States. The AAP does not recommend planned home birth, which has been reported to be associated with a twofold to threefold increase in infant mortality in the United States. The AAP recognizes that women may choose to plan a home birth. This statement is intended to help pediatricians provide constructive, informed counsel to women considering home birth while retaining their role as child advocates and to summarize appropriate care for newborn infants born at home that is consistent with care provided for infants born in a medical care facility. Regardless of the circumstances of his or her birth, including location, every newborn infant deserves health care consistent with that highlighted in this statement, which is more completely described in other publications from the AAP, including Guidelines for Perinatal Care and the Textbook of Neonatal Resuscitation. All health care clinicians and institutions should promote communications and understanding on the basis of professional interaction and mutual respect.


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Emerging Issues in Male Adolescent Sexual and Reproductive Health Care

Pediatricians are encouraged to address male adolescent sexual and reproductive health on a regular basis, including taking a sexual history, discussing healthy sexuality, performing an appropriate physical examination, providing patient-centered and age-appropriate anticipatory guidance, and administering appropriate vaccinations. These services can be provided to male adolescent patients in a confidential and culturally appropriate manner, can promote healthy sexual relationships and responsibility, can and involve parents in age-appropriate discussions about sexual health.


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Nickel Allergic Contact Dermatitis: Identification, Treatment, and Prevention

Nickel is a ubiquitous metal added to jewelry and metallic substances for its hardening properties and because it is inexpensive. Estimates suggest that at least 1.1 million children in the United States are sensitized to nickel. Nickel allergic contact dermatitis (Ni-ACD) is the most common cutaneous delayed-type hypersensitivity reaction worldwide. The incidence among children tested has almost quadrupled over the past 3 decades. The associated morbidities include itch, discomfort, school absence, and reduced quality of life. In adulthood, individuals with Ni-ACD may have severe disabling hand eczema. The increasing rate of Ni-ACD in children has been postulated to result from early and frequent exposure to metals with high amounts of nickel release (eg, as occurs with ear piercing or with products used daily in childhood such as toys, belt buckles, and electronics).

To reduce exposure to metal sources with high nickel release by prolonged and direct contact with human skin, Denmark and the European Union legislated a directive several decades ago with the goal of reducing high nickel release and the incidence of Ni-ACD. Since then, there has been a global reduction in incidence of Ni-ACD in population-based studies of adults and studies of children and young adults being tested for allergic contact dermatitis. These data point to nickel exposure as a trigger for elicitation of Ni-ACD and, further, provide evidence that legislation can have a favorable effect on the economic and medical health of a population.

This policy statement reviews the epidemiology, history, and appearances of Ni-ACD. Examples of sources of high nickel release are discussed to highlight how difficult it is to avoid this metal in modern daily lives. Treatments are outlined, and avoidance strategies are presented. Long-term epidemiological interventions are addressed. Advocacy for smarter nickel use is reviewed. The American Academy of Pediatrics supports US legislation that advances safety standards (as modeled by the European Union) that protect children from early and prolonged skin exposure to high–nickel-releasing items. Our final aim for this article is to aid the pediatric community in developing nickel-avoidance strategies on both individual and global levels.


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Surviving Sepsis Campaign International Guidelines


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Initial Resuscitation Algorithm for Children


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Shellhaas RA, Burns JW, Barks JDE, Fauziya Hassan F, Chervin RD. Maternal Voice and Infant Sleep in the Neonatal Intensive Care Unit. Pediatrics. 2019;144(3):e30190288


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Optimizing Resources in Childrens Surgical Care: An Update on the American College of Surgeons' Verification Program

Surgical procedures are performed in the United States in a wide variety of clinical settings and with variation in clinical outcomes. In May 2012, the Task Force for Children’s Surgical Care, an ad hoc multidisciplinary group comprising physicians representing specialties relevant to pediatric perioperative care, was convened to generate recommendations to optimize the delivery of children’s surgical care. This group generated a white paper detailing the consensus opinions of the involved experts. Following these initial recommendations, the American College of Surgeons (ACS), Children’s Hospital Association, and Task Force for Children’s Surgical Care, with input from all related perioperative specialties, developed and published specific and detailed resource and quality standards designed to improve children’s surgical care (https://www.facs.org/quality-programs/childrens-surgery/childrens-surgery-verification). In 2015, with the endorsement of the American Academy of Pediatrics (https://pediatrics.aappublications.org/content/135/6/e1538), the ACS established a pilot verification program. In January 2017, after completion of the pilot program, the ACS Children’s Surgery Verification Quality Improvement Program was officially launched. Verified sites are listed on the program Web site at https://www.facs.org/quality-programs/childrens-surgery/childrens-surgery-verification/centers, and more than 150 are interested in verification. This report provides an update on the ACS Children’s Surgery Verification Quality Improvement Program as it continues to evolve.


