Which Febrile Children With Sickle Cell Disease Need a Chest X-Ray?

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Abstract

Controversy exists regarding which febrile children with sickle cell disease (SCD) should receive a chest x-ray (CXR). Our goal is to provide data informing the decision of which febrile children with SCD presenting to the emergency department (ED) require a CXR to evaluate for acute chest syndrome (ACS).

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Most cited references 19

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Causes and outcomes of the acute chest syndrome in sickle cell disease. National Acute Chest Syndrome Study Group.

E P Vichinsky, L Neumayr, A Earles … (2000)

The acute chest syndrome is the leading cause of death among patients with sickle cell disease. Since its cause is largely unknown, therapy is supportive. Pilot studies with improved diagnostic techniques suggest that infection and fat embolism are underdiagnosed in patients with the syndrome. In a 30-center study, we analyzed 671 episodes of the acute chest syndrome in 538 patients with sickle cell disease to determine the cause, outcome, and response to therapy. We evaluated a treatment protocol that included matched transfusions, bronchodilators, and bronchoscopy. Samples of blood and respiratory tract secretions were sent to central laboratories for antibody testing, culture, DNA testing, and histopathological analyses. Nearly half the patients were initially admitted for another reason, mainly pain. When the acute chest syndrome was diagnosed, patients had hypoxia, decreasing hemoglobin values, and progressive multilobar pneumonia. The mean length of hospitalization was 10.5 days. Thirteen percent of patients required mechanical ventilation, and 3 percent died. Patients who were 20 or more years of age had a more severe course than those who were younger. Neurologic events occurred in 11 percent of patients, among whom 46 percent had respiratory failure. Treatment with phenotypically matched transfusions improved oxygenation, with a 1 percent rate of alloimmunization. One fifth of the patients who were treated with bronchodilators had clinical improvement. Eighty-one percent of patients who required mechanical ventilation recovered. A specific cause of the acute chest syndrome was identified in 38 percent of all episodes and 70 percent of episodes with complete data. Among the specific causes were pulmonary fat embolism and 27 different infectious pathogens. Eighteen patients died, and the most common causes of death were pulmonary emboli and infectious bronchopneumonia. Infection was a contributing factor in 56 percent of the deaths. Among patients with sickle cell disease, the acute chest syndrome is commonly precipitated by fat embolism and infection, especially community-acquired pneumonia. Among older patients and those with neurologic symptoms, the syndrome often progresses to respiratory failure. Treatment with transfusions and bronchodilators improves oxygenation, and with aggressive treatment, most patients who have respiratory failure recover.

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Normal ranges of heart rate and respiratory rate in children from birth to 18 years of age: a systematic review of observational studies.

Susannah Fleming, Matthew Thompson, Richard Stevens … (2011)

Although heart rate and respiratory rate in children are measured routinely in acute settings, current reference ranges are not based on evidence. We aimed to derive new centile charts for these vital signs and to compare these centiles with existing international ranges. We searched Medline, Embase, CINAHL, and reference lists for studies that reported heart rate or respiratory rate of healthy children between birth and 18 years of age. We used non-parametric kernel regression to create centile charts for heart rate and respiratory rate in relation to age. We compared existing reference ranges with those derived from our centile charts. We identified 69 studies with heart rate data for 143,346 children and respiratory rate data for 3881 children. Our centile charts show decline in respiratory rate from birth to early adolescence, with the steepest fall apparent in infants under 2 years of age; decreasing from a median of 44 breaths per min at birth to 26 breaths per min at 2 years. Heart rate shows a small peak at age 1 month. Median heart rate increases from 127 beats per min at birth to a maximum of 145 beats per min at about 1 month, before decreasing to 113 beats per min by 2 years of age. Comparison of our centile charts with existing published reference ranges for heart rate and respiratory rate show striking disagreement, with limits from published ranges frequently exceeding the 99th and 1st centiles, or crossing the median. Our evidence-based centile charts for children from birth to 18 years should help clinicians to update clinical and resuscitation guidelines. National Institute for Health Research, Engineering and Physical Sciences Research Council. Copyright © 2011 Elsevier Ltd. All rights reserved.

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Acute care utilization and rehospitalizations for sickle cell disease.

M L Mosso, Julie A Panepinto, David C Brousseau … (2010)

Published rates of health care utilization and rehospitalization by people with sickle cell disease have had limited generalizability and are not population based. To provide benchmark data for rates of acute care utilization and rehospitalizations for patients with sickle cell disease. Retrospective cohort of sickle cell disease-related emergency department (ED) visits and hospitalizations from select states in the 2005 and 2006 Healthcare Cost and Utilization Project (HCUP) State Inpatient Databases and State Emergency Department Databases. Eight geographically dispersed states (Arizona, California, Florida, Massachusetts, Missouri, New York, South Carolina, and Tennessee) that provide encrypted identifiers and have sufficient numbers of patients with sickle cell disease; together these states have 33% of the US population with sickle cell disease. A total of 21,112 patients with sickle cell-related treat-and-release ED visits or inpatient hospitalizations. Rates of acute care utilization and rehospitalizations. Population-based utilization rates were also calculated. The 21,112 people with sickle cell disease had 109,344 encounters, a mean of 2.59 (95% confidence interval [CI], 2.53-2.65) encounters per patient per year, 1.52 (95% CI, 1.48-1.55) encounters for hospitalizations and 1.08 (95% CI, 1.04-1.11) for treat-and-release ED visits. Utilization was highest for 18- to 30-year-olds, 3.61 (95% CI, 3.47-3.75) encounters per patient per year, and those with public insurance, 3.22 (95% CI, 3.13-3.31) encounters per patient per year. Publicly insured 18- to 30-year-olds had 4.80 (95% CI, 4.58-5.02) encounters per patient per year. Approximately 29% of the population had no encounters while 16.9% had 3 or more encounters per year. The 30-day and 14-day rehospitalization rates were 33.4% (95% CI, 33.0%-33.8%) and 22.1% (95% CI, 21.8%-22.4%), respectively. The rehospitalization rate was highest for 18- to 30-year-olds, with 41.1% (95% CI, 40.5%-41.7%) rehospitalized within 30 days and 28.4% (95% CI, 27.8%-29.0%) within 14 days. Rehospitalizations were also highest for publicly insured patients. Among patients with sickle cell disease, acute care encounters and rehospitalizations were frequent, particularly for 18- to 30-year-olds.