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(A) Weight status in sickle cell anemia patients518 years old from the Ibadan and Chicago cohorts. A higher proportion of sickle cell anemia patients from Ibadan were underweight (42.0 vs. 13.0%), while higher proportions of sickle cell anemia patients from Chicago were overweight or obese (6.0 vs. 3.0%), respectively. (B) Weight status in sickle cell anemia patients !18 years old from the Ibadan and Chicago cohorts. A higher proportion of sickle cell anemia patients from Ibadan were underweight (34.0 vs. 9.0%), while higher proportions of sickle cell anemia patients from Chicago were overweight or obese (25.0 vs. 3.0%), respectively.
Source publication
Abstract To identify factors that affect manifestations of sickle cell anemia we compared patients 11-30 years of age from University of Ibadan, Ibadan, Oyo, Nigeria (n = 214) and University of Illinois at Chicago, Chicago, IL, USA (n = 209). Paralleling findings in the general populations of the two countries, the Chicago patients were more often...
Contexts in source publication
Context 1
... sickle cell anemia patients 518 years old, these differences persisted for weight (33 vs. 42 kg) and BMI (15.7 vs. 17.8 kg/m 2 ) between the Ibadan and Chicago cohorts, respectively, although the differences were not significant after the Bonferonni correction. A higher propor- tion of patients from the Ibadan cohort were underweight ( Figure 1A and B), while a higher proportion of patients from the Chicago cohort were overweight or obese in both age groups of sickle cell anemia patients ( Figure 1A and B). ...
Context 2
... sickle cell anemia patients 518 years old, these differences persisted for weight (33 vs. 42 kg) and BMI (15.7 vs. 17.8 kg/m 2 ) between the Ibadan and Chicago cohorts, respectively, although the differences were not significant after the Bonferonni correction. A higher propor- tion of patients from the Ibadan cohort were underweight ( Figure 1A and B), while a higher proportion of patients from the Chicago cohort were overweight or obese in both age groups of sickle cell anemia patients ( Figure 1A and B). ...
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Citations
... There is also empirical evidence suggesting disparities in the prevalence, type, severity, and complications, and cost of care related to SCD based on race/ethnicity [26][27][28]. For instance, among Black newborns in the United States 1 in 365 is affected by SCD compared to approximately 1 in 16,300 in Hispanic newborns [26,29]. ...
... 1,2 Despite understanding the proportion of overweight and obese adults nationally, the current prevalence of overweight and obesity among adults with SCD is unclear. [3][4][5][6][7][8][9][10][11][12] Many individuals with SCD experience multiple musculoskeletal and cardiopulmonary complications due to chronic vaso-occlusion; these conditions can be exacerbated by an overweight or obese BMI status. Individuals with SCD with higher BMIs may be faced with the compounded burden of the morbidity and mortality associated with SCD and the well-known social, financial, and comorbid complications associated with overweight and obesity. ...
... These studies have often yielded inconsistent results about the relationship between BMI and SCD, SCD complications, comorbidities, and socioeconomic factors. 3,4,6,7,10,15 Existing studies are limited by inclusion of mostly paediatric samples, which are often not adjusted for growth charts, the recommended means to determine BMI up to the age of 20 years. 16 Additional limitations include small sample sizes from single centres. ...
... Similarly, Farooqui et al. 5 reported a higher prevalence of HTN in an overweight/obese population of SCD in a single centre retrospective study in the USA. Akingbola et al. 10 compared two cohorts of patients with sickle cell anaemia from University of Illinois at Chicago and University of Ibadan, Nigeria and observed that the Chicago cohort was more likely to be overweight and obese, and rising BMI was independently associated with higher systolic and diastolic blood pressure and history of stroke. These associations are not only limited to the SCD population as associations between obesity, HTN, and ischaemic heart disease have also been found among the general population. ...
Individuals with sickle cell disease (SCD) have historically been considered underweight. Despite increasing body mass index (BMI) in the general population, the prevalence of overweight and obese status remains unclear in the adult SCD population. Our primary aim was to determine the prevalence of overweight and obese status and to identify associations between BMI, demographic, and clinical characteristics. We conducted an analysis of abstracted electronic health record data and patient‐reported outcomes from the Sickle Cell Disease Implementation Consortium registry; individuals aged 20–45 years were included. The median (interquartile range) BMI for the 1664 adults in this analysis was 23.9 (21.1–28) kg/m². In this cohort, 42.9% had a BMI of >25 kg/m² (Centers for Disease Control and Prevention definition of overweight/obese). In multivariable analysis, higher odds of being overweight or obese were associated with female gender, older age, college education, private insurance, and hypertension diagnosis. Higher odds of a BMI of >25 kg/m² were observed in individuals with HbSC or HbSβ⁺ thalassaemia regardless of hydroxycarbamide (hydroxyurea) exposure (odds ratio [OR] 3.4, p < 0.0001) and HbSS or HbSβ⁰ thalassaemia exposed to hydroxycarbamide (OR 1.6, p = 0.0003) compared to those with HbSS or HbSβ⁰ thalassaemia with no hydroxycarbamide exposure. These data highlight the importance of early identification, prevention, and intervention for increasing BMI to reduce obesity‐related complications that may impact SCD‐related complications.
