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      Precipitating factors and targeted therapies in combating the perils of sickle cell disease--- A special nutritional consideration

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          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.

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          Most cited references116

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          International consensus report on the investigation and management of primary immune thrombocytopenia.

          Previously published guidelines for the diagnosis and management of primary immune thrombocytopenia (ITP) require updating largely due to the introduction of new classes of therapeutic agents, and a greater understanding of the disease pathophysiology. However, treatment-related decisions still remain principally dependent on clinical expertise or patient preference rather than high-quality clinical trial evidence. This consensus document aims to report on new data and provide consensus-based recommendations relating to diagnosis and treatment of ITP in adults, in children, and during pregnancy. The inclusion of summary tables within this document, supported by information tables in the online appendices, is intended to aid in clinical decision making.
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            Nitric oxide synthase generates superoxide and nitric oxide in arginine-depleted cells leading to peroxynitrite-mediated cellular injury.

            Besides synthesizing nitric oxide (NO), purified neuronal NO synthase (nNOS) can produce superoxide (.O2-) at lower L-Arg concentrations. By using electron paramagnetic resonance spin-trapping techniques, we monitored NO and .O2- formation in nNOS-transfected human kidney 293 cells. In control transfected cells, the Ca2+ ionophore A23187 triggered NO generation but no .O2- was seen. With cells in L-Arg-free medium, we observed .O2- formation that increased as the cytosolic L-Arg levels decreased, while NO generation declined. .O2- formation was virtually abolished by the specific NOS blocker, N-nitro-L-arginine methyl ester (L-NAME). Nitrotyrosine, a specific nitration product of peroxynitrite, accumulated in L-Arg-depleted cells but not in control cells. Activation by A23187 was cytotoxic to L-Arg-depleted, but not to control cells, with marked lactate dehydrogenase release. The cytotoxicity was largely prevented by either superoxide dismutase or L-NAME. Thus, with reduced L-Arg availability NOS elicits cytotoxicity by generating .O2- and NO that interact to form the potent oxidant peroxynitrite. Regulating arginine levels may provide a therapeutic approach to disorders involving .O2-/NO-mediated cellular injury.
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              Updated systematic review of tonsillectomy and adenoidectomy for treatment of pediatric obstructive sleep apnea/hypopnea syndrome.

              Perform an updated systematic review and meta-analysis to determine the cure rate of tonsillectomy and adenoidectomy (T&A) for pediatric obstructive sleep apnea/hypopnea syndrome (OSAHS). A systematic review was performed to identify English-language studies that evaluate the treatment of pediatric (age < 20 years) OSAHS patients with T&A using polysomnography as a metric of cure. Twenty-three studies fit the inclusion criteria and a meta-analysis was performed to determine the overall success. Meta-analysis was also performed to determine the success in obese and comorbid populations vs cohorts of healthy children. The meta-analysis included 1079 subjects (mean sample size of 42 patients) with a mean age of 6.5 years. The effect measure was the percentage of pediatric patients with OSAHS who were successfully treated (k = 22 studies) with T&A based on preoperative and postoperative PSG data. Random-effects model estimated the treatment success of T&A was 66.3 percent, when cure was defined per each individual study. When "cure" was defined as an apnea-hypopnea index (AHI) of <1 (k = 9 studies), random-effects model estimate for OSAHS treatment success with T&A was 59.8 percent. Postoperative mean AHI was significantly decreased from preoperative levels. Contrary to popular belief, meta-analysis of current literature demonstrates that pediatric sleep apnea is often not cured by T&A. Although complete resolution is not achieved in most cases, T&A still offers significant improvements in AHI, making it a valuable first-line treatment for pediatric OSAHS.
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                Author and article information

                Contributors
                sakhan01@kau.edu.sa , shahidakhan2009@gmail.com
                gdamanhouri@gmail.com
                ashrafbiochem@gmail.com
                khan.sarah.aziz@gmail.com
                azizkfmrc@gmail.com
                Ahmed.Bakillah@downstate.edu
                samy_marouf@hotmail.com
                Alharbi98@hotmail.com
                shhalawani@uqu.edu.sa
                ahmedmakki1234@gmail.com
                Journal
                Nutr Metab (Lond)
                Nutr Metab (Lond)
                Nutrition & Metabolism
                BioMed Central (London )
                1743-7075
                8 August 2016
                8 August 2016
                2016
                : 13
                : 50
                Affiliations
                [1 ]King Fahd Medical Research Center, King Abdulaziz University, P.O. Box 80216, Jeddah, 21589 Kingdom of Saudi Arabia
                [2 ]National Brain Research Center, Manesar, Gurgaon, 122051 India
                [3 ]Department of Medicine, SUNY Downstate Medical Center, 450 Clarkson Ave., Brooklyn, New York 11203 United State of America (USA)
                [4 ]Department of Hematology, King Fahd Hospital of the Armed forces, Jeddah, Kingdom of Saudi Arabia
                [5 ]Department of Hematology, Soliman Fakeeh Hospital Jeddah, Jeddah, Kingdom of Saudi Arabia
                [6 ]Department of Hematology, Umm Al Qura University, Faculty of Medicine, Makkah, Kingdom of Saudi Arabia
                [7 ]Department of Medical Laboratory, King Fahd Hospital of the Armed forces, Jeddah, Kingdom of Saudi Arabia
                Article
                109
                10.1186/s12986-016-0109-7
                4977632
                27508000
                08e04b4b-dfe3-4325-b725-b91c88f4e4f9
                © The Author(s). 2016

                Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License ( http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

                History
                : 20 March 2016
                : 22 July 2016
                Funding
                Funded by: FundRef http://dx.doi.org/10.13039/501100004919, King Abdulaziz City for Science and Technology;
                Award ID: AT35-25
                Award Recipient :
                Categories
                Review
                Custom metadata
                © The Author(s) 2016

                Nutrition & Dietetics
                sickle cell disease,hydroxyurea,vaso-occlusive crisis,nutrient deficiencies,nutritional approaches

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