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Results for “sickle cell disease”

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At least 19 recordsLinked to original sources

PubMed2003

A revisit of sickle cell disease, sickle cell trait, and the extra transverse digital crease on the fingers.

BACKGROUND: Alteration in the location and number of palmar creases has been found in association with certain disorders. The extra transverse digital crease (ETDC) has been reported in sickle cell disease. This study was carried out to determine the importance of ETDC as a diagnostic tool for sickle cell disease amongst Nigerians. METHODS: Medical students and student nurses with available hemoglobin electrophoresis records were studied. Their palms were examined for the presence of ETDC. RESULTS: An ETDC was present in 80 of 178 (44.9%) cases with genotype AA, 26 of 68 (38.2%) cases with sickle cell trait (AS, 65; AC, 3), and 10 of 22 (45.4%) cases with sickle cell disease (SS). CONCLUSIONS: The results from our study show that the ETDC is not a diagnostic sign of sickle cell disease in Nigerians.

Anemia, Sickle Cell↗

PubMed1997

Hemoglobin A2 levels in healthy persons, sickle cell disease, sickle cell trait, and beta-thalassemia by capillary isoelectric focusing.

Simultaneously measuring major and minor hemoglobin (Hb) variants by capillary isoelective focusing, we obtained HbA2 intervals in healthy volunteers (n = 412) (reference value) and patients with HbS or beta-thalassemia. We classified normal HbA2 reference intervals into three age groups: 5 months or younger (1.2% +/- 1.5%), 6 months to 1 year (2.2% +/- 0.9%), and 1 year or older (2.4% +/- 0.9%). These intervals were comparable to those used with other methods. Patients 1 year of age or older with HbS had significantly higher HbA2 levels (sickle cell trait, 2.9% +/- 0.9%; sickle cell anemia, 2.8% +/- 1.0%; P < .05). Although reference HbA2 intervals overlapped those in patients with HbS, no overlap in HbA2 levels was noted between these groups and patients with beta-thalassemia (observed range, 4.3% to 7.5%). The higher than normal HbA2 interval in patients with HbS must be considered before a diagnosis of sickle cell trait or sickle cell disease with beta-thalassemia is made.

Anemia, Sickle Cell↗

PubMed1998

The natural history of sickle cell disease.

Sickle cell disease results in significant morbidity and mortality for those affected with this disease. Despite the fact that every case of sickle cell disease results from the same genetic mutation, there is considerable heterogeneity in the clinical course of the disease. Considerable knowledge has been gained regarding the complications of sickle cell disease including pain episodes, infections, stroke, and lung disease. Clarifying the clinical course of sickle cell disease has enabled us to develop therapies to decrease the morbidity of this disease and, with hope, will direct further investigations into novel treatment interventions.

Bacterial Infections↗

PubMed2004

Established and experimental treatments for sickle cell disease.

Sickle cell disease (SCD) is characterized by the presence of sickle hemoglobin (HbS), which has the unique property of polymerizing when deoxygenated. The sickling process is markedly accelerated when intracellular concentration of HbS is increased. Due to the unique dependence of HbS polymerization on its cell concentration, a slight reduction in HbS concentration is likely to have a beneficial effect on the kinetic of polymerization and on the generation of dense, dehydrated red cells. The pathophysiology of acute and chronic clinical manifestations of SCD is strictly related to the hemoglobin cyclic polymerization, to the generation of dense, dehydrated red cells and to the interaction between sickle red cells and abnormal activated vascular endothelial cells. In the present paper we have reviewed the principal therapeutic strategies and we have explored the future treatment options for sickle cell disease. Therapy of sickle cell disease is based on two major goals. The first one is the decrease in intracellular HbS concentration obtained with agents activating fetal hemoglobin synthesis, such as hydroxyurea (HU) or with erythrocyte-active agents blocking different red cell membrane ion pathways and preventing sickle cell dehydration. The second one is based on therapeutic strategies, which may reduce sickle cell-endothelial adhesive events.

Anemia, Sickle Cell↗

PubMed2004

Acute myocardial infarction in a young man with sickle cell disease.

