[Sickle cell disease: registry of the Spanish Society of Pediatric Hematology].
BACKGROUND
Sickle cell disease (SCD), a genetic anemia, is currently an emerging health problem in Spain. Since 2000, The Spanish Society of Pediatric Hematology has maintained a registry of these patients. The data corresponding to 2004 are presented herein.


METHODS
Information was sent by different national hospitals. Pediatric patients with SCD followed-up during 2003 were registered in the first quarter of 2004. Data on epidemiology, diagnosis, treatment and outcome in each patient were gathered.


RESULTS
A total of 138 patients in 24 national hospitals were registered. Of these, 99 were still under follow-up. There was no significant difference in sex. The mean age was 8.2 years. Seventy-eight percent of the patients were homozygous. Forty-four percent were born in Africa but 76% had abnormal genes originating in Africa. Neurophysiologic disorders were detected in 36% of the patients. Symptomatic treatment was given in 65%, hydroxyurea in 27%, hypertransfusional therapy in 3%, and chelation therapy, indicated for ferric overload, was provided in 4%. None of the patients underwent stem cell transplantation. Acute complications requiring hospitalization occurred in 21%, and chronic complications were observed in 27%. The most frequent chronic complications were delayed height and weight gain and liver and biliary tract disorders. Two patients died.


CONCLUSIONS
This study confirms a highly significant increase in the prevalence of pediatric patients with SCD in the last 4 years, requiring greater resources to be devoted to the diagnosis and follow-up of this disease.
EAP Cerro Almodóvar, Area 1, Madrid. Spain. martagarias@telefonica.net
M B
MB
García Arias
M A
MA
Cantalejo López
M E
ME
Cela de Julián
R
R
Bravo Clouzet
P
P
Galarón García
C
C
Beléndez Bieler
D000755:Anemia, Sickle Cell / Q000453:epidemiology*; D002648:Child; D005260:Female; D006801:Humans; D008297:Male; D012042:Registries; D013030:Spain / Q000453:epidemiology