[Evaluation of twenty-three episodes of acute thoracic syndrome in patients with sickle cell anemia].
BACKGROUND
Acute thoracic syndrome (pneumonia and/or lung infarction) is a significant cause of morbidity and mortality in sickle cell anemia.


OBJECTIVE
To review the clinical manifestations, management and outcome of episodes of acute thoracic syndrome in our hospital.


METHODS
We performed a retrospective review of all the episodes of acute thoracic syndrome diagnosed at our center in patients younger than 18 years of age with sickle cell anemia. Clinical, laboratory and radiological findings, outcome and treatment were analyzed. Data from patients < 3 years and > 3 years of age were compared (Fisher's exact test and the Mann-Whitney U test).


RESULTS
Twenty-three episodes of acute thoracic syndrome were evaluated in eight out of 12 patients with sickle cell anemia followed-up in our hospital. These episodes represented 36 % of the total time of admission in these patients. The most frequent cause was infection. The most frequent symptoms were fever (87 %), cough (61 %) and cold (35 %) symptoms. Seventy-four percent of the patients were not diagnosed at admission, either because the chest X-ray was normal (52 %) or because it was not performed (22 %) due to the absence of pulmonary manifestations. Patients aged more than 3 years old had more severe episodes, with greater clinical compromise and radiological involvement and increased use of analgesia. Transfusions were administered in 65 % of the episodes and in five patients (> 3 years) a partial exchange transfusion was performed. In five patients corticoid treatment was associated with febrile relapses.


CONCLUSIONS
Acute thoracic syndrome is frequent in sickle cell disease and is more severe in children older than 3 years. Its diagnosis requires a high index of suspicion, due to multiple forms of clinical presentations and normal chest radiology at admission.
Arnáez Solís
J
J
Servicio de Pediatría, Hospital de Móstoles, Madrid, Spain.
Ortega Molina
M
M
Cervera Bravo
A
A
Roa Francia
M A
MA
Alarabe Alarabe
A
A
Gómez Vázquez
María J
MJ
D000208:Acute Disease; D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000150:complications*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D011014:Pneumonia / Q000150:complications*; D011655:Pulmonary Embolism / Q000150:complications*; D012189:Retrospective Studies; D013577:Syndrome