scholarly journals L-glutamine for sickle cell disease: Knight or pawn?

2020 ◽  
Vol 245 (2) ◽  
pp. 146-154 ◽  
Author(s):  
Alina Sadaf ◽  
Charles T Quinn

Oxidative stress is an important contributor to the pathophysiology of sickle cell disease. The pathways involved are complex and interlinked. L-glutamine is an amino acid with myriad roles in the body, including the synthesis of antioxidants, such as reduced glutathione and the cofactors NAD(H) and NADP(H), as well as nitric oxide—so it has therapeutic potential as an antioxidant. However, the relative impact of L-glutamine on the redox environment in red blood cells in sickle cell disease is not fully understood, and there are few therapeutic trials in sickle cell disease. Following the FDA approval of L-glutamine for sickle cell disease, more research is still needed to understand its clinical effects and role in therapy. Impact statement L-glutamine has been recently approved by the FDA for the prevention of acute complications in sickle cell disease (SCD). However, there are many gaps in our understanding of the biologic role of glutamine and its therapeutic implications in SCD. This review summarizes the pre-clinical and clinical evidence that can inform clinical decision-making and future research on glutamine therapy in SCD patients.

2021 ◽  
Vol 2 (3) ◽  
pp. 9-17
Author(s):  
Dafni Eleftherou ◽  
Aristidis Arhakis ◽  
Sotiria Davidopoulou

Aim: This literature review aims to update the evidence for orofacial manifestations and current treatment recommendations for children and adolescents with sickle cell disease. Background: Sickle cell disease is a frequent hemoglobinopathy and a life-threatening genetic disorder. The lifelong condition is characterized by chronic hemolytic anemia and vaso-occlusive crisis that may occur in a variable range of clinical presentations in different regions of the body, including the oral cavity. Review results: This review explored the most common orofacial alterations of pediatric patients with SCD. Dental caries is a common finding in SCD pediatric patients, especially in those who are socio-economically vulnerable. Moreover, malocclusions occur in high prevalence in SCD pediatric patients. Other oral health complications seen in SCD patients include periodontal inflammation, bone changes, infections, mental nerve neuropathy, facial overgrowth, delayed tooth eruption, dental anomalies, pulp necrosis, soft tissue alterations and salivary changes. Dental infections may trigger a vaso-occlusive crisis leading the patient to a higher probability on arriving in hospital emergency departments and in need for further hospital admission to deal with the correlated complications. Thus, preventive dental care and non-invasive dental procedures are the principal focus in SCD patients in order to avoid possible subsequent complications. Conclusion: The review showed that in pediatric patients with SCD the risk for orofacial manifestations and complications depends not only on the presence of SCD but also on other confounding factors such as oral hygiene, diet habits and social conditions. Moreover, more well-designed epidemiological studies are necessary to assess the real link between SCD disease and its impact on stomatognathic health.


2002 ◽  
Vol 140 (6) ◽  
pp. 681-687 ◽  
Author(s):  
Dorothy J. VanderJagt ◽  
Paul Harmatz ◽  
Ajovi B. Scott-Emuakpor ◽  
Elliot Vichinsky ◽  
Robert H. Glew

2020 ◽  
pp. 112067212095761
Author(s):  
Oluwatoyin I. Oladimeji ◽  
Oluwagbemiga O. Adeodu ◽  
Oluwatoyin H. Onakpoya ◽  
Samuel A. Adegoke

Introduction: Sickle cell disease (SCD) ranks high among genetic disorders worldwide. It is characterised by repeated vaso-occlusion with resultant end-organ damage. This process can occur in all vascular beds in the body, including ocular blood vessels and may cause irreversible blindness in advanced stages. Little is known of the relationship between the prevalence of ocular abnormalities among children with SCD and their disease severity. Methods: A descriptive cross-sectional study was carried out at the Paediatric Haematology Clinics and the Eye Centre of the Obafemi Awolowo University Teaching Hospitals Complex (OAUTHC), Ile-Ife. Children with SCD in steady state were recruited from the Haematology Clinics and examined for ocular abnormalities at the Eye Centre of the hospital. The subjects SCD severity grade was determined using a previously validated scoring system. Results: One hundred and twenty (120) children aged 5 to 15 years were examined. Of these, 72 had one or more ocular abnormalities giving the prevalence of ocular abnormalities among them to be 60.0%. Though a higher proportion of children with moderate disease, 23 (65.7%) of 35, compared to those with mild disease, 49 (57.6%) of 85 had ocular abnormalities, this difference was not statistically significant, p = 0.412. Conclusion: Ocular abnormalities among Nigerian children with SCD are common even in steady-state, but not significantly associated with disease severity. Periodic screening for ocular abnormalities should thus be done on them irrespective of disease severity.


Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 2576-2576 ◽  
Author(s):  
Richard J. Labotka ◽  
Robert E. Molokie ◽  
A. Kyle Mack ◽  
Alexis A Thompson ◽  
Young Ok Kim ◽  
...  

Abstract Abstract 2576 Poster Board II-553 Pain remains the most frustrating and debilitating symptom of sickle cell disease (SCD). Yet, because pain is a perception of a sensation, objective assessment of pain remains elusive. In order to achieve adequate pain control, it is imperative that the patient be able to effectively communicate with the physician regarding the extent, severity and quality of the pain, as well as the response of the pain to the therapeutic intervention. Moreover, SCD pain may have two components, nociceptive (due to organ/tissue injury) and neuropathic (due to somatosensory system lesion or disease), with differing pathophysiologies, and differing responses to pain medications. PAINReportIt® is a multi-dimensional computerized, self-assessment pain reporting tool based on the McGill Pain Questionnaire (MPQ). The MPQ has been validated for adolescents and adults. However, the PAINReportIt®, which was developed originally for the evaluation of cancer pain, has not previously been used in adolescents, nor in the study of adolescents' SCD pain. The purpose of this study was to examine the frequency with which pain experienced in various body areas by adolescents and young adults had characteristics consistent with nociceptive and neuropathic pain. Methods. Inclusion criteria included an SCD diagnosis (SS, SC or S-beta thal) and age at least 14 years. Patients attending two pediatric/adolescent comprehensive sickle cell clinics were invited to participate. Consenting subjects were first instructed and then allowed to complete the PAINReportIt® tool, in which they marked their painful sites on a graphic body outline, selected pain quality descriptors from word lists provided by the computer, and for each site they matched each site to the pain quality descriptors that represented the site. The descriptors included sensory descriptors that are known to be characteristic of either neuropathic pain or nociceptive pain. Results. PAINReportIt® tools were completed by 49 SCD subjects, whose ages ranged from 14 to 27 years (mean, 18 +/− 2.6 years). For analysis, the body was divided into nine segments, and the computer analyzed the marked painful body outline sites and identified the body segments involved. Descriptive statistics were used to determine the frequency for which each body segment included a painful site, and the frequencies for which that site was characterized as having neuropathic or nociceptive pain qualities, or both. These results are summarized in the Table below. As the Table shows, virtually all body segments were frequently involved with pain, the upper back and legs being the most frequently reported, and the lower back the least (Column 1). For body segments reported as painful, few were reported as having only nociceptive (Column 2) or neuropathic (Column 3) pain qualities. The majority of painful sites were described by subjects as having mixed pain qualities (Column 4). As Column 5 shows, from 76% to 100% of all painful sites were characterized as having a neuropathic pain component. When the number of descriptors associated with each site was reviewed (data not shown), the right leg was matched to the largest number of neuropathic descriptors (Aching, Burning, Cold, Drilling, Flickering, Numb, Penetrating, Radiating, Shooting, Spreading, Tight, Tingling). The upper back was matched to the largest number of nociceptive descriptors (Beating, Cramping, Crushing, Gnawing, Hurting, Piercing, Pounding, Pressing, Pulsing, Sharp, Sore, Splitting, Squeezing, Tender, Throbbing). Conclusions. When utilizing a computer-based self-reporting pain tool, SCD patients overwhelmingly describe a neuropathic component to their pain as well as a nociceptive component. The high frequency of neuropathic pain has been underappreciated, and this may contribute to the difficulty in managing sickle cell pain, since this pain component is not well controlled by opioid analgesics. Disclosures: No relevant conflicts of interest to declare.


Anemia ◽  
2015 ◽  
Vol 2015 ◽  
pp. 1-6 ◽  
Author(s):  
Arch G. Mainous ◽  
Rebecca J. Tanner ◽  
Christopher A. Harle ◽  
Richard Baker ◽  
Navkiran K. Shokar ◽  
...  

Objective. Sickle cell disease (SCD) is a disease that requires a significant degree of medical intervention, and family physicians are one potential provider of care for patients who do not have access to specialists. The extent to which family physicians are comfortable with the treatment of and concerned about potential complications of SCD among their patients is unclear. Our purpose was to examine family physician’s attitudes toward SCD management.Methods. Data was collected as part of the Council of Academic Family Medicine Educational Research Alliance (CERA) survey in the United States and Canada that targeted family physicians who were members of CERA-affiliated organizations. We examined attitudes regarding management of SCD.Results. Overall, 20.4% of respondents felt comfortable with treatment of SCD. There were significant differences in comfort level for treatment of SCD patients depending on whether or not physicians had patients who had SCD, as well as physicians who had more than 10% African American patients. Physicians also felt that clinical decision support (CDS) tools would be useful for treatment (69.4%) and avoiding complications (72.6%) in managing SCD patients.Conclusions. Family physicians are generally uncomfortable with managing SCD patients and recognize the utility of CDS tools in managing patients.


