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 ...

Background: Sickle cell anemia is relatively common in Khuzestan province located in Southwest Iran. The characteristics of sickle cell disease in Iran are apparently different from other regions some of these characteristics might be related to β-chain haplotypes. The purpose of this study was to determine the frequency of β-chain haplotypes in 50 patients with homozygous sickle cell anemia in...

Background: A low level of L-glutamine, a precursor of nicotinamide adenine dinucleotide (NAD) in red blood cells (RBCs), is identified as an underlying mechanism for the potential decrement of the NAD redox and the incidence of pain crisis in sickle cell anemia (SCA). The aim of this study is to assess the impact of oral L-glutamine therapy on pain crisis reduction in patients with SCA and sic...

ABSTRACT A Case Control Study: The Psychosocial Functioning and Academic Achievement in Siblings with and without Sickle Cell Disease Lisa Thaniel, MSW Phyllis Solomon, Ph.D. Objective: The aim of this study was to compare the psychosocial functioning and academic achievement in siblings with and without sickle cell disease. Methods: Using convenience sampling, we recruited (N=133) 45 siblings ...

background: there are many parameters that modulate the severity of sickle cell anemia. fetal hemoglobin (hb f) is one of these major variables. however, its effect is clinically inconsistent. we conducted a descriptive study to assess the influence of hb f on clinical events and hematological variables in patients with sickle cell anemia. methods: 151 patients with sickle cell anemia with a st...

Chronic nephropathy is a recognized complication of sickle cell disease. Using a transgenic sickle mouse, we examined whether oxidative stress occurs in the sickle kidney, the origins and functional significance of such oxidant stress, and the expression of the oxidant-inducible, potentially protective gene, heme oxygenase-1 (HO-1); we also examined the expression of HO-1 in the kidney and in c...

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 ...

‘Sickle hepatopathy’ is a condition which seen in only 10% cases of sickle cell disease (SCD). Sickle hepatopathy could be due to acute crisis, sequestration, cholangiopathy, severe intrahepatic cholestasis or as result chronic blood transfusion related infection and hemosiderosis. Differentiating the cause for SCD challenging. Hepatopathy manifestation can range from mild liver function test a...

We have previously reported that sickle erythrocytes sedimenting at high specific density after gradient centrifugation exhibit increased IgG binding in vivo as compared with low-density paired samples. We have performed the present study to determine whether the opsonization of dense sickle cells in vivo could also involve autologous IgM, IgA, and complement. IgA, IgM, and complement binding i...

Hebbel and colleagues have proposed that increased adherence of sickle red cells to vascular endothelium may initiate vasoocclusive events in sickle cell disease. We have developed a micropipette technique to obtain direct, quantitative measure of the adherence of individual red cells to vascular endothelial cells. Using this technique, we found that the vast majority of sickle cells suspended ...