Race and sex differences in sickle cells retinopathy

Presentation Description : Sickle cell anemia is an inherited blood disorder, in which red blood cells, normally round, become misshaped, like sickles, and clog the vessels causing little vessels occlusion with painful episodes, known as sickle cell crises. The retinal vessels, being the smallest of the body, often are closed. The erythrocytes can take their normal elastic structures after normalization of the stress, but after repeated cycles of sickling and unsickling, they become hard bodies, permanently. The rigid cells induced chronic endothelial damage causes tissue ischemia, infarctions, and end-organ failures even in the absence of obvious vascular occlusions due to the damaged and oedematous endothelium. The high prevalence of sickle-cell retinopathy and the potentially severe complications associated with this disease justify the interest to perform an analysis of some epidemiologic features linked to genetic assessment of the disease. We tried to understand whether or not there is a gender and race difference in clinical severity of SCDs. The study was performed in the Ophthalmologic Service of the Santa Marta Hospital Catania University between January 2007 and April 2015. All patients with SCDs were enrolled into the study. SCDs are diagnosed by the haemoglobin electrophoresis performed via high performance liquid chromatography (HPLC). Their medical histories including numbers of painful crises per year, units of transfused red blood cell (RBC) in their lives, regular alcohol consumption, smoking habit, leg ulcers, and stroke were recorded. We observed a correlation between gender and race in sickle-cell retinopathy in a group of people in the South of Italy (Sicily). Furthermore, due to the immigration from North Africa, in Sicily we can detect race differences in sickle-cell retinopathy using the same method of observation. In fact, Sickle-cell retinopathy is often misunderstood in the African populations because of inadequate ophthalmologic screening.