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Neurology: Clinical Practice


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Unusual and important cause of acute neck pain: Longus colli calcific tendinitis

Early recognition of longus colli calcific tendinitis can prevent unnecessary interventions including antibiotics and surgical procedures.


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Serial 18F-FDG PET/CT findings in a patient with neurocutaneous melanosis

With high sensitivity in detecting acute brain events such as seizures, FDG PET can be used as an important tool for neurocutaneous melanosis disease monitoring.


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Progressive supranuclear palsy and pawpaw

Consider consumption of annonacin-containing plant products, including pawpaw, as a possible environmental risk factor for atypical parkinsonism.


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Issue Highlights

Wardrope et al. (p. 96) reported on the feasibility of using an artificial intelligence tool to distinguish reliably between syncope, epilepsy, and psychogenic nonepileptic seizures in patients presenting with transient loss of consciousness. In an accompanying editorial, Cormac O'Donovan (p. 94) observes that "the likelihood of a one-size-fits-all diagnostic method is unlikely but should not deter the very important research in this field that needs to be performed."


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Reader response: Symptom burden among individuals with Parkinson disease: A national survey

We read with great interest the study by Tarolli et al.,1 which explored the burden of disease in Parkinson disease (PD) by evaluating the prevalence of nonmotor symptoms and their association with quality of life. The authors selected nonmotor symptoms based on literature review, expert opinions, and patient interviews. We note that apathy, which has major consequences for patients and carers, was not included as a relevant nonmotor symptom in their study. We performed a subcohort analysis of 60 patients from a study of pain in PD in 110 outpatients (PaCoMo-study, registered trial number: NL6311402917 [toetsingonline.nl]). We retrospectively reviewed the medical records to check whether the clinician identified apathy in these patients in the previous year, which was the case in 15% of the patients (n = 9). Blind to those results, patients were examined with the Apathy Scale (AS).2 In total, 63.3% (n = 38) of the patients scored positive on the AS. Only 18.4% of the patients who scored positive on the AS were also classified or mentioned with apathy in the medical records by clinicians.


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Author response: Symptom burden among individuals with Parkinson disease: A national survey

We appreciate the readers' comments on the prevalence and impact of apathy on quality of life among individuals with Parkinson disease. In constructing our survey instrument, we discussed the inclusion of apathy as a symptom. However, we ultimately opted against inclusion because of concerns about the specificity of terminology in our online survey. Patients and care partners may not be familiar with the term "apathy,"and near-synonyms such as "reduced motivation" have substantial overlap with other nonmotor features. Still, as the readers point out, apathy is extremely common and under-recognized. Similar to many of the nonmotor symptoms identified in our study,1 we agree that clinicians should be screening for apathy among those with Parkinson disease.


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Reader response: Functional neurologic disorders: Bringing the informal and hidden curriculum to light

I read with interest the editorial by Strom1 about functional neurologic disorders (FNDs). As a treating physician, I have struggled with the multiple diagnostic labels attached to these patients by physicians of different medical specialties during the course of their clinical disease presentation. A neurologist may assign a patient who presents with chronic fatigue the diagnostic labels of narcolepsy, idiopathic hypersomnia, or chronic Lyme disease. A rheumatologist may assign the label of collagen vascular disease, and a psychiatrist may diagnose depression. This diagnostic ambiguity is troublesome for patients and clinicians alike. I contend that even the term FND needs to be revisited. A patient should be broadly labeled as having a functional disorder and only after characterization sublabeled and referred to an appropriate specialty physician.


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Author response: Functional neurologic disorders: Bringing the informal and hidden curriculum to light

Dr. Sethi raises an excellent point about the term functional neurologic disorder (FND) in his comment on the editorial.1 It seems clear that reticence to use the term functional creates the ambiguity he mentions. Medically unexplained symptoms, categorized in the international classification of diseases as undifferentiated somatoform disorders, are a diagnosis that many providers are loathed to give. Whether that is because of concern about missing a diagnosis is not clear. Having evaluated and treated more than 400 of these individuals in the FND clinic at the University of Colorado, I can attest to the fact that patients arrive confused about their diagnosis. Multiple incorrect diagnoses, as Dr. Sethi points out, pack the medical histories of patients with FND, leading doctors and patients astray. I believe that the commentary by Perez et al.2 gives us the best chance for a way forward, by teaching a new generation of residents and fellows how to approach patients in a nonjudgmental and open-minded fashion. It took 30 years to add Functional Neurologic Disorder to the Diagnostic and Statistical Manual, and it is still parenthetical to the term Conversion.3 Stripping the diagnosis of FND of its stigma and empowering care providers to rule in functional disorders is an actionable step which should be taken.


