Precipitating factors and targeted therapies in combating the perils of sickle cell disease--- A special nutritional con
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REVIEW
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Precipitating factors and targeted therapies in combating the perils of sickle cell disease— A special nutritional consideration Shahida A. Khan1*, Ghazi Damanhouri1, Ashraf Ali1, Sarah A. Khan2, Aziz Khan1, Ahmed Bakillah3, Samy Marouf4,7, Ghazi Al Harbi5, Saeed H. Halawani6 and Ahmad Makki1
Abstract Nutritional research in sickle cell disease has been the focus in recent times owing to not only specific nutritional deficiencies, but also the improvements associated with less painful episodes. Though hydroxyurea remains the drug of choice, certain adverse health effects on long term supplementation makes room for researches of different compounds. Macro and micro nutrient deficiencies, along with vitamins, play an important role in not only meeting the calorific needs, but also reducing clinical complications and growth abnormalities. Symptoms of hyper protein metabolism, increased cell turnover, increased cardiac output, and appetite suppression due to enhanced cytokine production, might give us leads for better understanding of the mechanisms involved. Different nutritional approaches comprising of traditional herbal therapies, antioxidants, flavonoids, vitamins, minerals etc., reducing oxidative stress and blood aggregation, have been tried out to increase the health potential. Nutritional therapies may also serve complementary to the newer therapies using ozone, hematopoietic stem cell transplantation, antifungal medications, erythropoietin etc. Herein we try to present a holistic picture of the different patho-physiological mechanisms, and nutritional strategies adopted. Keywords: Sickle cell disease, Hydroxyurea, Vaso-occlusive crisis, Nutrient deficiencies, Nutritional approaches Abbreviations: ACS, Acute chest syndrome; DHA, Docosahexaenoic acid; EPA, Eicosapentaenoic acid; HbF, Fetal hemoglobin; HSCT, Hematopoietic stem cell transplantation; HU, Hydroxyurea; IL, Interleukins; NO, Nitric Oxide; REE, Resting energy expenditure; ROS, Reactive oxygen species; SCD, Sickle cell disease; VOC, Vaso occlusive crisis
Background Sickle cell disease (SCD) one of the commonest prevalent autosomal recessive disease around the globe [1], is an inherited hematological disorder wherein, the oxygen-carrying molecule namely hemoglobin (Hb) present in the red blood cell, is defective. A mutation in the in the 6th codon of the 11th chromosome of the β globin chain, renders the amino acid valine to be substituted by glutamic acid resulting in HbS, the sickle cell haemoglobin [2]. * Correspondence: [email protected]; [email protected] 1 King Fahd Medical Research Center, King Abdulaziz University, P.O. Box 80216, Jeddah 21589, Kingdom of Saudi Arabia Full list of author information is available at the end of the article
The deoxygenated HbS undergoes hydrophobic interactions to form rod-like structures which are clusters of hemoglobin protein stuck to each other. These long fibers push the cell membrane out of shape causing the whole cell to become rigid,take a sickle shape, become deformed and
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