Transition of care in CHD: a single-centre experience: an enigma remains.

Transition of care refers to the continuity of health care during the movement from one healthcare setting to another as care needs change during a chronic illness. We sought to describe social, demographic, and clinical factors related to successful transition in a tertiary urban care facility in patients with CHD. Patients were identified utilising the electronic medical record. Inclusion criteria were patients with CHDs aged ≥15 years seen in the paediatric cardiology clinic between 2013 and 2014. Deceased patients were excluded. Clinical and demographic variables were collected. Patient charts were reviewed in 2015-2021 to determine if included patients were a) still in paediatric cardiology care, b) transitioned to adult cardiology/adult CHD, or were c) lost to follow-up. A total of 322 patients, 53% male (N:172), 46% female (N:149) were included. Majority had moderately complex lesions (N:132, 41%). Most patients had public insurance (N:172, 53%), followed by private insurance (N:67, 21%), while 15% of patients (N:47) were uninsured. Only 49% (N = 159) had successful transition, while 22% (N = 70) continued in care with paediatric cardiology, and 29% (N = 93) were lost to follow-up. Severity of CHD (p = 0.0002), having healthcare insurance (p < .0001), presence of a defibrillator (p = 0.0028), and frequency of paediatric cardiology visits (p = 0.0005) were significantly associated with successful transition. Most patients lost to follow-up (N:42,62%) were either uninsured or had public insurance. Lack of successful transition is multifactorial, and further efforts are needed to improve the process in patients with CHD.

Determining an infectious or autoimmune etiology in encephalitis.

OBJECTIVES: Early presentation and workup for acute infectious (IE) and autoimmune encephalitis (AE) are similar. This study aims to identify routine laboratory markers at presentation that are associated with IE or AE. METHODS: This was a multi-center retrospective study at three tertiary care hospitals in New York City analyzing demographic and clinical data from patients diagnosed with definitive encephalitis based on a confirmed pathogen and/or autoantibody and established criteria for clinical syndromes. RESULTS: Three hundred and thirty-three individuals with confirmed acute meningoencephalitis were included. An infectious-nonbacterial (NB) pathogen was identified in 151/333 (45.40%), bacterial pathogen in 95/333 (28.50%), and autoantibody in 87/333 (26.10%). NB encephalitis was differentiated from AE by the presence of fever (NB 62.25%, AE 24.10%; p < 0.001), higher CSF white blood cell (WBC) (median 78 cells/µL, 8.00 cells/µL; p < 0.001), higher CSF protein (76.50 mg/dL, 40.90 mg/dL; p < 0.001), lower CSF glucose (58.00 mg/dL, 69.00 mg/dL; p < 0.001), lower serum WBC (7.80 cells/µL, 9.72 cells/µL; p < 0.050), higher erythrocyte sedimentation rate (19.50 mm/HR, 13.00 mm/HR; p < 0.05), higher C-reactive protein (6.40 mg/L, 1.25 mg/L; p = 0.005), and lack of antinuclear antibody titers (>1:40; NB 11.54%, AE 32.73%; p < 0.001). CSF-to-serum WBC ratio was significantly higher in NB compared to AE (NB 11.3, AE 0.99; p < 0.001). From these findings, the association of presenting with fever, CSF WBC ≥50 cells/µL, and CSF protein ≥75 mg/dL was explored in ruling-out AE. When all three criteria are present, an AE was found to be highly unlikely (sensitivity 92%, specificity 75%, negative predictive value 95%, and positive predictive value 64%). INTERPRETATIONS: Specific paraclinical data at initial presentation may risk stratify which patients have an IE versus AE.

Selumetinib in children with neurofibromatosis type 1 and asymptomatic inoperable plexiform neurofibroma at risk for developing tumor-related morbidity.

BACKGROUND: Selumetinib was recently approved for the treatment of inoperable symptomatic plexiform neurofibromas (PNs) in children with neurofibromatosis type 1 (NF1). This parallel phase II study determined the response rate to selumetinib in children with NF1 PN without clinically significant morbidity. METHODS: Children with NF1 and inoperable PNs, which were not yet causing clinically significant morbidity but had the potential to cause symptoms, received selumetinib at 25 mg/m2 orally twice daily (1 cycle = 28 days). Volumetric magnetic resonance imaging analysis and outcome assessments, including patient-reported (PRO), observer-reported, and functional outcome measures were performed every 4 cycles for 2 years, with changes assessed over time. A confirmed partial response (cPR) was defined as PN volume decrease of ≥20% on at least 2 consecutive scans ≥3 months apart. RESULTS: 72% of subjects experienced a cPR on selumetinib. Participants received selumetinib for a median of 41 cycles (min 2, max 67) at data cutoff. Approximately half of the children rated having some target tumor pain at baseline, which significantly decreased by pre-cycle 13. Most objectively measured baseline functions, including visual, motor, bowel/bladder, or airway function were within normal limits and did not clinically or statistically worsen during treatment. CONCLUSIONS: Selumetinib resulted in PN shrinkage in most subjects with NF1 PN without clinically significant morbidity. No new PN-related symptoms developed while on selumetinib, and PRO measures indicated declines in tumor-related pain intensity. This supports that selumetinib treatment may prevent the development of PN-related morbidities, though future prospective studies are needed to confirm these results. CLINICAL TRIAL REGISTRATION: ClinicalTrials.gov NCT01362803.

