Table 1.

Course of acute chest syndrome

Case no.Clinical symptomsLowest Hgb, g/dL (change from baseline)O2 saturation on room air (change from baseline)Cognitive test scoresTreatmentNeurologic symptomsMRI findings
Back pain, fever,*cough 6.6 (−1.8) 80% (−16%) Verbal IQ, 86; performance IQ, 103 Blood transfusion for 5 y Frontal headache RPLS, silent cerebral ischemia  
Abdominal, back pain, respiratory distress 3.7 (−2.7) 92% (−6%) Verbal IQ, 73; performance IQ, 73 Hydroxyurea for 2 mo Seizure, change in mental status RPLS, with superimposed ischemia  
Fever,*abdominal pain, cardiorespiratory arrest 5.1 (−3.5) 89% (−9%) Verbal IQ, 79; performance IQ, 105 Blood transfusion for 2 y; currently, on hydroxyurea Delayed recovery to baseline alertness RPLS, hemorrhage  
Abdominal pain, fever,* respiratory distress 6.5 (−2.1) 89% (−11%) Verbal IQ, 78; performance IQ, 100 Blood transfusion for 4 mo Dependent for locomotion and communication Laminar necrosis and incomplete infarction  
Chest, abdominal pain, fever,* respiratory distress 6.9 (−1.0) 90% (−5%) General IQ, 85 None Complex partial seizure with secondary generalization Frontal subarachnoid hemorrhage 
Case no.Clinical symptomsLowest Hgb, g/dL (change from baseline)O2 saturation on room air (change from baseline)Cognitive test scoresTreatmentNeurologic symptomsMRI findings
Back pain, fever,*cough 6.6 (−1.8) 80% (−16%) Verbal IQ, 86; performance IQ, 103 Blood transfusion for 5 y Frontal headache RPLS, silent cerebral ischemia  
Abdominal, back pain, respiratory distress 3.7 (−2.7) 92% (−6%) Verbal IQ, 73; performance IQ, 73 Hydroxyurea for 2 mo Seizure, change in mental status RPLS, with superimposed ischemia  
Fever,*abdominal pain, cardiorespiratory arrest 5.1 (−3.5) 89% (−9%) Verbal IQ, 79; performance IQ, 105 Blood transfusion for 2 y; currently, on hydroxyurea Delayed recovery to baseline alertness RPLS, hemorrhage  
Abdominal pain, fever,* respiratory distress 6.5 (−2.1) 89% (−11%) Verbal IQ, 78; performance IQ, 100 Blood transfusion for 4 mo Dependent for locomotion and communication Laminar necrosis and incomplete infarction  
Chest, abdominal pain, fever,* respiratory distress 6.9 (−1.0) 90% (−5%) General IQ, 85 None Complex partial seizure with secondary generalization Frontal subarachnoid hemorrhage 
*

Fever is defined as temperature >38.5°C.

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