Sickle cell disease is considered protective against large vessel coronary artery disease. Although sickle cell patients do develop myocardial degeneration and fibrosis at a higher rate than age-matched controls, they rarely suffer from an acute myocardial infarction. We present a case of a 29-year-old man with sickle cell disease who presented with an acute non-ST segment myocardial infarction. In sickle cell patients who present with chest pain as an element of their sickle cell crisis, the clinician must consider acute myocardial infarction in the differential along with more common entities like acute chest syndrome.

Adult↗

PubMed2002

Sickle cell disease.

Sickle cell disease is a serious and life threatening disease that affects approximately 1 in 600 African-Americans. Since its first description in 1910 by Herrick, our understanding and treatment of this disease has grown tremendously. Most pediatricians will encounter one or more children with sickle cell disease and should have a thorough understanding of the disease, its complications, and treatment. This article summarizes present day knowledge about the complications and potential treatments for patients with sickle cell disease.

Anemia, Sickle Cell↗

PubMed1999

The lung in sickle cell disease.

Sickle cell disease is the most common inherited disorder in African-Americans. Although the primary defect is hematological, the changes in the erythrocytes lead to a vasculopathy with multiorgan injury. The pulmonary complications, i.e., acute chest syndrome and chronic sickle cell lung disease, are significant causes of morbidity and mortality. The pulmonary manifestations result from a unique constellation of factors which come into play in sickle cell disease. Based on the growing understanding of the molecular and cellular biology of sickle cell disease, new therapies are being developed that are likely to ameliorate the natural history of this disease and its complications.

Anemia, Sickle Cell↗

PubMed2005

Anaesthesia for peculiar cells--a century of sickle cell disease.

Sickle cell disease is a congenital haemoglobinopathy with a high incidence of perioperative complications. Traditional anaesthetic management, based largely on extrapolation from biochemical models, has emphasized avoidance of red cell sickling to prevent exacerbations of the disease. This historical review outlines the evolution of the traditional approach to sickle cell pathology, assesses the validity of this model, describes the emergence of the concept of the disease as one defined by chronic inflammatory vascular damage, and outlines the practical implications of this new approach.

Acute Disease↗

PubMed1994

Genotypic evaluation of ocular pathologies in sickle cell diseases.

Sickle cell disease patients are known to manifest different types of ocular problems. These problems include proliferative and non-proliferative retinopathies, and refractive errors. The distribution of these pathologic and refractive problems among the sickle cell genotypes is analyzed according to the individual genotypes. Data collected from a total of 63 sickle cell disease patients who responded to the questionnaires shows that fifty-six percent (35) are males and forty-four percent (28) are females. The genotype distributions are: SS, 55% (34); SC, 33% (21); S-Thal, 11% (7); while 2% (1), is AS-genotype. Overall findings show that severe ophthalmic abnormalities were reported by approximately 90% of the respondents with sickle cell disease. The majority of sickle cell disease patients in this research show consistent susceptibility to both fungal and bacterial infections with varying degree of refractive errors. It is observed that refractive errors are prevalent in SC and S-Thal sickle cell genotypes, while the severity of anemia and painful crises are more prevalent in SS and S-Thal genotypes. Systemic ocular problems are also prevalent among the SC and S-Thal disease genotypes.

Adolescent↗

PubMedDate not supplied

Otolaryngologic manifestations of sickle cell disease.

Sickle cell disease is a common hereditary hematologic disorder characterized by the presence of a structurally abnormal hemoglobin molecule. Physicochemical properties of sickle hemoglobin result in a chronic hemolytic anemia and in vaso-occlusive episodes with ischemic injury of many tissues. In patients with sickle cell disease the otolaryngologist may be confronted with lesions affecting the ear, the bones and soft tissues, and the nervous system. Surgery and anesthesia also present special problems. An outline and discussion of these clinical concerns are presented.

Anemia, Sickle Cell↗

PubMedDate not supplied

Management of the patient with sickle cell disease.

Sickle cell disease is a group of genetic disorders of hemoglobin synthesis, of which sickle cell anemia is the most severe. In the United States, sickle cell disease primarily affects persons of African-American and Hispanic origin. An estimated 50,000 Americans have the disease, and approximately 2.5 million persons carry the sickle cell trait. The primary pathophysiologic features are chronic hemolysis and vaso-occlusion. This disease is recurrent and unpredictable, causing considerable physiologic and psychologic stress. This article provides an overview of the disease and addresses the role of the infusion nurse in the care and support of the patient with sickle cell disease in the hospital and home setting.

