Morbidity and mortality in patients with sickle cell disease: study of a series of cases
Keywords:
anemia de células falciformes, mortalidad, crisis vaso-oclusivas, complicacionesAbstract
Introduction: Sickle cell disease is a disease with great clinical variability.
Objective: To describe the evolution of a series of patients followed up in an institution.
Methods: Observational, descriptive, longitudinal, retrospective study. Universe was made up of 98 adults with sickle cell disease followed up between 2001 and 2017, at the “Dr. Gustavo Aldereguía” Hospital. By reviewing the medical records, it was verified if different clinical events related to the disease occurred and they were quantified when appropriate. The association with other pathological conditions and the existence of chronic organic damage were recorded. In the case of women, we also collected their obstetric history. Causes of death were taken from death certificates and autopsy reports. Survival was estimated using Kaplan-Meier curves.
Results: 88 patients were included, 46 of them women, the type of hemoglobinopathy that prevailed was sickle cell anemia (67%). Painful vaso-occlusive crises (89.8%) and chest syndrome (35.2%) were the most common acute events, and gallstones (36.3%) and legs ulcers (22.7%) predominated among the chronic ones. In the 64 pregnancies followed, we registered a bad outcome in three (one maternal death and two late fetal deaths). Overall survival was 64 years, with significant differences between the sexes (69 years old in women and 58 years old in men). The most common causes of death were chronic kidney disease and congestive heart failure.
Conclusions: The frequencies of most clinical events were similar to those described in the literature. Late fetal and maternal mortality are higher than in the general population. The most common causes of death are related to the chronic organ damage manifestations of the disease. Overall survival was higher than that previously described in Cuba.
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