Copper-to-Zinc Ratio Correlates with an Inflammatory Marker in Patients with Sickle Cell Disease (Version 1)

Sci ◽  
2019 ◽  
Vol 1 (2) ◽  
pp. 34
Author(s):  
Mathias Abiodun Emokpae ◽  
Emmanuel Babatunde Fatimehin

Sickle cell disease (SCD) is an inherited disorder of major health challenge in Nigeria. Micronutrients deficiencies often associated with the disorder may cause inflammation and abnormal metabolisms in the body. The copper-to-zinc ratio is a more important assessment than the concentrations of either of the metals in clinical practice. This study seeks to evaluate serum levels of c-reactive protein (CRP), copper, zinc and copper-to-zinc ratio and to correlate copper-to-zinc ratio 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 haemoglobin. Serum copper and zinc were assayed by 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 Students t-test and Pearson correlation coefficient was used to relate CRP with the other parameters. Serum copper, CRP, fibrinogen and copper-to-zinc ratio were significantly higher (p < 0.001) while zinc level was lower in SCD patients than controls. Serum CRP concentration correlated with copper (r = 0.10; p < 0.02), zinc (r = −0.199; p < 0.05) and Copper-to-zinc ratio (r = 0.312; p < 0.002) but the correlation between CRP and fibrinogen was not significant. Inflammatory condition may modulate copper and zinc homeostasis and copper-to-zinc ratio may be used as marker of nutritional deficiency and inflammation in SCD 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.


Sci ◽  
2019 ◽  
Vol 1 (2) ◽  
pp. 55
Author(s):  
Mathias Abiodun Emokpae ◽  
Emmanuel Bamidele Fatimehin

Sickle cell disease (SCD) is an inherited disorder of major health challenge in Nigeria. Micronutrients deficiencies often associated with the disorder may cause inflammation and abnormal metabolisms in the body. The copper-to-zinc ratio is a more important assessment than the concentrations of either of the metals in clinical practice. This study seeks to evaluate serum levels of c-reactive protein (CRP), copper, zinc and copper-to-zinc ratio and to correlate copper-to-zinc ratio 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 haemoglobin. Serum copper and zinc were assayed by 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 Students t-test and Pearson correlation coefficient was used to relate CRP with the other parameters. Serum copper, CRP, fibrinogen and copper-to-zinc ratio were significantly higher (p < 0.001) while zinc level was lower in SCD patients than controls. Serum CRP concentration correlated with copper (r = 0.10; p < 0.02), zinc (r = −0.199; p < 0.05) and Copper-to-zinc ratio (r = 0.312; p < 0.002) but the correlation between CRP and fibrinogen was not significant. Inflammatory condition may modulate copper and zinc homeostasis and copper-to-zinc ratio may be used as marker of nutritional deficiency and inflammation in SCD patients.


Medicina ◽  
2019 ◽  
Vol 55 (5) ◽  
pp. 180
Author(s):  
Charles Antwi-Boasiako ◽  
Gifty Dankwah ◽  
Robert Aryee ◽  
Charles Hayfron-Benjamin ◽  
Alfred Doku ◽  
...  

Background and Objectives: Altered copper and zinc homeostasis may influence the antioxidant defense system and consequently lead to oxidative stress and associated complications in sickle cell disease (SCD) patients. Iron levels have been reported to increase in sickle cell patients due to frequent blood transfusion, chronic intravenous haemolysis and increased absorption of iron from the gastrointestinal tract. These elevated levels of iron may also lead to extensive oxidative damage. The current study evaluated serum levels of iron, copper and zinc in SCD patients and “healthy” controls. Materials and Methods: The study was a cross-sectional one, comprising 90 SCD patients with Haemoglobin SS and Haemoglobin SC genotypes and 50 HbAA “healthy” controls. Serum levels of iron, copper and zinc were measured using a Flame Atomic Absorption Spectrometer (Variant 240FS manufactured by VARIAN Australia Pty Ltd, VIC, Australia). Copper and zinc ratios were calculated and analyzed. Results: Serum levels of iron and copper were significantly elevated in the SCD patients, compared to their “healthy” counterparts (p < 0.001). These levels were further increased in patients with haemoglobin SS in vaso-occlusive crises (HbSS VOCs). Serum zinc levels were, however, significantly lower in the SCD patients, particularly during vaso-occlusion. The copper-to-zinc ratio was also found to be significantly higher in the SCD patients. Conclusion: Elevated copper-to-zinc ratio may be a biomarker of sickle cell oxidative stress and associated complications. The ratio may also be informative for the management of sickle cell oxidative burden. The significantly lower levels of zinc in the SCD patients may warrant zinc supplementation.


