l-Glutamine for sickle cell anemia: more questions than answers

Quinn, Charles T.

doi:10.1182/blood-2018-03-834440

Cited by 74 publications

(67 citation statements)

References 21 publications

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“…There were also significant reductions in the frequency of acute chest crises (8.6% on the l-glutamine group versus 23.1% in the placebo group, p = 0.003), and duration of hospital admissions (median 6.5 in the l-glutamine group versus 11 in the placebo group, p = 0.02) [69]. Potential concerns around use include the lack of long-term follow up data, financial cost compared with hydroxycarbamide, and theoretical concern around reducing treatment concordance with hydroxycarbamide therapy amongst patients seeking more naturalistic medication [70].…”

Section: L-glutaminementioning

confidence: 99%

Sickle Cell Disease—Genetics, Pathophysiology, Clinical Presentation and Treatment

Inusa

Hsu

Kohli

et al. 2019

IJNS

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Sickle cell disease (SCD) is a monogenetic disorder due to a single base-pair point mutation in the β-globin gene resulting in the substitution of the amino acid valine for glutamic acid in the β-globin chain. Phenotypic variation in the clinical presentation and disease outcome is a characteristic feature of the disorder. Understanding the pathogenesis and pathophysiology of the disorder is central to the choice of therapeutic development and intervention. In this special edition for newborn screening for haemoglobin disorders, it is pertinent to describe the genetic, pathologic and clinical presentation of sickle cell disease as a prelude to the justification for screening. Through a systematic review of the literature using search terms relating to SCD up till 2019, we identified relevant descriptive publications for inclusion. The scope of this review is mainly an overview of the clinical features of pain, the cardinal symptom in SCD, which present following the drop in foetal haemoglobin as young as five to six months after birth. The relative impact of haemolysis and small-vessel occlusive pathology remains controversial, a combination of features probably contribute to the different pathologies. We also provide an overview of emerging therapies in SCD.

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Section: L-glutaminementioning

confidence: 99%

Sickle Cell Disease—Genetics, Pathophysiology, Clinical Presentation and Treatment

Inusa

Hsu

Kohli

et al. 2019

IJNS

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“…L‐glutamine is an antioxidant that was approved by the Food and Drug Administration in 2017 for the treatment of sickle cell anemia . In the enzymatic antioxidant system, glutathione (GSH) and superoxide dismutase (SOD) are the most important antioxidants which work together to counteract oxidative stress in cells and protect brain from ischemia‐reperfusion damage.…”

Section: Introductionmentioning

confidence: 99%

“…L‐glutamine is involved in nitrogen transport, regulation of acid‐base homeostasis, and catabolic signaling . It is also a substrate for glutathione synthesis, basic building block for proteins, and a potential inhibitory agent for inflammatory cytokine release .…”

Section: Introductionmentioning

confidence: 99%

L‐glutamine protects mouse brain from ischemic injury via up‐regulating heat shock protein 70

Luo

Shan

et al. 2019

CNS Neurosci Ther

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Introduction L‐glutamine is an antioxidant that plays a role in a variety of biochemical processes. Given that oxidative stress is a key component of stroke pathology, the potential of L‐glutamine in the treatment of ischemic stroke is worth exploring. Aims In this study, we investigated the effect and mechanisms of action of L‐glutamine after cerebral ischemic injury. Results L‐glutamine reduced brain infarct volume and promoted neurobehavioral recovery in mice. L‐glutamine administration increased the expression of heat‐shock protein 70 (HSP70) in astrocytes and endothelial cells. Such effects were abolished by the coadministration of Apoptozole, an inhibitor of the ATPase activity of HSP70. L‐glutamine also reduced oxidative stress and neuronal apoptosis, and increased the level of superoxide dismutase, glutathione, and brain‐derived neurotrophic factor. Cotreatment with Apoptozole abolished these effects. Cell culture study further revealed that the conditioned medium from astrocytes cultured with L‐glutamine reduced the apoptosis of neurons after oxygen‐glucose deprivation. Conclusion L‐glutamine attenuated ischemic brain injury and promoted functional recovery via HSP70, suggesting its potential in ischemic stroke therapy.

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“…27 In 2017, the FDA approved pharmaceutical grade L-glutamine for children and adults with sickle cell disease. For individuals >5 years of age with repeated VOC pain events, oral L-glutamine at a dose of 0.3 g/kg twice per day is recommended, with a maximum daily dose of 30 g. 28 L-Histidine, leucine, valine, and cysteine are also insufficient in SCD subjects. 29 D 3) in fatty fish, cheese, egg yolks, and liver.…”

Section: Glutaminementioning

confidence: 99%

<p>Nutrition in sickle cell disease: recent insights</p>

Umeakunne¹,

Hibbert²

2019

NDS

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A cure for sickle cell anemia (SCA) is not available to all who have inherited this devastating genetically inherited disease. However, increasing knowledge that nutritional problems are fundamental to the severity of the disease, has produced interest in promoting dietary supplementation for treating these patients. This review seeks to emphasize the understanding that both children and adults with sickle cell disease require much higher energy and protein consumption (more macronutrient intake) than healthy individuals and tend to suffer from undernutrition if energy intake is consistently low. Shortages may also exist for micronutrients, eg, Glutathione, which has both anti-inflammatory and anti-oxidant properties. Both chronic inflammation and oxidant stress are central issues for increased sickle cell disease severity. In conclusion, dedicating more effort and resources to establishing recommended dietary reference intakes (DRIs)/recommended dietary allowances (RDAs) for SCA patients is essential, and nutritional intervention should be included as an adjunct treatment in tandem with standard practice.

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l-Glutamine for sickle cell anemia: more questions than answers

Cited by 74 publications

References 21 publications

Sickle Cell Disease—Genetics, Pathophysiology, Clinical Presentation and Treatment

Sickle Cell Disease—Genetics, Pathophysiology, Clinical Presentation and Treatment

L‐glutamine protects mouse brain from ischemic injury via up‐regulating heat shock protein 70

<p>Nutrition in sickle cell disease: recent insights</p>

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