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Prepublished online as a Blood First Edition Paper on September 26, 2002; DOI 10.1182/blood-2002-04-1183.
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Blood, 15 January 2003, Vol. 101, No. 2, pp. 415-419
CLINICAL OBSERVATIONS, INTERVENTIONS, AND THERAPEUTIC TRIALS
Reversible posterior leukoencephalopathy syndrome and silent
cerebral infarcts are associated with severe acute chest syndrome in
children with sickle cell disease
Jessica N. Henderson,
Michael J. Noetzel,
Robert C. McKinstry,
Desiree A. White,
Melissa Armstrong, and
Michael R. DeBaun
From the Department of Pediatrics, Division of
Pediatric Hematology Oncology, Department of Neurology, Department of
Pediatrics, Mallinkrodt Institute of Radiology, Neuroradiology, and
Department of Psychology, Washington University School of Medicine, St
Louis, MO.
Patients with severe acute chest syndrome (ACS) requiring
endotracheal intubation and erythrocytopheresis are at increased risk
for neurologic morbidity. This study examines patients with sickle cell
disease who developed severe episodes of ACS, leading to endotracheal
intubation, ventilatory support for respiratory failure, and
erythrocytapheresis. Magnetic resonance imaging (MRI) and magnetic
resonance angiography (MRA) studies, a neurologic examination by a
pediatric neurologist, and cognitive testing were done in all patients.
Five consecutive patients, aged 3 to 9 years, were identified with
severe ACS. All patients developed neurologic complications resulting
from ACS episodes, including seizures (n = 2), silent cerebral
infarcts (n = 3), cerebral hemorrhage (n = 2), and reversible
posterior leukoencephalopathy syndrome (n = 3). Children with severe
ACS should have a magnetic resonance image of the brain, neurologic
examination by a neurologist, and cognitive testing to detect the
presence of neurologic morbidity.

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