2019 ◽  
Vol 19 (3) ◽  
pp. 2798-2805
Author(s):  
Mathias A Emokpae ◽  
Emmanuel B Fatimehin ◽  
Progress A Obazelu

Background: Micronutrient deficiency is recognized in sickle cell anaemia (SCA) but it is not known for certain whether changes in zinc, copper and copper-to-zinc ratio are associated with Sickle cell disease severity scores. Objective: To compare serum levels of copper, zinc and copper-to-zinc ratio in SCA subjects with control group and correlate the variables with objective disease severity scores. Methods: Serum copper and zinc were determined in 100 SCA patients and 50 controls using kits supplied by Centronic, Germany. Unpaired Students’t-test was used to compare the variables between SCA patients in steady clinical state, vaso-occlusive crisis and controls, while Spearman correlation coefficient was used to associate the parameters with disease severity scores. Results: Serum copper level was higher (P=0.008) in SCA patients than controls, while serum zinc level was lower (P<0.001) in SCA patients than controls. The copper/zinc ratio was higher (P<0.001) in SCA patients than controls. Significantly higher (P<0.001) copper and lower (P<0.001) zinc levels were observed in patients in vaso-occlusive crisis than in steady clinical state. Zinc correlated inversely (r=-0.2743; P=0.006) while copper-to-zinc ratio correlated positively with disease severity scores. Conclusion: Copper-to-zinc ratio may be an indicator of disease severity in SCA patients.Keywords: Copper/zinc ratio, disease severity score, sickle cell anaemia.


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 ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 3787-3787
Author(s):  
Ashley J. Duits ◽  
John B. Schnog

Abstract Angiogenesis plays a central role in neovascular processes. In sickle cell patients neovascularization is considered a common feature that is associated with complications such as retinopathy and cerebrovascular disease. Several studies have reported increased vascular growth factor levels such as vascular endothelial growth factor (VEGF) and placenta growth factor (PlGF) in small groups of patients. Recently the angiopoietin/Tie2 system has been shown to be of major importance in vessel maturation. In order to thoroughly characterize vascular growth factor profiles in sickle cell patients we analyzed serum levels of VEGF, PlGF, Angiopoietin-1, Angiopoietin-2, Tie-2 and erythropoietin (EPO) during the asymptomatic phase as well as during painful crises in HbSS patients (n=42) and compared these to levels in HbAA controls (n=30). Also, levels were compared between patients with and without a history of acute vaso-occlusive complications the year prior to sample collection. Serum Angiopoietin-2, Tie-2 and EPO levels were significantly higher in sickle cell patients as compared to controls (p&lt;0.01, p=0.03 and p&lt;0.01 respectively), whereas PlGF and VEGF serum levels were similar between patients and controls (p= 0.21 and p=0.5). Only Angiopoietin-2 and EPO serum levels were significantly increased in sickle cell patients during acute vaso-occlusive crises as compared to levels in asymptomatic patients (p&lt;0.01 and p=0.03 respectively). No differences were detected in measured parameters between patients with a history of acute vaso-occlusive complications as compared to patients without acute vaso-occlusive complications in the preceding year. Our results show a specific pattern of angiopoietins in sickle cell patients. Considering the importance of endothelial cell activation in sickle cell disease and the regulation of endothelial cell survival and blood vessel maturation by the angiopoietin/Tie-2 system, further analysis of angiogenesis in sickle cell disease is warranted.


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.


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.


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