Anemia, Sickle Cell↗

PubMed2004

[Acute complications in sickle cell disease].

Sickle cell disease is an inherited disease characterised by the presence of an abnormal haemoglobin. Sickle cell disease can be complicated by acute vaso-occlusive crisis, which are the major clinical problem prompting admission to hospital and the major cause of death. It mainly manifests by osteo-articular pain and acute chest syndrome and can be complicated by multi-organ failure. The main treatment of severe acute vaso-occlusive crisis is based on transfusion.

Acute Disease↗

PubMed2002

Developing treatment for sickle cell disease.

Sickle cell disease pathophysiology results from sickle haemoglobin polymerisation and its effects on the sickle erythrocyte and the vasculature. Many of the abnormalities of sickle cell disease are secondary to the damage caused by the polymer and the injured red cell. Pharmacological treatment of the disease is focused on the inhibition of sickle haemoglobin polymerisation, prevention or repair of red cell dehydration and interruption of the interaction of sickle cells with the endothelium.

Anemia, Sickle Cell↗

PubMed1990

Rheumatoid arthritis in a patient with sickle cell disease.

Sickle cell disease has various articular manifestations. Coexistent rheumatoid arthritis (RA) and sickle cell disease has been reported rarely. We present a patient with sickle cell disease and seropositive erosive RA demonstrating characteristic radiographic findings of the 2 entities.

Anemia, Sickle Cell↗

PubMed1997

The role of CT and MR in imaging the complications of sickle cell disease.

Sickle cell disease is the most common inherited haemoglobinopathy described. Complications of sickle cell disease (SCD) are due to chronic haemolysis of fragile red cells or secondary to vascular occlusion by sickled red cells with subsequent tissue infarction. Traditionally plain film radiography has been the mainstay in the assessment of patients with SCD, but increasingly magnetic resonance (MR) imaging and computed tomography (CT) are being used. In this review the imaging features of a range of complications of SCD are demonstrated with particular emphasis on CT and MR.

Anemia, Sickle Cell↗

PubMed2006

The cognitive and academic impact of sickle cell disease.

Sickle cell disease (SCD) affects over 30,000 students in the United States. Central nervous system complications are widespread among students with SCD and include stroke, silent cerebral infarction, and cognitive impairment. The effects of these complications may lead to academic failure, limited career options, and for some, total disability. Despite studies describing the significant academic and cognitive impact of sickle cell disease, reports describing interventions are limited. There is a lack of awareness among educators of the academic risks associated with sickle cell disease and a lack of appropriate resource allocation. The school nurse, as community health advocate, will be called upon to bridge the gap among healthcare providers, parents, students, and educators. This article provides a review of both recent and landmark studies describing the cognitive and academic impact of sickle cell disease and discusses the role of the school nurse as an advocate, liaison, and educator.

Anemia, Sickle Cell↗

PubMed1995

Optimal haematocrit and haemoglobin S levels in pregnant women with sickle cell disease.

Sickle cell blood has abnormal rheological characteristics and its viscosity is determined by the level of haemoglobin S and the PCV. Rheological studies have shown that alteration of these parameters will improve oxygen delivery to the tissues. However, there is no agreed policy for optimum levels for haemoglobin S and PCV in the management of pregnancies complicated by sickle cell disease. In this study rheological measurements have been performed on pregnant women with sickle cell disease and compared with women without haemoglobinopathy. Potential oxygen delivery was not found to approach normal levels until very low levels of HbS were achieved. The optimal PCV for women with sickle cell disease was found to be 0.26 compared with 0.33 for normal women.

Anemia, Sickle Cell↗

PubMed1993

Contraceptives, counselling, and pregnancy in women with sickle cell disease.

Sickle cell disease is listed in the manufacturers' data sheets in the United Kingdom as a contraindication to the use of most combined contraceptive pills; the result is confused advice on family planning to a group of women who are at substantial risk from both planned and unplanned pregnancy. A study in north London on the use of contraceptives by women with sickle cell disease indicates that the use of combined oral contraceptives is common. Although medical staff usually advised against pregnancy, such advice was almost always ignored. Over half of the women surveyed had some knowledge about antenatal diagnosis. Family planning advice should be an integral part of the care of women with sickle cell disease. In the absence of specific data to the contrary all methods of contraception may be considered, although with appropriate caution.

Adolescent↗