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Neurology and baseball: Who wants to win?

Teams fail when they cannot achieve a common goal. They also fail when they do not have one. In baseball, different goals between players and staff are unusual. Everyone wants to win. In neurology, where teams may be loosely defined and comprise people from many disciplines, goals differ. A win for you may not be a win for me.


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Diagnosing spells: Machines or humans?

In the current era of technological advances in medicine, public interest seems focused on advances in laboratory testing, imaging, and surgical instrumentation. Modern-day expectations in the diagnostic part of medicine appear to demand answers which are instant, specific, and without ambiguity. This is consistent with the idea of developing the science of medicine. However, many still consider obtaining a diagnosis through a careful and thoughtful history as an example of the art of medicine.


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Machine learning as a diagnostic decision aid for patients with transient loss of consciousness

Background

Transient loss of consciousness (TLOC) is a common reason for presentation to primary/emergency care; over 90% are because of epilepsy, syncope, or psychogenic non-epileptic seizures (PNES). Misdiagnoses are common, and there are currently no validated decision rules to aid diagnosis and management. We seek to explore the utility of machine-learning techniques to develop a short diagnostic instrument by extracting features with optimal discriminatory values from responses to detailed questionnaires about TLOC manifestations and comorbidities (86 questions to patients, 31 to TLOC witnesses).

Methods

Multi-center retrospective self- and witness-report questionnaire study in secondary care settings. Feature selection was performed by an iterative algorithm based on random forest analysis. Data were randomly divided in a 2:1 ratio into training and validation sets (163:86 for all data; 208:92 for analysis excluding witness reports).

Results

Three hundred patients with proven diagnoses (100 each: epilepsy, syncope and PNES) were recruited from epilepsy and syncope services. Two hundred forty-nine completed patient and witness questionnaires: 86 epilepsy (64 female), 84 PNES (61 female), and 79 syncope (59 female). Responses to 36 questions optimally predicted diagnoses. A classifier trained on these features classified 74/86 (86.0% [95% confidence interval 76.9%–92.6%]) of patients correctly in validation (100 [86.7%–100%] syncope, 85.7 [67.3%–96.0%] epilepsy, 75.0 [56.6%–88.5%] PNES). Excluding witness reports, 34 features provided optimal prediction (classifier accuracy of 72/92 [78.3 (68.4%–86.2%)] in validation, 83.8 [68.0%–93.8%] syncope, 81.5 [61.9%–93.7%] epilepsy, 67.9 [47.7%–84.1%] PNES).

Conclusions

A tool based on patient symptoms/comorbidities and witness reports separates well between syncope and other common causes of TLOC. It can help to differentiate epilepsy and PNES. Validated decision rules may improve diagnostic processes and reduce misdiagnosis rates.

Classification of evidence

This study provides Class III evidence that for patients with TLOC, patient and witness questionnaires discriminate between syncope, epilepsy and PNES.


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Risk of stroke after emergency department visits for neurologic complaints

Objective

To assess the risk of subsequent stroke among older patients discharged from an emergency department (ED) without a diagnosis of TIA or stroke.

Methods

Using electronic health record data from a large urban, university hospital and a community-based hospital, we analyzed patients aged 60–89 years discharged to home from the ED without an International Statistical Classification of Diseases and Related Health Problems, 9th or 10th Revision diagnosis of TIA or stroke. Based on the presence/absence of a head CT and the presence/absence of a chief complaint suggestive of TIA or stroke ("symptoms") during the index ED visit, we created 4 mutually exclusive groups (group 1, reference: head CT no, symptoms no; group 2: head CT no, symptoms yes; group 3: head CT yes, symptoms no; and group 4: head CT yes, symptoms yes). We calculated rates of stroke in the 30, 90, and 365 days after the index visit and used multivariable logistic regression to estimate odds ratios (ORs) for subsequent stroke.

Results

Among 35,622 patients (mean age 70 years, 59% women, and 16% African American), unadjusted rates of stroke in 365 days were as follows: group 4: 2.5%; group 3: 1.1%; group 2: 0.69%; and group 1: 0.54%. The adjusted OR for stroke was 3.30 (95% confidence interval [CI], 1.61–6.76) in group 4, 1.56 (95% CI, 1.16–2.09) in group 3, and 0.61 (95% CI, 0.22–1.67) in group 2.

