UNLABELLED INTRODUCTION The occurrence of systemic lupus erythematosus has been only rarely reported in patients with sickle-cell disease. CASE PRESENTATION We describe the case of a 23-year-old North-African woman with sickle-cell disease and systemic lupus erythematosus, and discuss the pointers to the diagnosis of this combination of conditions and also present a review of literature. T...

BACKGROUND Interstitial lung disease is a frequent complication in sickle cell disease and is characterized by vascular remodeling and interstitial fibrosis. Bone marrow-derived fibrocytes have been shown to contribute to the pathogenesis of other interstitial lung diseases. The goal of this study was to define the contribution of fibrocytes to the pathogenesis of sickle cell lung disease. ME...

Sickle cell disease (SCD) is caused by a mutation in the sixth codon of the β-globin gene on chromosome 11, which leads to a single amino acid substitution (glutamine to valine). Sickle-(δβ)0-thalassemia is a rare variant of sickle cell disease (delta-beta thalassemia occurring in association with sickle hemoglobin, HbS), sparsely reported in literature, and has been associated with symptomatol...

The aim of the study was to determine the factors associated with resting and exercise-induced hemoglobin oxygen desaturation. The well-established six-minute walk test was conducted in 107 sickle cell children (50 with sickle hemoglobin C disease and 57 with sickle cell anemia) at steady state. Hemoglobin oxygen saturation was measured before and immediately after the six-minute walk test. Blo...

Background Drugs used by patients with sickle cell anemia to relieve pain have side effects, so the use of non-pharmacological palliative methods (such as massage, muscle relaxation, heat, drinking fluids, etc.) can be considered. We aimed to determine effect of non-pharmacological palliative methods training among adolescents with sickle cell anemia. <stro...

Sickle cell trait is not usually regarded as a disease state because it has complications that are either uncommon or mild. Nevertheless, under unusual circumstances, serious morbidity or mortality can result from complications related to polymerization of deoxy-hemoglobin S. A previous study was earlier conducted to study Sickle cell traits and it revealed that there was enhanced lipid per oxi...

By JAMES V. NEEL, PH.D., M.D., HARVEY A. ITANO, PH.D., M.D., AND JOHN S. LAWRENCE, M.D. I N RECENT YEARS our knowledge of the etiology of sickle cell disease has increased considerably. As a rule, both parents of a child with this disease show the sickle cell trait. This has led to the hypothesis that usually sickle cell disease is due to homozygosity for a gene which, when heterozygous, result...

BACKGROUND Patients with sickle cell disease cope with their disease in various ways, such as psychological counseling, hypnosis, medication, and prayer. Spirituality is a coping mechanism in a variety of diseases. This study evaluates the role of spirituality in patients coping with the pain of sickle cell disease. METHODS Seventy-one patients from the Georgia Sickle Cell Clinic completed a ...