Sickle Cell Anemia Research Today is a free monthly online journal that collates and summarizes the latest research about Sickle Cell Anemia, including details on genetics, causes, symptoms.

Complications associated with sickle cell trait: a brief narrative review.

Tsaras G, Owusu-Ansah A, Boateng FO, Amoateng-Adjepong Y

Combined Medicine-Pediatrics, Bridgeport Hospital, Yale New Haven Health, Bridgeport, Connecticut 06610, USA.

Sickle cell trait occurs in approximately 300 million people worldwide, with the highest prevalence of approximately 30% to 40% in sub-Saharan Africa. Long considered a benign carrier state with relative protection against severe malaria, sickle cell trait occasionally can be associated with significant morbidity and mortality. Sickle cell trait is exclusively associated with rare but often fatal renal medullary cancer. Current cumulative evidence is convincing for associations with hematuria, renal papillary necrosis, hyposthenuria, splenic infarction, exertional rhabdomyolysis, and exercise-related sudden death. Sickle cell trait is probably associated with complicated hyphema, venous thromboembolic events, fetal loss, neonatal deaths, and preeclampsia, and possibly associated with acute chest syndrome, asymptomatic bacteriuria, and anemia in pregnancy. There is insufficient evidence to suggest an independent association with retinopathy, cholelithiasis, priapism, leg ulcers, liver necrosis, avascular necrosis of the femoral head, and stroke. Despite these associations, the average life span of individuals with sickle cell trait is similar to that of the general population. Nonetheless, given the large number of people with sickle cell trait, it is important that physicians be aware of these associations.

Published 2 June 2009 in Am J Med, 122(6): 507-12.
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Articles on Sickle Cell Anemia published 1 June 2009:

Nutritional deficiencies in iron overloaded patients with hemoglobinopathies.   Am J Hematol, 84(6): 344-8.

One of the hallmarks of both sickle cell disease (SCD) and thalassemia major (TM) is accelerated oxidative damage. Decreased antioxidant levels and increased oxidant stress biomarkers are found in both diseases. Although isolated vitamin deficiencies have been reported in TM and nontransfused SCD patients, a comprehensive evaluation of vitamin and trace mineral levels has never been performed in chronically transfused SCD or TM patients. As vitamins and trace minerals may be consumed as a ... [Abstract] [Full-text]

Microarray analysis of liver gene expression in iron overloaded patients with sickle cell anemia and beta-thalassemia.   Am J Hematol, 84(6): 328-34.

Chronic transfusion therapy is used clinically to supply healthy erythrocytes for patients with sickle cell anemia (SCA) or beta-thalassemia major (TM). Despite the benefits of red blood cell transfusions, chronic transfusions lead to iron accumulation in key tissues such as the heart, liver, and endocrine glands. Transfusion-acquired iron overload is recognized as a cause of morbidity and mortality among patients receiving chronic transfusions. At present, there is little understanding of ... [Abstract] [Full-text]

The cost of health care for children and adults with sickle cell disease.   Am J Hematol, 84(6): 323-7.

Although sickle cell disease (SCD) is marked by high utilization of medical resources, the full cost of care for patients with SCD, including care not directly related to SCD, is unknown. The purpose of this study was to estimate the total cost of medical care for a population of children and adults with SCD. We used data from individuals diagnosed with SCD enrolled in the Florida Medicaid program during 2001-2005 to estimate total, SCD-related, and non-SCD-related cost per patient-month based ... [Abstract] [Full-text]

Hormonal contraception, sickle cell trait, and risk for venous thromboembolism among African American women.   Am J Obstet Gynecol, 200(6): 620.e1-3.

OBJECTIVE: We evaluated the effect of oral and other hormonal contraceptive (HC) use on venous thromboembolism risk among African American women and investigated whether the association was modified by the sickle cell trait. STUDY DESIGN: We report the findings of a case-control study that included 60 African American women with an idiopathic, first episode of venous thromboembolism and 196 African American controls. RESULTS: The odds of current HC use compared with noncurrent use contrasting ... [Abstract] [Full-text]

Articles on Sickle Cell Anemia published 13 May 2009:

Growth status in children and adolescents with sickle cell disease.   Pediatr Hematol Oncol, 26(4): 202-15.

OBJECTIVES: To assess the BMI status of children and adolescents with sickle cell disease (SCD) and determine if zBMI status during adolescence is predicted by gender, childhood zBMI status, disease genotype, and healthcare utilization (emergency department visits or hospitalizations). STUDY DESIGN: Medical chart reviews were conducted on 133 patients followed through a regional Comprehensive Sickle Cell Center to obtain anthropometric measures and healthcare utilization data. Gender-specific ... [Abstract] [Full-text]

Bone marrow transplantation or hydroxyurea for sickle cell anemia: long-term effects on semen variables and hormone profiles.   Pediatr Hematol Oncol, 26(4): 186-94.

Ten male subjects affected by sickle cell anemia (SCA) were studied to evaluate the long-term effects of therapies on their fertility. Their ages ranged from 18 to 34 years (median: 32 years). Four subjects were treated by hydroxyurea (HU) and 6 by hematopoietic stem cell transplantation (HSCT). The median follow-up after HU initiation and HSCT was 10.5 years (range: 8-15 years) and 15.5 years (range: 8-21 years), respectively. Three of the 6 in the HSCT group and two of the 4 in the HU group ... [Abstract] [Full-text]

Articles on Sickle Cell Anemia published 5 May 2009:

Religious/Spiritual coping in adolescents with sickle cell disease: a pilot study.   J Pediatr Hematol Oncol, 31(5): 313-8.

Religious/spiritual (R/S) coping has been associated with health outcomes in chronically ill adults; however, little is known about how adolescents use R/S to cope with a chronic illness such as sickle cell disease (SCD). Using a mixed method approach (quantitative surveys and qualitative interviews), we examined R/S coping, spirituality, and health-related quality of life in 48 adolescents with SCD and 42 parents of adolescents with SCD. Adolescents reported high rates of religious attendance ... [Abstract] [Full-text]

Hepatic iron overload in children with sickle cell anemia on chronic transfusion therapy.   J Pediatr Hematol Oncol, 31(5): 309-12.

Hepatic iron overload is a serious complication of chronic transfusion therapy in patients with sickle cell disease (SCD). No firm consensus has been reached with regard to correlation between hepatic iron content (HIC) and variables including age, number of transfusions, and serum iron makers. Also, the role of HIC in determining hepatic injury is not well established. There is scarcity of data on chronically transfused children with SCD and no other confounding liver pathology. We aimed to ... [Abstract] [Full-text]

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