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Spontaneous epidural hematoma (EDH) is a rare sickle cell disease (SCD) complication. We report 3 pediatric cases with SCD and spontaneous EDH and 1 with subgaleal hematomas in the setting of vaso-occlusive crises and elaborate on their presentation and management. Through a scoping review, we identified 71 additional cases reported from 1970 to 2024 and highlighted notable features. We highlight skull infarction as a prognostic factor associated with 88% lower odds of death relative to patients without skull infarction (P<0.01). We clarify optimal management strategies like early and aggressive treatment of disseminated intravascular coagulation (DIC), as 57% of patients with DIC died such that DIC was associated with 7.56 times higher odds of death (P=0.01)., Competing Interests: The authors declare no conflict of interest., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Acute chest syndrome (ACS) is a life-threatening complication of sickle cell disease (SCD). The Prevent Acute Chest Syndrome checklist (PACScheck) was created to drive appropriate ordering of opioids, incentive spirometry (IS), intravenous fluids (IVF), evaluation of oxygen desaturation, and bronchodilator use., Objectives: Decrease the development of ACS by 5% in a hospitalized pediatric SCD population., Methods: A multidisciplinary team conducted a quality improvement (QI) project between April 2020 and August 2021 on an inpatient pediatric hematology unit. At-risk hospitalizations were patients with SCD who did not have ACS upon hospital admission. PACScheck was implemented and weekly run charts assessed documentation. Process control (p) charts, geometric control (g) charts, and chi-square tests assessed checklist process measures pre- and post-PACScheck. G chart assessed the number of encounters between ACS events., Results: A total of 483 at-risk hospitalizations were identified in the 12 months prior and 363 during the study period. A g chart demonstrated that fewer encounters developed ACS during PACScheck. A p chart demonstrated that IS documentation increased during PACScheck. A run chart of PACScheck documentation demonstrated a median of 100% documentation at least once per hospitalization during the last six months of the intervention., Conclusion: Development of ACS can be reduced by implementing a best-practices checklist (PACScheck) on an inpatient pediatric hematology unit with a multidisciplinary team., (© 2024 Wiley Periodicals LLC.)

Hemoglobinopathies in neonates constitute a group of disorders influenced by genetic mutations in the human globin genes. They are often broadly categorized into quantitative defects or qualitative defects, though they are not mutually exclusive. In quantitative defects, the mutation causes insufficient production of a normal globin chain, which can range from no production to mild deficiency. These are typically referred to as thalassemias. In qualitative defects, the structure of the hemoglobin is altered. The most common structural hemoglobinopathy is sickle cell disease. During fetal development, distinct globin chains are synthesized, which undergo a progressive switch to adult globin chains perinatally. This affects the timing of the clinical presentation of these disorders and thus, our ability to diagnose them. In this review, we focus on the epidemiology, genetic causes, clinical presentation, and general overview and management of common hemoglobin disorders that may be encountered in the neonatal period., (Copyright © 2024 by the American Academy of Pediatrics.)

Point-of-care ultrasound (POCUS) is a valuable tool in pediatric care, but formalized education on POCUS is not a standard part of pediatric residency. Our primary objective was to conduct a needs assessment to determine pediatric residents' attitudes toward ultrasound education. A Likert scale-based survey was distributed to residents at 48 pediatric residency programs. Respondents were asked to rate their agreement to statements about ultrasound education at their respective programs. Participants were also asked to rank ultrasound indications in order of importance. Ninety-seven survey responses were included in the final data set. Sixty percent of respondents had not performed any ultrasound scans during residency. Ninety-two percent of respondents indicated an interest in ultrasound training during residency and that they desired a formal POCUS curriculum. Eighty-one percent of residents indicated that having more ultrasound education would make their programs more attractive to applicants. Pediatric residents recognize the utility of POCUS and desire additional education during training., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: Acute chest syndrome (ACS) is an important cause of morbidity in sickle cell disease (SCD). A standardized tool for reporting chest radiographs in pediatric SCD patients did not previously exist., Objective: To analyze the interobserver agreement among pediatric radiologists' interpretations for pediatric ACS chest radiographs utilizing a standardized reporting tool. We also explored the association of radiographic findings with ACS complications., Methods: This was a retrospective cohort study of pediatric ACS admissions from a single institution in 2019. ICD-10 codes identified 127 ACS admissions. Two radiologists independently interpreted the chest radiographs utilizing a standardized reporting tool, a third radiologist adjudicated discrepancies, and κ analysis assessed interobserver agreement. Clinical outcomes were correlated with chest radiograph findings utilizing Pearsons' χ 2 , t tests, and Mann-Whitney U tests. Odds ratios (ORs) with 95% confidence intervals (CIs) were calculated., Results: Interobserver agreement was moderate to near-perfect across variables, with κ analysis showing near-perfect agreement for opacity reported in the right upper lobe (0.84), substantial agreement for right lower lobe (0.63), and vertebral bony changes (0.72), with moderate agreement for all other reported variables. On the initial chest radiograph, an opacity located in the left lower lobe (LLL) correlated with pediatric intensive care unit transfer (p = .03). Pleural effusion on the initial chest radiograph had a 3.98 OR (95% CI: 1.35-11.74) of requiring blood products and a 10.67 OR (95% CI: 3.62-31.39) for noninvasive ventilation., Conclusion: The standardized reporting tool showed moderate to near-perfect agreement between radiologists. LLL opacity, and pleural effusion were associated with increased risk of ACS complications., (© 2023 Wiley Periodicals LLC.)

