Incidence of Sickle Cell Disease and Other Hemoglobin Variants in 10,095 Lebanese Neonates

Abstract

Hemoglobinopathies are highly prevalent diseases and impose a public health burden. Early diagnosis and treatment can ameliorate the course of these diseases and improve survival. Despite purported high incidence of hemoglobinopathies in Lebanon, there are no nationwide screening programs. In this study, newborn screening utilizing high pressure liquid chromatography was executed in all public hospitals across Lebanon between 2010 and 2013. All newborns with an abnormal hemoglobin (Hb) were offered genetic counseling and all those with disease were enrolled in comprehensive hemoglobinopathy clinics. Among newborns, 2.1% were found to have an abnormal Hb variant with sickle Hb being the most common while 0.1% were found to have sickle cell disease (SCD). The majority of those with SCD had non-Lebanese origins. The most common causes of hospitalizations in infants with SCD were acute splenic sequestration and pain crises. No bacteremia or other life threatening infections were noted. At a median follow up 14 months (follow up range 7 to 34 months), all children with disease are alive and compliant with treatment. Systematic screening for SCD and other Hb variants was shown to be feasible, cost effective, and of accurate predictive value. This program was also clinically effective because it led to the identification of babies with disease and to providing them with free early multidisciplinary care. Conclusively, a newborn screening program should be implemented across Lebanon to detect hemoglobinopathies and initiate early therapeutic and preventive strategies and genetic counseling.

Prevalence of Sickle Cell Trait in the Southern Suburbs of Beirut, Lebanon

“…Another factor that could have affected our results could be due to the change in the ethnic background, as reported by Khoriaty et al. 25 Also our results were lower than some of the reported prevalence of Sickle Cell Trait internationally. In a study conducted by Ojudu et al, it was reported that in 44 states of the United States for which data were available, of all infants screened for the Sickle Cell Trait in 2010, 1.5% tested positive.…”

“…The carrier rate of Hb variant varried amongst regions, between 5.7 and 50.2%, being the highest in Northern Lebanon, whereas it is 13.2% (0.27% of the studied population) in Beirut. 25 …”

The Daily Experiences of Adolescents in Lebanon With Sickle Cell Disease

“…The World Health Organization estimates that 21.7% of people in the Middle East region carry variants for either SCD or thalassemias (Modell & Darlison, 2008;World Health Organization, 2006). In Lebanon, a recent study found that among newborns, 2.1% were found to have an abnormal hemoglobin variant, with sickle hemoglobin being the most common variant (84%) and carrier rates being highest in Northern Lebanon (Khoriaty et al, 2014). These estimates may be lower than the actual incidence because of the early discharge of newborns and lack of newborn screening at many institutions.…”

“…The term refers to a collection of autosomal-recessive genetic disorders characterized by the hemoglobin S (HbS) variant of the b-globin gene that is resistant to malaria development and in which more than 50% the hemoglobin-b gene is replaced with HbS (El-Hazmi, Al-Hamzi, & Warsy, 2011;Saraf et al, 2014). SCD affects 1 in 500 African Americans and more than 30 million people worldwide, with predominance in populations of the sub-Sahara, India, and the Middle East (Inati et al, 2007;Khoriaty et al, 2014;McGann, 2014). There are different variants of the b-globin gene in SCD, including but not limited to sickle cell anemia, sickle-hemoglobin C disease, sickle beta-plus thalassemia, and sickle beta-zero thalassemia, with prevalence of certain variants types in particular ethnic groups (El-Hazmi et al, 2011;Saraf et al, 2014).…”

Multi-Informative and Specific Detection of Blood in Fingermarks via MALDI-MS Based Strategies

“…Once the presence and provenance of blood has been demonstrated, it is, in theory, possible to obtain a plethora of additional information from it. Considering various blood diseases and protein variants like haemoglobinopathies, it might be possible to determine if the injured is suffering from a particular disease such as sickle cell disease [85], diabetes [86][87][88] or even myocardial infarction [89]. It can be hypothesized that this can narrow down the search by indicating that the injured will have to be taking a certain medication to treat the condition.…”