included. References 1. Almeida A, Roberts I. Bone involvement in sickle cell disease. Br J Haematol 2005;129:482-490. 2. Booth C, Inusa B, Obaro SK. Infection in sickle cell disease: a review. Int J Infect Dis 2010;14:e2-e12. 3. Atkins BL, Price EH, Tillyer L, Novelli V, Evans J. Salmonella osteomyelitis in sickle cell disease children in the East End of London. J Infect 1997;34:133-138. 4. Bu...

A 22-year-old female with factitious sickle cell anemia and recurrent painful crises is described. Because she had sickle cell trait and iron deficiency anemia, she could successfully feign the symptoms of homozygous sickle cell anemia. The identification of this syndrome in patients with genetic disorders is presented.

Sickle cell anemia is a common and disabling disorder profoundly affecting mortality as well as quality of life. Up to 35% of men with sickle cell disease are affected by painful, prolonged erections termed ischemic priapism. A priapic episode may result in fibrosis and permanent erectile dysfunction. The severity of sickle cell disease manifestations is variable dependent on a number of contri...

Children with sickle cell disease, including those without evidence for cerebral infarcts, are at increased risk for cognitive deficits that can contribute to difficulties in academic and social functioning. Chronic inflammatory processes are endemic to sickle cell disease and are apparent in common comorbidities including asthma. Cytokines mediating inflammatory processes can influence cogniti...

Studies have questioned whether renal dysfunction in sickle cell disease is linked to hemolysis-associated vasculopathy. We have investigated renal function and markers of hemolysis in a cohort of 424 adult African-British patients with sickle cell disease. While significant associations were found in HbSS and HbSβ(0) (sickle cell anemia) patients with and without controlling for covariates bet...

Sickle cell nephropathy (SCN) is a common complication of sickle cell disease (SCD). It has variable presentation, ranging from hyposthenuria to end-stage renal disease (ESRD). Management of ESRD in SCD patients is froth with multiple challenges which has potential to impact negatively the outcome of the patient. Kidney transplant is the preferred renal replacement therapy in these patients. Th...

This study examined the frequency and severity of sickle related pain, its impact on quality of life, and methods of coping for 25 children with sickle cell disease, aged 6-16 years. Subjects were matched with non-affected peers and asked to complete the Central Middlesex Hospital Children's Health Diary for four weeks. Results indicated that sickle pain occurred on average one in 14 days, and ...

Band 3 aggregation in the plane of the red blood cell (RBC) membrane is postulated to be important in the pathophysiology of hemolysis of dense sickle and normal RBCs. We used the fluorescence photobleaching recovery and polarized fluorescence depletion techniques to measure the lateral and rotational mobility of band 3, glycophorins, and phospholipid analogues in membranes of density-separated...

PURPOSE The purpose of this study was to characterize the impact of sickle cell disease (SCD) on oral health and examine its impact on quality of life. METHODS Fifty-four study subjects were recruited from the sickle cell clinic and 52 control subjects from the adolescent medicine clinic at Nationwide Children's Hospital, Columbus, Ohio. A dental exam was performed to determine each participa...

This review addresses several areas of concern in the care of patients with sickle cell disease. In Sections I and II, the fundamental pathogenetic mechanisms of sickle cell disease and their clinical consequences are discussed. Dr. Narla presents the evidence for abnormal cell adhesiveness by SS cells and Dr. Rosse examines the role of the increased whole blood viscosity. In Section III, Dr. P...