Conclusions

Among patients discharged from the ED without a diagnosis of TIA or stroke, the occurrence of a head CT and/or specific neurologic symptoms established a clinically meaningful risk gradient for subsequent stroke.


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Cerebral venous thrombosis: Associations between disease severity and cardiac markers

Background

Plasma cardiac troponin (cTn) elevation occurs in acute ischemic stroke and intracranial hemorrhage and can suggest a poor prognosis. Because acute cerebral venous thrombosis (CVT) might lead to venous stasis, which could result in cardiac stress, it is important to evaluate whether cTn elevation occurs in patients with CVT.

Methods

Inpatients at Johns Hopkins Hospital from 2005 to 2015 meeting the following criteria were included: CVT (ICD-9 codes with radiologic confirmation) and available admission electrocardiogram (ECG) and cTn level. In regression models, presence of ECG abnormalities and cTn elevation (>0.06 ng/mL) were evaluated as dependent variables in separate models, with location and severity of CVT involvement as independent variables, adjusted for age, sex, and hypertension.

Results

Of 81 patients with CVST, 53 (66%) met the inclusion criteria. Participants were, on average, aged 42 years, white (71%), and female (66%). The left transverse sinus was most commonly thrombosed (47%), with 66% having >2 veins thrombosed. Twenty-two (41%) had cTn elevation. Odds of cTn elevation increased per each additional vein thrombosed (adjusted OR 2.79, 95% CI [1.08–7.23]). Of those with deep venous involvement, 37.5% had cTn elevation compared with 4.4% without deep clots (p = 0.02). Venous infarction (n = 15) was associated with a higher mean cTn (0.14 vs 0.02 ng/mL, p = 0.009) and was predictive of a higher cTn in adjusted models (β = 0.15, 95% CI [0.06–0.25]).

Conclusions

In this single-center cohort study, markers of CVT severity were associated with increased odds of cTn elevation; further investigation is needed to elucidate causality and significance.


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Factors associated with 30-day readmission for patients hospitalized for seizures

Background

We sought to determine the cumulative incidence of readmissions after a seizure-related hospitalization and identify risk factors and readmission diagnoses.

Methods

We performed a retrospective cohort study of adult patients hospitalized with a primary discharge diagnosis of seizure (International Classification of Diseases, Ninth Edition, Clinical Modification codes 345.xx and 780.3x) using the State Inpatient Databases across 11 states from 2009 to 2012. Hospital and community characteristics were obtained from the American Hospital Association and Robert Wood Johnson Foundation. We performed logistic regressions to explore effects of patient, hospital, and community factors on readmissions within 30 days of discharge.

Results

Of 98,712 patients, 13,929 (14%) were readmitted within 30 days. Reasons for readmission included epilepsy/convulsions (30% of readmitted patients), mood disorders (5%), schizophrenia (4%), and septicemia (4%). The strongest predictors of readmission were diagnoses of CNS tumor (odds ratio [OR] 2.1, 95% confidence interval [CI] 1.9–2.4) or psychosis (OR 1.8, 95% CI 1.7–1.8), urgent index admission (OR 2.0, 95% CI 1.8–2.2), transfer to nonacute facilities (OR 1.7, 95% CI 1.6–1.8), long length of stay (OR 1.7, 95% CI 1.6–1.8), and for-profit hospitals (OR 1.7, 95% CI 1.6–1.8). Our main model's c-statistic was 0.66. Predictors of readmission for status epilepticus included index admission for status epilepticus (OR 3.5, 95% CI 2.6–4.7), low hospital epilepsy volume (OR 0.4, 95% CI 0.3–0.7), and rural hospitals (OR 4.8, 95% CI 2.1–10.9).

Conclusion

Readmission is common after hospitalization for seizures. Prevention strategies should focus on recurrent seizures, the most common readmission diagnosis. Many factors were associated with readmission, although readmissions remain challenging to predict.


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Ataxic-hypotonic cerebral palsy in a cerebral palsy registry: Insights into a distinct subtype

Objective

To specifically report on ataxic-hypotonic cerebral palsy (CP) using registry data and to directly compare its features with other CP subtypes.

Methods

Data on prenatal, perinatal, and neonatal characteristics and gross motor function (Gross Motor Function Classification System [GMFCS]) and comorbidities in 35 children with ataxic-hypotonic CP were extracted from the Canadian Cerebral Palsy Registry and compared with 1,804 patients with other subtypes of CP.

