ISCHEMIC STROKE AS AN OUTCOME OF SICKLE CELL ANEMIA IN CHILDHOOD: CASE REPORT (Atena Editora)

Abstract

Sickle cell anemia results from a genetic alteration characterized by a mutant hemoglobin, hemoglobin S (HbS), which in its homozygous state causes distortion of erythrocytes. The decompensation of the disease occurs when this hemoglobin undergoes polymerization at the time it is deoxygenated, either due to a reduction in the oxygen concentration or due to factors that reduce the oxygen-hemoglobin bond, such as an increase in temperature or a drop in pH. Such an event can cause premature destruction of abnormal erythrocytes by the spleen and microvascular vasocclusion, causing tissue ischemia, acute pain and gradual damage to the affected organ. The ischemic cerebrovascular accident (CVA) is one of the most serious outcomes of this disease, which is 280 times more common in children with Sickle Cell Anemia. of an 8-month-old infant with Sickle Cell Anemia, who presented fever and prostration, requiring blood transfusion and evolving with 2 epileptic seizures, with an extensive CVA being evidenced in the cranial tomography. In order to elucidate the importance of the reported case, an active search of epidemiological data was carried out at a global and regional level, highlighting the prevalence of cases of sickle cell anemia at Hospital Alcides Carneiro in relation to age group, gender and reason for hospitalization. Finally, with the outcome of the case, the importance of screening and early diagnosis of this disease becomes evident, with a description of an initial approach protocol for children with sickle cell anemia who present with fever in the emergency room.