Background: Degree of cerebrovascular stenosis in pediatric patients with sickle cell anemia (SCA) informs need for chronic transfusion therapy, which has significant risks. Flow artifact, intrinsic to magnetic resonance angiography (MRA), is dependent on technical parameters and can lead to overinterpretation of stenosis. The primary objective of this study was to document any change in stroke prevention therapy that could be attributed to the implementation of a standardized MRA scanning protocol for patients with SCA., Methods: A standardized MRA scanning protocol with an echo time of less than 5 ms was implemented at Montefiore Medical Center (MMC), NY in May 2016. Retrospective chart review identified 21 pediatric patients with SCA, with an MRA head both pre- and post-May 2016. Arterial stenosis on MRA, machine parameters, and treatment plans were compared pre- and post-implementation., Results: Ten of the 21 patients met inclusion criteria. Previously seen stenosis was re-classified to a lower degree in six of the 10 patients, leading to discontinuation of transfusions in five patients. No patients required escalation of therapy to chronic transfusions., Conclusion: Optimizing flow artifact by decreasing echo time to less than 5 ms can improve accurate interpretation of cerebrovascular disease, and ensure appropriate treatment plans are in place for stroke prevention. This is especially important for implementing "TCD With Transfusions Changing to Hydroxyurea (TWiTCH)" clinical trial results in the real-world setting., (© 2022 Wiley Periodicals LLC.)

Background: Strokes and silent cerebral infarcts (SCIs) lead to significant morbidity and mortality in children with sickle cell disease (SCD). Higher systolic blood pressures increase risk for stroke and SCIs; however, patients with SCD often have lower clinic blood pressures than the general population. Twenty-four-hour ambulatory blood pressure monitoring (ABPM) allows for more robust examination of blood pressures. This study evaluated associations between abnormal ABPM measurements with stroke and SCIs., Procedure: A cross-sectional study was performed. Children with SCD completed 24-hour ABPMs. Children with a documented magnetic resonance imaging (MRI) brain within a year of the ABPM were included in the analysis. Bivariate analyses were performed to identify associations between ABPM parameters with cerebrovascular outcomes., Results: Forty-two children with a median age of 13 years (10, 17) were included in the analysis. Seven (17%) had history of stroke and seven (17%) had SCIs. Nocturnal hypertension, elucidated via 24-hour ABPM, was noted in 25% of subjects. The presence of nocturnal hypertension was significantly higher in the SCI/stroke group (55% vs 12%, P = .01). Sensitivity analyses were performed during which stroke patients were removed from analysis. Nocturnal hypertension remained significantly associated with the presence of SCIs (P = .006)., Conclusions: This study reveals an association between nocturnal hypertension and a higher prevalence of SCI and stroke in children with SCD. Larger, prospective studies are needed to confirm these findings and evaluate the contributory nature of blood pressure abnormalities to cerebrovascular events in children with SCD., (© 2021 Wiley Periodicals LLC.)

New York City has emerged as one of the epicenters of the SARS-COV-2 pandemic, with the Bronx being disproportionately affected. This novel coronavirus has caused significant respiratory manifestations raising the concern for development of acute chest syndrome (ACS) in patients with sickle cell disease (SCD). We report a series of pediatric SCD SARS-COV-2-positive patients admitted with ACS. SARS-COV-2-positive SCD patients, who did not develop ACS, were the comparison group. Hydroxyurea use (P-value = .02) and lower absolute monocyte counts (P-value = .04) were noted in patients who did not develop ACS. These preliminary findings need to be further evaluated in larger cohorts., (© 2020 Wiley Periodicals LLC.)

Objective: Point-of-care (POC) urine dipstick is a highly used test in the pediatric emergency department (PED) owing to its fast turn-around time and inexpensive cost. Past studies have shown hand-held urine dipsticks and automated urinalysis in children younger than 48 months to be sensitive predictors for urinary tract infection (UTI). It is hypothesized that POC dip testing is as accurate as laboratory urinalysis in the diagnosis of UTI., Methods: A retrospective chart review was conducted on patients (aged birth through 18 years) presenting to a PED between January 2015 and December 2015. Eligible subjects included those that had a POC dip, laboratory urinalysis (lab UA), and urine culture performed during their PED visit. Subjects were selected, using a random number generator; 334 charts were selected. A positive POC dip was defined as having a positive leukocyte esterase or the presence of nitrites. A positive lab UA was defined as having a positive leukocyte esterase, nitrites, or greater than 10 white blood cells per high-power field. Urine culture was used as the criterion standard for comparison., Results: A total of 334 subjects' charts were reviewed. Sensitivity and specificity of the POC dip were 91.4% (95% confidence interval [CI], 76.9%-98.2%) and 63.9% (95% CI, 57.2%-69.3%); lab UA, 91.4% (95% CI, 76.9%-98.2%) and 63.9% (95% CI, 58.2%-69.3%); and lab dip, 88.6% (95% CI, 73.3%-96.8%) and 65.6% (95% CI, 59.9%-70.9%)., Conclusions: Point-of-care dips are as sensitive in detecting UTI as the lab UA. A prospective study could allow for further demographic evaluation of POC dip diagnosed UTI.

Competing Interests: POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of interest to disclose.