Results

Perinatal adversity was detected significantly more frequently in other subtypes of CP (odds ratio [OR] 4.3, 95% confidence interval [CI] 1.5–11.7). The gestational age at birth was higher in ataxic-hypotonic CP (median 39.0 weeks vs 37.0 weeks, p = 0.027). Children with ataxic-hypotonic CP displayed more intrauterine growth restriction (OR 2.6, 95% CI 1.0–6.8) and congenital malformation (OR 2.4, 95% CI 1.2–4.8). MRI was more likely to be either normal (OR 3.8, 95% CI 1.4–10.5) or to show a cerebral malformation (OR 4.2, 95% CI 1.5–11.9) in ataxic-hypotonic CP. There was no significant difference in terms of GMFCS or the presence of comorbidities, except for more frequent communication impairment in ataxic-hypotonic CP (OR 4.2, 95% CI 1.5–11.6).

Conclusions

Our results suggest a predominantly genetic or prenatal etiology for ataxic-hypotonic CP and imply that a diagnosis of ataxic-hypotonic CP does not impart a worse prognosis with respect to comorbidities or functional impairment. This study contributes toward a better understanding of ataxic-hypotonic CP as a distinct nosologic entity within the spectrum of CP with its own pathogenesis, risk factors, clinical profile, and prognosis compared with other CP subtypes.


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Worldwide survey of neurologists on approach to autoimmune encephalitis

Objective

To explore practice differences in the diagnosis and management of autoimmune encephalitis (AE), which is complicated by issues with sensitivity/specificity of antibody testing, nonspecific MRI/EEG/CSF findings, and competing differential diagnoses.

Methods

We used a worldwide electronic survey with practice-related demographic questions and clinical questions about 2 cases: (1) a 20-year-old woman with a neuropsychiatric presentation strongly suspicious of AE and (2) a 40-year-old man with new temporal lobe seizures and cognitive impairment. Responses among different groups were compared using multivariable logistic regression.

Results

We received 1,333 responses from 94 countries; 12.0% identified as neuroimmunologists. Case 1: those treating >5 AE cases per year were more likely to send antibodies in both serum and CSF (adjusted odds ratio [aOR] vs 0 per year: 3.29, 95% CI 1.31–8.28, p = 0.011), pursue empiric immunotherapy (aOR: 2.42, 95% CI 1.33–4.40, p = 0.004), and continue immunotherapy despite no response and negative antibodies at 2 weeks (aOR: 1.65, 95% CI 1.02–2.69, p = 0.043). Case 2: neuroimmunologists were more likely to send antibodies in both serum and CSF (aOR: 1.80, 95% CI 1.12–2.90, p = 0.015). Those seeing >5 AE cases per year (aOR: 1.86, 95% CI 1.22–2.86, p = 0.004) were more likely to start immunotherapy without waiting for antibody results.

Conclusions

Our results highlight the heterogeneous management of AE. Neuroimmunologists and those treating more AE cases generally take a more proactive approach to testing and immunotherapy than peers. Results highlight the need for higher-quality cohorts and trials to guide empiric immunotherapy, and evidence-based guidelines aimed at both experts and nonexperts. Because the average AE patient is unlikely to be first seen by a neuroimmunologist, ensuring greater uniformity in our approach to suspected cases is essential to ensure that patients are appropriately managed.


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Neurology consults in emergency departments: Opportunities to streamline care

Objective

To use the variations in neurology consultations requested by emergency department (ED) physicians to identify opportunities to implement multidisciplinary interventions in an effort to reduce ED overcrowding.

Methods

We retrospectively analyzed ED visits across 3 urban hospitals to determine the top 10 most common chief complaints leading to neurology consultation. For each complaint, we evaluated the likelihood of consultation, admission rate, admitting services, and provider-to-provider variability of consultation.

Results

Of 145,331 ED encounters analyzed, 3,087 (2.2%) involved a neurology consult, most commonly with chief complaints of acute-onset neurologic deficit, subacute neurologic deficit, or altered mental status. ED providers varied most in their consultation for acute-onset neurologic deficit, dizziness, and headache. Neurology consultation was associated with a 2.3-hour-longer length of stay (LOS) (95% CI: 1.6–3.1). Headache in particular has an average of 6.7-hour-longer ED LOS associated with consultation, followed by weakness or extremity weakness (4.4 hours) and numbness (4.1 hours). The largest estimated cumulative difference (number of patients with the specific consultation multiplied by estimated difference in LOS) belongs to headache, altered mental status, and seizures.

Conclusion

A systematic approach to identify variability in neurology consultation utilization and its effect on ED LOS helps pinpoint the conditions most likely to benefit from protocolized pathways.


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