Sickle cell disease. Primary Care management
Reference of this article: Cervera Bravo A, Cela de Julián E. Sickle cell disease. Primary Care management. Rev Pediatr Aten Primaria. 2007;9:649-68.
Published in Internet: 31-12-2007
Sickle cell disease is a genetic structural haemoglobinopathy characterized by the presence
of haemoglobin S that is becoming more prevalent in our environment because of the present
immigrating pressure. The abnormal haemoglobin is unstable, tends to polymerize occluding
the microcirculation what produces acute and chronic multisystem manifestations and
increases the susceptibility of infections. Genetic aspects, physiopathology, clinical and laboratory
diagnosis, neonatal screening, appropriate care of the main acute manifestations as lifethreatening
complications that may develop rapidly, a follow-up plan, immunizations and treatment
are discussed. Due to the complexity of the disease a multidisciplinary care is
necessary coordinating primary care with specialized clinical management that includes periodic
comprehensive evaluations and patient and family education as this decreases morbidity
and mortality and improves quality of life for these patients.
Key words: Sickle cell disease, Primary Care, Children.
Keywords● Primary Care ● Sickle cell disease