Abstract
Background: the number of cases with Sickle-Cell Disease (SCD) registered in Italy at 2000 was 928. Six-hundred-twenty-two (67%) of these were with Sickle-Cell/βthalassemia. Moreover, while the main causes of death in Afro-American patients with SCD is well known, we do not have many informations in Italian subjects. For this reason, we carried ahead a case-control study in this group of patients.
Aim of the study: the evaluation of the main hematological and clinical parameters predictive of mortality.
Materials and Methods: clinical and hematological findings of 14 SCD died patients were compared with a SCD alive control population homogeneous for age, sex, phenotype and number of painful crises per year. Two alive control subjects were studied for each died patient.
Results: the main causes of death were: Acute Chest Syndromes (ACS) (n°6), Cardiovascular Diseases (n°3),Hepatic Failure (n°1),Hodgkin Lymphoma (n°1), Stroke (n°1), Lung Cancer (n°1), Surgery Complication (n°1). The main correlated hematological findings with mortality were Hb (gr/dl) levels (p=<.05), WBC count (103 / ml) (p= <.05), ferritin levels (ng/ml) (p=<.01). The main correlated clinical features were: the number of crises >3/year (Odd Ratio= 1.64; p= <.01), previous stroke (Odd Ratio= 1.53; p= <.01), HU treatment (Odd Ratio=<.01; p=<.01), leg ulcers (Odd Ratio=1.46;p=<.01).
Conclusions: these data suggest that the main causes of death in Italian patients with SCD are similar to those reported for Afro-American subjects (M.H. Steinberg, JAMA, April 2, Vol. 289, N°13, 1645–1651). Particularly, the lack of HU treatment, anemia, high ferritin levels, number of crises per year and previous stroke are the most important predictive factors of mortality.
Hearing loss among adults with sickle cell disease in an endemic region: a prospective case-control study