Delays in Diagnosis and Treatment of Bacterial Meningitis in NYC: Retrospective Cohort Analysis.

Community-acquired bacterial meningitis (CABM) morbidity and mortality remains high in those infected. Rapid diagnosis and treatment is paramount to reducing mortality and improving outcome. This retrospective cohort study aims to assess the time from presentation to diagnosis and treatment of vaccine preventable CABM as well as identify possible factors associated with delays in diagnosis and antibiotic administration. A retrospective chart review was conducted of individuals who presented to Columbia University Irving Medical Center (CUIMC), Children's Hospital of New York (CHONY), Mount Sinai Medical Center, and Weill Cornell Medical Center with BM due to Haemophilus influenzae type B, Streptococcus pneumoniae, and Neisseria meningitidis between January 1, 2012 and December 31, 2017. Diagnosis was delayed by more than 8 hours in 13 patients (36.1%) and 5 individuals (13.9%) had a delay of 4 hours or more from presentation to the administration of antibiotics with appropriate CNS coverage. All of these patients were also initially misdiagnosed at an outpatient clinic, outside hospital, or emergency department. This retrospective study identified febrile and/or viral infections not otherwise specified and otitis media as the most common misdiagnoses underlying delays from presentation to diagnosis and to antibiotic treatment in those with BM.

Definition and time course of pericavity edema after minimally invasive endoscopic intracerebral hemorrhage evacuation.

BACKGROUND: Perihematomal edema (PHE) volume correlates with intracerebral hemorrhage (ICH) volume and is associated with functional outcome. Minimally invasive surgery (MIS) for ICH decreases clot burden and PHE. MIS may therefore alter the time course of PHE, mitigating a critical source of secondary injury. OBJECTIVE: To describe a new method for the quantitative measurement of cerebral edema surrounding the evacuated hematoma cavity, termed pericavity edema (PCE), and obtain details of its time course following MIS for ICH. METHODS: The study included 48 consecutive patients presenting with ICH who underwent MIS evacuation. Preoperative and postoperative CT scans were assessed by two independent raters. Hematoma, edema, cavity, and pneumocephalus volumes were calculated using semi-automatic, threshold-guided volume segmentation software (AnalyzePro). Follow-up CT scans at variable delayed time points were available for 36 patients and were used to describe the time course of PCE. RESULTS: Mean preoperative, postoperative, and delayed PCE were 21.0 mL (SD 15.5), 18.6 mL (SD 11.4), and 18.4 mL (SD 15.5), respectively. The percentage of ICH evacuated correlated significantly with a decrease in postoperative PCE (r=-0.46, p<0.01). Linear regression analysis revealed a significant relation between preoperative hematoma volume and both postoperative PCE (p<0.001) and postoperative relative PCE (p<0.001). The mean peak PCE was 26.4 mL (SD 15.6) and occurred at 6.5 days (SD 4.8) post-ictus. The 2-week postoperative time course of relative PCE did not fluctuate, suggesting stability in edema during the perioperative period surrounding evacuation and up to 2 weeks after the initial bleed. CONCLUSIONS: We present a detailed and accurate method for measuring PCE volume with semi-automatic, threshold-guided segmentation software in the postoperative patient with ICH. Decrease in PCE after MIS evacuation correlated with evacuation percentage, and relative PCE remained stable after minimally invasive endoscopic ICH evacuation.

Reward magnitude enhances early attentional processing of auditory stimuli.

Reward associations are known to shape the brain's processing of visual stimuli, but relatively less is known about how reward associations impact the processing of auditory stimuli. We leveraged the high-temporal resolution of electroencephalography (EEG) and event-related potentials (ERPs) to investigate the influence of low- and high-magnitude stimulus-reward associations in an auditory oddball task. We associated fast, correct detection of certain auditory target stimuli with larger monetary rewards, and other auditory targets with smaller rewards. We found enhanced attentional processing of the more highly rewarded target stimuli, as evidenced by faster behavioral detection of those stimuli compared with lower-rewarded stimuli. Neurally, higher-reward associations enhanced the early sensory processing of auditory targets. Targets associated with higher-magnitude rewards had higher amplitude N1 and mismatch negativity (MMN) ERP components than targets associated with lower-magnitude rewards. Reward did not impact the latency of these early components. Higher-reward magnitude also decreased the latency and increased the amplitude of the longer-latency P3 component, suggesting that reward also can enhance the final processing stages of auditory target stimuli. These results provide insight into how the sensory and attentional neural processing of auditory stimuli is modulated by stimulus-reward associations and the magnitude of those associations, with higher-magnitude reward associations yielding enhanced auditory processing at both early and late stages compared with lower-magnitude reward associations.