Sci ◽  
2020 ◽  
Vol 2 (4) ◽  
pp. 89
Author(s):  
Mathias Abiodun Emokpae ◽  
Emmanuel Bamidele Fatimehin

Sickle cell disease (SCD) is an inherited disorder and a major health challenge in Nigeria. Micronutrient deficiencies often associated with the disorder may cause inflammation and abnormal metabolism in the body. The copper-to-zinc ratio is a more relevant diagnostic measure than the concentration of either metal alone in clinical practice. This study seeks to evaluate serum levels of c-reactive protein (CRP), copper, zinc and the copper-to-zinc ratio, and to correlate the latter with CRP in adult subjects with SCD. Serum copper, zinc, CRP and plasma fibrinogen were assayed in 100 confirmed SCD patients in steady clinical state and 100 age- and sex-matched subjects with normal hemoglobin. Serum copper and zinc were assayed by the colorimetric method using reagents supplied by Centronic, Germany, while CRP and fibrinogen were assayed using reagents supplied by Sigma (St. Louis, MO, USA) and Anogen (Ontario, Canada), respectively. The copper-to-zinc ratio was calculated from serum levels of copper and zinc. The measured parameters were compared between the groups using the Students t-test, and the Pearson correlation coefficient was used to relate CRP with the other parameters. Serum copper, CRP, fibrinogen and the copper-to-zinc ratio were significantly higher (p < 0.001), while zinc level was lower in SCD patients than in controls. Serum CRP concentration correlated with copper (r = 0.10; p < 0.02), zinc (r = −0.199; p < 0.05) and the copper-to-zinc ratio (r = 0.312; p < 0.002), but the correlation between CRP and fibrinogen was not significant. Inflammation may modulate copper and zinc homeostasis, and the copper-to-zinc ratio may be used as a marker of nutritional deficiency and inflammation in SCD patients.


F1000Research ◽  
2015 ◽  
Vol 4 ◽  
pp. 1050 ◽  
Author(s):  
Marilyn J. Telen

Although production of hemoglobin S, the genetic defect that causes sickle cell disease (SCD), directly affects only red blood cells, the manifestations of SCD are pervasive, and almost every cell type and organ system in the body can be involved. Today, the vast majority of patients with SCD who receive modern health care reach adulthood thanks to vaccine prophylaxis and improvements in supportive care, including transfusion. However, once patients reach adulthood, they commonly experience recurrent painful vaso-occlusive crises and frequently have widespread end-organ damage and severely shortened life expectancies. Over the last several decades, research has elucidated many of the mechanisms whereby abnormal red blood cells produce such ubiquitous organ damage. With these discoveries have come new ways to measure disease activity. In addition, new pharmaceutical interventions are now being developed to address what has been learned about disease mechanisms.


PEDIATRICS ◽  
1983 ◽  
Vol 72 (1) ◽  
pp. 88-92
Author(s):  
Ugo Carpentieri ◽  
Leigh Smith ◽  
Charles W. Daeschner ◽  
Mary Ellen Haggard

Neutrophils can be distinguished as EA negative (EA-N) or EA positive (EA+N), according to rosette formation with sheep erythrocytes. EA- neutrophils show a bactericidal activity 50% to 70% lower than EA+ neutrophils. Thirty children with sickle cell disease were studied during steady state and crises/infections, together with matched control children. EA+ and EA- neutrophils, zinc levels in the body, and frequency of previous bacterial infection were evaluated. Sixty percent of the patients (18/30) had zinc deficiency (zinc &lt; 8.5 µg/1010 RBC): of this group, more than three fourths (14/18) had a high frequency of infections (≥3/yr) and most of those (11/14) also had an increased percentage of EA- neutrophils (80% to 85% v 35% to 45% in control subjects). Only 4/18 of patients with zinc deficiency had a low frequency of infections, and only one of these four had a higher percentage of EA- neutrophils. In patients with normal zinc levels (12/30 or 40%), only three had frequent infections, but only one of these had an increased percentage of EA- neutrophils. The number of EA+ neutrophils increased after stimulation with epinephrine or during crises/infections. In six patients with more pronounced zinc deficiency and more severe crisis/infection, a delay in the increase of EA+ neutrophils occurred and was corrected by treatment. These findings suggest that a higher percentage of neutrophils with less bactericidal activity in many children with sickle cell disease and zinc deficiency may be a factor in the higher incidence of infections noted in these patients, and zinc might play a role in the formation, release, and activity of neutrophils.


Sign in / Sign up

Export Citation Format

Share Document