Iron Overload in Sickle Cell Disease

Raghupathy, Radha; Manwani, Deepa; Little, Jane A.

doi:10.1155/2010/272940

Cited by 59 publications

(55 citation statements)

References 98 publications

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“…Mechanism of end organ damage in iron overload. [8] Cholelithiasis Cholelithiasis is one of the common complications of SCA. These are usually pigment stones that result from chronic haemolysis leading to increased bilirubin production.…”

Section: Hepatomegalymentioning

confidence: 99%

Study of Hepatobiliary Involvement in Children with Sickle Cell Disease

Bokade¹,

Chauhan²,

Dhole³

2017

Ann. Int. Med. Den.Res.

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Section: Hepatomegalymentioning

confidence: 99%

Study of Hepatobiliary Involvement in Children with Sickle Cell Disease

Bokade¹,

Chauhan²,

Dhole³

2017

Ann. Int. Med. Den.Res.

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“…Importantly, in thalassemia patients, curcumin reduced oxidative stress [102]. Thalassemia often co-exists with SCD [103], and increased iron in the tissues due to hemolysis is a characteristic feature of SCD [104]. Therefore, these dietary supplements may provide an advantage in treating sickle pathobiology and pain without the inadvertent side efects of pharmacologics discussed above.…”

Section: Integrative Approachesmentioning

confidence: 99%

Mechanisms of Pain in Sickle Cell Disease

Aich¹,

Beitz²,

Gupta³

2016

Sickle Cell Disease - Pain and Common Chronic Complications

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Pain is one of the most common features of sickle cell disease SCD lacking efective therapy. Pain in SCD is relatively more complicated than other conditions associated with pain requiring understanding of the pathobiology of pain speciic to SCD. The characterization of pain to deine the diverse modalities of nociception in SCD is currently under progress via human studies accompanied by transgenic mouse models of SCD. Sickle pathobiology characterized by oxidative stress, inlammation and vascular dysfunction contributes to both peripheral and central nociceptive sensitization via mast cell activation in the periphery, and reactive oxygen species and glial activation and endoplasmic reticulum stress in the spinal cord among other efectors. These efects are mediated via several cellular receptors, which can be targeted to produce positive therapeutic outcomes. In this chapter, we will discuss the present understanding of molecular mechanisms of SCD pain and outline the mechanism-based translational potential of novel actionable targets to treat SCD pain.Keywords: pain, sickle cell disease, neurogenic inlammation, substance P, mast cell . IntroductionPain is a hallmark feature of sickle cell disease SCD , which can start in infancy, leading to hospitalization, reduced survival and poor quality of life. Pain in SCD is unique because of unpredictable and recurrent episodes of acute pain due to vaso-occlusive crises VOC , in addition to chronic pain experienced by a majority of adult patients on a daily basis [1]. Treatment choices remain limited to opioids, which impose liabilities of their own including constipation, mast cell activation, fear of addiction and respiratory depression [2]. Moreover, signiicantly larger doses of opioids are required to treat pain in SCD as compared to other acute and chronic pain conditions [1]. Pain can be lifelong in SCD and may therefore inluence © 2016 The Author(s). Licensee InTech. This chapter is distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.© 2016 The Author(s). Licensee InTech. This chapter is distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.cognitive function and lead to depression and anxiety, which can in turn promote the perception of pain [1].Treatment of chronic pain remains unsatisfactory overall, perhaps due to the diverse pathobiology in diferent diseases. Therefore, it is critical to understand the mechanisms speciic to the genesis of sickle pain to develop targeted therapies. Vascular dysfunction, inlammation, ischemia/reperfusion injury and oxidative stress in the wake of VOC can each evoke activation of the nociceptive nerve ibers leading to acute pain. On the other hand, con...

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“…2 After adjusting for ferritin level, lung involvement, hyperhemolysis and AHI, the finding of a high PLM index in the priapism group was surprising, considering that increased ferritin and iron overload were described in patients with SCD. 20 Although iron supplementation decreases PLM in patients with RLS, 21 the association of low body iron stores and low ferritin levels with PLM is less clear. 22 In contrast, activation of the hypoxia pathway may contribute to cellular iron deficiency and predispose individuals to PLM and RLS.…”

Section: Discussionmentioning

confidence: 99%