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Morbidity-mortality in sickle cell disease

The high morbidity-mortality rates in sickle cell disease (SCD) led us to study the epidemiological profile and respective clinical complications of patients seen at a Regional Blood Center (HR) and a University Clinical Hospital (HC-U) between 1998 and 2007. In a retrospective study, 151 patients were evaluated regarding age, gender, skin color, origin, diagnosis, reason of visit to HR or reason and length of stay in the HC-U, and cause and age in cases of death. Simple descriptive statistical analysis was performed. The mean age was 17.7 years, 52.4% of the patients were female, 58.2% were from the city and no information regarding skin color was available in 92.2% of the records. Sickle cell anemia was present in 82.5% of cases. The most frequent reason for the 910 visits to HR and 589 hospitalizations in HC-U were afebrile pain episodes (61.9% and 25.3%, respectively). The mean age of the 11 patients who died was 33.5 years with one patient being younger than 10 years old, and multiple organ failure was the most frequent cause. The epidemiological profile shows a predominance of children and young adults, women, and the SS genotype. The rates of hospitalization in HC-U and of visits to HR and the low mean age at death confirm the high morbidity and mortality related to SCD. However, a large number of children do not present with complications or require hospitalization which reflects the efficacy of preventive measures provided by the early diagnosis implemented over the last 10 years.

Anemia, sickle cell; Morbidity; Mortality


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