Fatigue in Survivors of Autoimmune Encephalitis.

BACKGROUND AND OBJECTIVES: To evaluate the impact of fatigue after autoimmune encephalitis, determine associations with patients' characteristics, and identify factors that contribute to its development. METHODS: In a first cohort recruited via several encephalitis support organizations, self-reported questionnaires were used to evaluate fatigue, depression, and sleep quality in adults after autoimmune encephalitis. In a second cohort where more in-depth clinical characterization could be performed, adults with encephalitis from 2 tertiary hospitals were evaluated using the same questionnaires. Patients' characteristics were retrospectively captured. RESULTS: In the first cohort (mean [SD] age; 43 [16] years, 220 [65%] female), 220 of 338 participants (65%) reported fatigue, 175 of 307 (57%) depression, and 211 of 285 (74%) poor sleep quality. In the second cohort (48 [19] years; 43 [50%] women), 42 of 69 participants (61%) reported fatigue, whereas 23 of 68 (34%) reported depression and 44 of 66 (67%) poor sleep quality, despite more than 80% having "good" modified Rankin scale (mRS) scores (0-2). Individuals with anti-NMDA receptor encephalitis reported lower fatigue scores than those with other autoimmune encephalitis types. In a multivariate analysis examining factors at discharge that might predict fatigue scores, only anti-NMDA receptor encephalitis was a (negative) predictor of fatigue and remained so when potential confounders were included. DISCUSSION: The impact of fatigue after autoimmune encephalitis is prominent and not fully accounted for by depression or sleep quality, nor adequately captured by mRS scores for disability. Fatigue is pervasive across autoimmune encephalitis, although lower scores are reported in anti-NMDA receptor encephalitis. Fatigue should be screened routinely, considered as an outcome measure in clinical trials, and further studied from a mechanistic standpoint.

Popliteal Entrapment Syndrome as a Cause of Chronic Lower Extremity Pain in a 16-Year Old.

Popliteal entrapment syndrome is an uncommon cause of intermittent claudication in young patients lacking atherosclerotic risk factors. ZS is a 16-year-old cisgender female with type 1 diabetes complicated by microalbuminuria, obesity (body mass index (BMI) = 45.86 kg/m²), and a history of perinatal stroke with residual right-sided hemiparesis, who presented with six months of worsening bilateral, exertional lower extremity pain. Common causes of chronic bilateral lower extremity pain include peripheral vascular disease and diabetic neuropathy. Less common etiologies include trauma, infection, or juvenile idiopathic arthritis. Given her risk factors, the patient's pain was initially managed as a diabetic neuropathy with pregabalin. Symptoms failed to improve, and she re-presented with positional coolness of the right lower extremity, diminished pulses of the bilateral lower extremities, and weakness in her toes. CT angiography demonstrated occlusion of the right distal superficial femoral and popliteal arteries and diffused tibial disease. Ultimately, the patient was discovered to have right-sided femoral-popliteal occlusion, and she required urgent femoral-tibial bypass. Despite an initial improvement in symptoms postoperatively, she continued to have lower extremity pain and recurrent arterial thrombi, even with antiplatelet and anticoagulation therapy. Eventually, the patient required a right-sided below the knee amputation. This case highlights the high index of suspicion that clinicians must have in young patients with lower extremity pain, both with and without atherosclerotic risk factors, as early intervention facilitates better outcomes. Introduction.

Factors contributing to underuse of epinephrine autoinjectors in pediatric patients with food allergy.

BACKGROUND: Epinephrine autoinjectors (EAs) are the standard of care for severe food allergic reactions, although they are frequently underused or misused. OBJECTIVE: To understand the factors associated with underuse of EA by caregivers of pediatric patients with food allergy. METHODS: A survey was administered to 200 caregivers of pediatric patients with food allergies to assess most severe lifetime allergic reaction, EA education, and use and factors associated with incorrect use or underutilization. RESULTS: A total of 164 surveys were completed; of which 118 (72%) of lifetime most severe reactions warranted EA use, but the EA was used in only 45 (38.1%). Reasons caregivers indicated for not administering the EA included the following: reactions did not seem severe enough; it was the patient's first allergic reaction; use of other medication; and fear of using EA. CONCLUSION: Multiple factors contribute to underuse of EA in the treatment of severe allergic reactions. Results from this study highlight the need for continuous EA education in caregivers of and pediatric patients with food allergies, using a multipronged approach targeting clear symptom recognition and alleviation of fear of EA use.