... Unsurprisingly, this study also found that age was significantly associated with BP in SCD patients, SCD patients with RSH and systemic hypertension were older than SCD patients with normal BP values suggesting that advancing age contributed to their higher BP values. This finding corroborates with previous reports in developed countries; (1,8) and in Africa (18), that reported that BP rapidly increases with advancing age in SCD patients starting in the early twenties. With the improved survival of patients with SCD patients, the incidence of RSH or systemic hypertension is expected to rise, thus screening and awareness are necessary to prevent the expected complications, in all part of the world. ...
Increased blood pressure (BP) has been associated with higher risk of stroke and mortality in Sickle Cell Disease (SCD). We investigated risk factors associated with Relative Systemic Hypertension (RSH) or systemic hypertension in SCD patients in Cameroon. Using R, Multivariate multinomial logistic regression modeling was used to examine the effects of the demographic, anthropometric, clinical, and laboratory factors to determine risk factors. A total of 815 individuals with SCD, including 380 (46.6%) males were analyzed. At baseline, the median age [interquartile range] was 18.0 [12.0–25.0] years, ranging from 3 to 66 years. Approximately three-quarters of the patients (n = 645; 79.1%) had normal BP, 151 (18.5%) had RSH and 19 (2.3%) had hypertension. Age (P < 0.001) and gender (P = 0.022) were significantly different across the BP categories. Weight (P < 0.001), height (P < 0.001), BMI (P < 0.001), pulse pressure (P = 0.020), history of stroke (P = 0.012), hemoglobin level (P = 0.002), red blood cell count (P = 0.031), creatinine (P < 0.001), and (estimated glomerular filtration rate) eGFR (P = 0.002) was also significantly different across the three BP categories. After adjustment, the significantly associated factors of RSH in the SCD patients were age [OR = 1.03, (95% CI = 1.01–1.06), P < 0.010], male gender [OR = 1.54, (95% CI = 1.04–2.27), P = 0.029], BMI [OR = 1.10, (95% CI = 1.04–1.17), P = 0.001]. After adjustment, the independent variables significantly associated factors of Hypertension in the SCD patients were age [OR = 1.05, (95% CI = 1.01–1.10), P = 0.034], male gender [OR = 3.31, (95% CI = 1.04–10.52), P = 0.042], BMI [OR = 1.14, (95% CI = 1.01–1.29), P = 0.027]. Creatinine was significantly associated with RSH [OR =1.31 (1.05–1.63), P = 0.016]. SCD patients with RSH or hypertension maybe at increased risk of renal dysfunction. We found relatively high prevalence of RSH and hypertension (20.8%) in SCD patients in Cameroon. Tailored Interventions that consider major risk factors (age, gender, and BMI) may lower BP pressure and prevent severe complications.
... The nutritional status of people with SCD is traditionally characterized by malnutrition 49,50 . The identification of eutrophic predominance followed by overweight /obesity suggests that is experiencing a nutritional transition, characterized by the coexistence of malnutrition and overweight [51][52][53] . Some factors may have contributed to change the nutritional profile, such as improving the clinical treatment based on comprehensive care to patients with SCD 50 , as well as the characteristic of dietary intake of individuals with SCD in which there is a predominance of high levels of poor quality fat, a simple sugar, and fried foods, as well as low consumption of fruits, and vegetables 51,54,55 . ...
... The identification of eutrophic predominance followed by overweight /obesity suggests that is experiencing a nutritional transition, characterized by the coexistence of malnutrition and overweight [51][52][53] . Some factors may have contributed to change the nutritional profile, such as improving the clinical treatment based on comprehensive care to patients with SCD 50 , as well as the characteristic of dietary intake of individuals with SCD in which there is a predominance of high levels of poor quality fat, a simple sugar, and fried foods, as well as low consumption of fruits, and vegetables 51,54,55 . ...
