Objectives 1. Describe different combinations of normal and abnormal hemoglobins and their phenotypic expression 2. Describe the pathophysiology of vasooclusion secondary to hemoglobinopathies 3. Define and describe hand-foot syndrome 4. Describe features of osteomyelitis secondary to sickle cell disease and its differentiation from bone infarct 5. Describe features of avascular necrosis of bon...

Recently, ithas been demonstrated thatthe offspring of one parent with the sickle cell trait and one w’ith thalassemia may develop a severe hemolytic anemia with some of the characteristics of both thalassemia and sickle cell disease, ineluding microcytosis and numerous sickled erythrocytes in the blood film. This syndrome has been called mi(’rodrepanocytic,9 or sickle cell-thalassemia, disease...

background: children affected with sickle cell disease (scd) are at increased risk for severe morbidity and mortality, especially during the first 3-5 years of life. it is suggested that early treatment can improve the condition. the aim of this pilot study was to estimate the incidence of hemoglobin s (hbs) by umbilical cord blood screening in khorramshahr and abadan cities in southwest of ira...

conclusions the results of this study showed that self-management interventions are effective in promoting self-efficacy in patients with sickle cell disease. thus, the use of self-management programs is advisable to change behaviors and promote self-efficacy in such patients. background patients with sickle cell disease suffer from various complications during their lifetime. in order to cope ...

background: hemoglobin s arises is the result of a point mutation (a-t) in the sixth codon on the -globin gene on chromosome 11 causing sickle cell anemia. the presence of fetal hemoglobin in infancy plays a relatively protective role for vaso-occlusive symptoms that are the major contributor for the morbidity and mortality among patients with sickle cell anemia. hydroxyurea, an s-phase-specif...

Sickle cell trait is a relatively common condition in the African-American population. Individuals with this condition may have any of several complications under rare circumstances. We report a patient who presented with extensive compartment syndrome leading to death. A 31-year-old African-American male with known history of sickle cell trait developed extensive compartment syndrome followed ...

We describe for the first time retinal changes in sickle cell/hereditary persistence of fetal haemoglobin syndrome, which is a rare and benign disorder. The changes are qualitatively similar to retinal disease seen with sickle haemoglobin and sickle C haemoglobin, but are mild.

Objective. To review issues related to asthma in sickle cell disease and management strategies. Data Source. A systematic review of pertinent original research publications, reviews, and editorials was undertaken using MEDLlNE, the Cochrane Library databases, and CINAHL from 1947 to November 2010. Search terms were [asthma] and [sickle cell disease]. Additional publications considered relevant ...

objective(s) sickle cell disease is a genetic disorder characterized by chronic haemolytic anaemia. haemoglobin s containing red blood cells may be susceptible to oxidative stress due to imbalance between production of reactive oxygen species and the countering effect of the various antioxidants present in the body. materials and methods we evaluated some antioxidant enzymes which include gluta...