Sickle Cell Anemia Research - Genetics, Causes, Symptoms

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.


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Improvements in haemolysis and indicators of erythrocyte survival do not correlate with acute vaso-occlusive crises in patients with sickle cell disease: a phase III randomized, placebo-controlled, double-blind study of the Gardos channel blocker senicapoc (ICA-17043).

Ataga KI, Reid M, Ballas SK, Yasin Z, Bigelow C, James LS, Smith WR, Galacteros F, Kutlar A, Hull JH, Stocker JW,

University of North Carolina, Chapel Hill, NC, USA.

Red blood cell (RBC) hydration is regulated in part by the Ca(2+) -activated K(+) efflux (Gardos) channel. Senicapoc selectively blocks potassium efflux through the Gardos channel, reducing RBC dehydration and haemolysis, and increasing haemoglobin levels in sickle cell disease (SCD). This randomized, placebo-controlled trial was designed to determine the safety and clinical efficacy of senicapoc in SCD patients. One hundred and forty-five patients were randomized to receive senicapoc and 144 patients to receive placebo for 52 weeks. Consistent with a previous study, patients in the senicapoc group had significantly increased haematocrit, haemoglobin, and decreased numbers of both dense erythrocytes and reticulocytes when compared to the placebo group. The unblinded Data Monitoring Committee terminated this study early due to a lack of efficacy when it determined that, despite improvements in anaemia and haemolysis, no significant improvement in the rate of sickle cell painful crises was observed in patients treated with senicapoc compared to those on placebo (0·38 vs. 0·31, respectively). Comparisons of the times to first, second and third crises between the senicapoc and placebo groups were not statistically significant. Nausea and urinary tract infections occurred more frequently in the senicapoc group than placebo. Serious adverse events were similar in the two groups.

Published 11 March 2011 in Br J Haematol, 153(1): 92-104.
Full-text of this article is available online (may require subscription).


Articles on Sickle Cell Anemia published 11 March 2011:

Soluble CD163 levels in children with sickle cell disease.   Br J Haematol, 153(1): 105-10.

Sickle cell disease (SCD) is characterized by vasculopathy, which has been causally linked to intravascular haemolysis and high levels of free plasma haemoglobin. Soluble CD163 (sCD163) is implicated in the clearance of free plasma haemoglobin and high plasma concentrations have been linked to arterial disease. We therefore investigated the value of sCD163 as a biomarker in children with SCD, and also measured haptoglobin levels in this population. We measured sCD163 in 25 control children with ... [Abstract] [Full-text]


Articles on Sickle Cell Anemia published 2 March 2011:

Nitric oxide for inhalation in the acute treatment of sickle cell pain crisis: a randomized controlled trial.   JAMA, 305(9): 893-902.

[Abstract] [Full-text]

Nitric oxide for inhalation in the acute treatment of sickle cell pain crisis: a randomized controlled trial.   JAMA, 305(9): 893-902.

[Abstract] [Full-text]

Nitric oxide for inhalation in the acute treatment of sickle cell pain crisis: a randomized controlled trial.   JAMA, 305(9): 893-902.

[Abstract] [Full-text]


Articles on Sickle Cell Anemia published 23 February 2011:

Attenuated maximal muscle strength and peak power in children with sickle cell disease.   J Pediatr Hematol Oncol, 33(2): 93-7.

Dominant hand maximal handgrip strength evaluated with a handgrip dynamometer and peak power evaluated with a force plate, adjusted for body size and composition, were compared in African-American children aged 5 to 13 years, with and without type SS sickle cell disease (SCD-SS). Children with SCD-SS (n = 35; age, 9.0 ± 2.0 y) compared with healthy control children (n = 103; age, 8.6 ± 1.8 y) did not differ by age, sex, or pubertal status, yet had significantly lower Z scores for height, ... [Abstract] [Full-text]


Articles on Sickle Cell Anemia published 14 February 2011:

Increase in invasive Streptococcus pneumoniae infections in children with sickle cell disease since pneumococcal conjugate vaccine licensure.   J Pediatr, 158(3): 505-7.

Invasive pneumococcal disease (IPD) in children with sickle cell disease has decreased with prophylactic penicillin, pneumococcal polysaccharide vaccine, and pneumococcal protein-conjugate vaccine usage. We report 10 IPD cases since pneumococcal protein-conjugate vaccine licensure, including a recent surge of non-vaccine serotypes. IPD continues to be a serious risk in sickle cell disease. [Abstract] [Full-text]


Articles on Sickle Cell Anemia published 7 February 2011:

Neurocognitive screening with the Brigance preschool screen-II in 3-year-old children with sickle cell disease.   Pediatr Blood Cancer, 56(4): 620-4.

[Abstract] [Full-text]

Neurocognitive screening with the Brigance preschool screen-II in 3-year-old children with sickle cell disease.   Pediatr Blood Cancer, 56(4): 620-4.

[Abstract] [Full-text]


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Sickle Cell Anemia Research Today Archive:

Volume 1 (2006)
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Sickle Cell Anemia Books

Sickle Cell Anemia - A Medical Dictionary, Bibliography, and Annotated Research Guide to Internet References

Sickle Cell Anemia - A Medical Dictionary, Bibliography, and Annotated Research Guide to Internet References