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      A Phase 3 Trial of l-Glutamine in Sickle Cell Disease

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          Abstract

          Oxidative stress contributes to the complex pathophysiology of sickle cell disease. Oral therapy with pharmaceutical-grade l-glutamine (USAN, glutamine) has been shown to increase the proportion of the reduced form of nicotinamide adenine dinucleotides in sickle cell erythrocytes, which probably reduces oxidative stress and could result in fewer episodes of sickle cell-related pain.

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          Causes and outcomes of the acute chest syndrome in sickle cell disease. National Acute Chest Syndrome Study Group.

          E P Vichinsky, L Neumayr, A Earles … (2000)
          The acute chest syndrome is the leading cause of death among patients with sickle cell disease. Since its cause is largely unknown, therapy is supportive. Pilot studies with improved diagnostic techniques suggest that infection and fat embolism are underdiagnosed in patients with the syndrome. In a 30-center study, we analyzed 671 episodes of the acute chest syndrome in 538 patients with sickle cell disease to determine the cause, outcome, and response to therapy. We evaluated a treatment protocol that included matched transfusions, bronchodilators, and bronchoscopy. Samples of blood and respiratory tract secretions were sent to central laboratories for antibody testing, culture, DNA testing, and histopathological analyses. Nearly half the patients were initially admitted for another reason, mainly pain. When the acute chest syndrome was diagnosed, patients had hypoxia, decreasing hemoglobin values, and progressive multilobar pneumonia. The mean length of hospitalization was 10.5 days. Thirteen percent of patients required mechanical ventilation, and 3 percent died. Patients who were 20 or more years of age had a more severe course than those who were younger. Neurologic events occurred in 11 percent of patients, among whom 46 percent had respiratory failure. Treatment with phenotypically matched transfusions improved oxygenation, with a 1 percent rate of alloimmunization. One fifth of the patients who were treated with bronchodilators had clinical improvement. Eighty-one percent of patients who required mechanical ventilation recovered. A specific cause of the acute chest syndrome was identified in 38 percent of all episodes and 70 percent of episodes with complete data. Among the specific causes were pulmonary fat embolism and 27 different infectious pathogens. Eighteen patients died, and the most common causes of death were pulmonary emboli and infectious bronchopneumonia. Infection was a contributing factor in 56 percent of the deaths. Among patients with sickle cell disease, the acute chest syndrome is commonly precipitated by fat embolism and infection, especially community-acquired pneumonia. Among older patients and those with neurologic symptoms, the syndrome often progresses to respiratory failure. Treatment with transfusions and bronchodilators improves oxygenation, and with aggressive treatment, most patients who have respiratory failure recover.
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            Semiparametric regression for the mean and rate functions of recurrent events

            L Wei, I. Yang, Z Ying … (2000)
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              Role of oxidative stress in the pathogenesis of sickle cell disease.

              Erica N Chirico, Vincent Pialoux (2012)
              Sickle cell disease (SCD) is a class of hemoglobinopathy in humans, which causes a disruption of the normal activities in different systems. Although this disease begins with the polymerization of red blood cells during its deoxygenating phase, it can erupt into a cascade of debilitating conditions such as ischemia-reperfusion injury, inflammation, and painful vaso-occlusion crises. The purpose of this review is to discuss how these phenomena can result in the formation of oxidative stress as well as limit nitric oxide (NO) bioavailability and decrease antioxidant status. The cumulative effects of these traits cause an increase in other forms of reactive oxygen species (ROS), which in turn intensify the symptoms of SCD and generate a vicious circle. Finally, we will discuss antioxidant therapeutic strategies that limit ROS generation and subsequently increase NO bioavailability with respect to endothelial protection in SCD. Copyright © 2011 Wiley Periodicals, Inc.
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                Author and article information

                Journal
                Title: New England Journal of Medicine
                Abbreviated Title: N Engl J Med
                Publisher: New England Journal of Medicine (NEJM/MMS)
                ISSN (Print): 0028-4793
                ISSN (Electronic): 1533-4406
                Publication date Created: July 19 2018
                Publication date (Print): July 19 2018
                Volume: 379
                Issue: 3
                Pages: 226-235
                Affiliations
                [1 ]From Emmaus Medical, Torrance (Y.N., L.T.T., R.L.R., C.W.S.), University of California at Los Angeles (Y.N.), Harbor–UCLA and Los Angeles BioMedical Research Institute (J.L.L., E.H.P.), and University of Southern California (C.W.S.), Los Angeles, Kaiser Permanente Medical Center, Inglewood (L.S., O.A.B.), and UCSF Benioff Children’s Hospital and Research Center, Oakland (L.D.N., E.P.V.) — all in California; State University of New York–Downstate Medical Center (S.T.M.), Brookdale University Hospital and...
                Article
                DOI: 10.1056/NEJMoa1715971
                PubMed ID: 30021096
                SO-VID: 0c4645b2-7984-479d-8f8f-e2585d4c766d
                Copyright © © 2018
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