Acute chest syndrome (ACS) is defined as a new pulmonary infiltrate detected by chest radiography (CXR) associated with fever, respiratory symptoms, or chest pain [1,2]. It is the second most common cause of hospital admissions in SCD, and can lead to significant morbidity and mortality [2]. Evidence in the literature has established that clinical assessment alone is not a sensitive predictor of ACS [1,2]. Although these studies were performed nearly 20 years ago, they advocate for empiric CXR utilization all febrile children with SCD [1,2]. Since then, the incidence of ACS in febrile children has decreased from 27 to 17.4% (P < 0.001) [3], likely due in part to routine penicillin prophylaxis, expanded pneumococcal immunization coverage, and hydroxyurea use. National Institutes of Health (NIH) guidelines for febrile SCD patients were updated in 2014 recommending that patients with fever associated with shortness of breath, tachypnea, cough, and/or rales should undergo CXR to evaluate for ACS [4]. No systemic review of the literature was done regarding these SCD/fever guidelines, which utilized panel consensus expert opinion. Since there are discrepancies between the current NIH guidelines, many institutional clinical pathway recommendations and evidence from the literature, this study was undertaken to help identify febrile children with SCD requiring a CXR as part of their assessment.2013 emergency department (ED) data were obtained by electronic medical record using ICD-9 codes linked to fever and SCD in children 2 months-18 years through a retrospective chart review at two urban tertiary-care campuses of Children's Healthcare of Atlanta (CHOA). Demographics, past medical history, vital signs, review of systems (ROS), physical exam (PE) findings, CXR radiology interpretation, and ACS diagnosis were obtained by chart review; see "Supporting Information Methods" for details. Children with Negative Vs. Positive CXR (ACS). Supporting Information Table II summarizes demographics, clinical characteristics and risk factors for patients with ACS (n 5 66) compared to those with negative CXRs. Of encounters evaluated by CXR, the prevalence of ACS was 17.2% in our cohort. Risk factors for ACS as determined by univariate analysis include the presence of tachypnea, history of ACS, and abnormal ROS (presence of cough, wheeze, or chest pain). An abnormal lung exam was also more frequently found in children diagnosed with ACS. However, 62% of patients with ACS had a normal lung exam. Patients with ACS were also more frequently admitted to the hospital. Of all admitted patients, those with ACS more frequently utilized oxygen and BiPAP. However, there was no difference in age, gender, height of fever, asthma diagnosis, presence of rhinorrhea, congestion, grunting, or shortness of breath in children diagnosed with ACS compared to those with a negative CXR. Clinical characteristics involving patients discharged from the ED and <72-h ED returns are summarized in Supporting Information Methods/Results. One patient who was discharge...
Background: Acute chest syndrome (ACS) is a common complication of sickle cell disease (SCD) detected by chest radiography (CXR) with significant morbidity and mortality. Previous studies show that many children with ACS present without obvious signs or symptoms. We evaluate the sensitivity of clinical assessment for predicting ACS in children with SCD and fever. Methods: This is a prospective cross-sectional, observational study that took place from June 2014-March 2015. Consented providers in 2 CHOA EDs caring for patients aged 0-18 years with SCD and fever 38.0 C completed a questionnaire including vital signs, past history of ACS or asthma, clinical signs, symptoms, physical exam (PE) findings and whether the provider suspected pneumonia/ACS on CXR. CXRs were read by a blinded pediatric radiologist. CXR results were compared to questionnaire responses completed prior to CXR. Sensitivity, specificity, positive and negative predictive value and accuracy of clinical assessment for predicting ACS compared to CXR was determined. Signs, symptoms, PE findings, and clinical course was compared in cases with positive versus negative CXRs as well as suspected versus unsuspected ACS. Results: 111 SCD fever events with CXR were evaluated over 10 months. Thirteen patients (12%) had evidence of ACS on CXR. Based on MD questionnaires, only 46% of ACS cases were predicted by clinical assessment alone. Physician assessment sensitivity to identify ACS was low at only 50%. (Table 1). An abnormal lung exam, history of ACS, lack of rhinorrhea and a trend towards chest pain were more common in ACS cases compared to children with a negative CXR. However, 62% of children with ACS had a normal lung exam. Cases of unsuspected ACS had a lower degree of fever at presentation to the ED, but ultimately required more packed red blood cell transfusions during hospitalization than suspected ACS cases. There were no other significant differences in signs, symptoms, PE findings or clinical course in suspected vs. unsuspected ACS cases. The recently published NIH guidelines for including CXR in a fever work-up for children with SCD (risks include presence of cough, tachypnea, shortness of breath or abnormal lung exam) performed poorly in our cohort, with an Area under the Curve (AUC) = 0.725 Conclusions: Clinical impression alone is a poor predictor of ACS in children with SCD and fever. We and others have identified clinical criteria to help identify a high risk group, however many children with ACS lack signs and symptoms of ACS and may be missed based on clinical assessment alone. Given the high mortality and morbidity associated with ACS, empiric CXR may still be indicated in children with SCD. Due to radiation exposure risks with CXR, our team is studying lung ultrasound as an alternative modality for ACS imaging. Disclosures No relevant conflicts of interest to declare.
Background: Acute chest syndrome (ACS) is a common complication in patients with sickle cell disease (SCD) and is a leading cause of morbidity and mortality. Physical assessment alone is not sensitive for diagnosing ACS and therefore Chest x-ray (CXR) is recommended because of the difficulty of diagnosing ACS on clinical grounds alone. Children with SCD are repeatedly exposed to diagnostic radiation for the evaluation of ACS. Focused chest ultrasound (US) has been used to evaluate for lung consolidation. If lung US can identify patients with ACS this application could potentially limit radiation exposure in patients with SCD at risk for ACS. We evaluated the utility of physician performed US as compared to CXR to identify patients with SCD who have ACS. Methods: This is a prospective observational study that took place from November 2014-July 2015 in 2 urban pediatric emergency departments (EDs). The study population consisted of a convenience sample of patients with SCD from birth to 18 years of age at risk for ACS and who received a CXR for suspected ACS. Medical students and clinicians with training in lung sonography consented patients and performed a focused study to evaluate for lung consolidation. A blinded expert in point-of-care US reviewed for quality assurance and agreement. Sensitivity, specificity, and likelihood ratios were calculated for test performance characteristics of ultrasound using CXR as a reference standard. Inter-observer agreement (κ) between enrolling sonologists and reviewer was also calculated. Results: 85 patients were enrolled for a total of 98 cases. Median age was 7 years (IQR 2-13 years) and 53% of patients were male. The prevalence of ACS by CXR was 14%. Lung US was able to detect consolidation with a sensitivity of 86% (95% CI, 56-97%), specificity of 95% (95% CI, 87%-98%), positive likelihood ratio (LR) 18 (95% CI, 7-48) and negative LR 0.2 (95% CI, 0.04-0.5). The agreement between enrolling novice sonologists' interpretation and blinded reviewer's interpretation was very good with a Cohen κ of 0.86 (95% CI, 0.7-1). Conclusions: Focused lung US was able to identify ACS with high specificity. There was very good agreement between novice and expert sonologist interpretation. Lung US may decrease the need for CXR in patients at risk for ACS. Further studies are needed to see how this test performs within current clinical practice guidelines. Disclosures No relevant conflicts of interest to declare.
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