Background: Historically, malnutrition is described in individuals with SCD. However, more recent studies have shown a change in the profile of the nutritional status and distribution of body composition of SCD patients, mainly adult individuals. Aims: To assess the body composition (BC), resting energy expenditure (REE), and the biomarkers of hemolysis in adults with sickle cell disease (SCD). Subjects and Methods: A cross-sectional observational study was performed with 64 individuals over 39 years old in the treatment from two reference centers for SCD located in the city of Rio de Janeiro, Brazil. The dual-energy X-ray absorptiometry (DXA) and indirect calorimetry were used to assess BC and REE, respectively. Blood levels of hemoglobin, reticulocytes, lactate dehydrogenase (LDH), leukocytes, platelets, total and direct bilirubin, total protein, and albumin were measured to assess the hemolysis and protein status. The descriptive and inferential analysis was composed of the different methods (one-way ANOVA with the multiple comparison test of Tukey, Student t-test, and Pearson's correlation coefficient). Were considered statistically significant when the p-values were ≤ 0.05. Results: Most participants with SCD were female sex, colored (brown/black), and mean age of 51.2 years old. The obesity prevalence was 70.7% according to the body fat (BF%), with a major mean among women (p < 0.0001). Men had a higher mean of lean mass (LM) (p=0.0005) and fat-free mass (FFM) (p=0.0007). There was no difference for REE in comparing the genotypes (p= 0.53), and genders (p=0.075). The hemolysis markers (LDH, reticulocytes, and TB) correlated inversely with BMI (p=0.013), FM (p=0.022), and FFM (p=0.034). Conclusions: The important change observed in body composition in people with sickle cell disease was characterized by a high percentage of fat body and a decrease in lean mass. The hemolysis markers LDH, reticulocytes, and BT correlated inversely with BMI, FM, and FFM indicating that high levels of hemolysis may affect nutritional status, without influencing the REE. Keywords: sickle cell disease, body composition, fat mass, fat-free mass, energy expenditure, hemolysis. Cite this article as : Cordovil, K., Marise Criveli, M., Barbosa, F.S., Fleury, M. (2022). Body composition, Energy Expenditure, and Markers of Hemolysis in Adults with Sickle Cell Disease. The North African Journal of Food and Nutrition Research, 6(13): 55-65. https://doi.org/10.51745/najfnr.6.13.55-65
... In addition, pulse rate and pulse pressure being higher in SCD patients is a concern because pulse pressure is a well-documented predictor of sudden death [29,30]. Akingbola et al., reported that the history of stroke was associated with higher SBP and BMI on univariate analyze (p < 0.006) [31]; however, Kuma et al., found a high prevalence of RSH and hypertension with a relatively low frequency of renal insufficiency, and no association between RSH and stroke. The study attributed these differences mostly to selection bias and under documentation of complications in their study design [16]. ...
... Furthermore, SCD patients with elevated BP have higher waist, neck circumference, elevated BMI, history of blood transfusion, and prior diagnosis [17,19,35]. Elevated BP has been reported to be associated with a history of stroke [31], higher prevalence of silent cerebral infarcts (SCI), and stroke in children with SCD [28]. Therefore, the ability to identify individuals prior to the development of cardiovascular complications is of paramount importance, particularly in SCD patients [35]. ...
Objectives:
Blood pressure (BP) values ≥ 120/70 mmHg considerably increase the risk for pulmonary hypertension and renal dysfunction in Sickle Cell Disease (CSD) patients, and ultimately increased morbidity and mortality. This has led to the development of the term relative systemic hypertension (RSH). RSH was defined as Systolic BP 120-139 mm Hg or diastolic BP 70-89 mm Hg, whereas systemic hypertension is defined as Systolic BP ≥ 140 mm Hg or diastolic BP ≥90 mm Hg. Systematic identification of BP variations and risk factors in SCD patients could promote effective management. This review aimed to identify factors associated with BP variation among SCD patients.
Methods:
We searched PubMed, Scopus, Web of Science and Google Scholar up to December 2020 with no geographical or language restrictions. Two reviewers independently screened, and summarized data from eligible studies.
Results:
Advancing age, gender, higher body weight, haemoglobin, eGFR, triglycerides, greater heamatocrit, higher blood viscosity, history of blood transfusion, and targeted variants in DRD2 and MIR4301 genes were independently associated with the risk of hypertension in SCD patients.
Conclusion:
Interventions that consider these risk factors may potentially contribute to lower BP pressure in SCD patients and prevent developing severe complications.
... Studies have shown Nigerian patients with sickle cell disease, and indeed, the general population, have significantly lower BMI than those in more developed countries [12]. This pattern is likely to be true for other countries in sub-Saharan Africa. ...
Sickle cell disease is often complicated by retinopathy, which can be proliferative or non-proliferative. Proliferative sickle cell retinopathy potentially leads to blindness. There is a paucity of data on sickle cell disease-related retinopathy from Africa, where the disease is most prevalent. We aimed to determine the clinical, ophthalmic, and laboratory predictors of sickle cell retinopathy in an African population. We conducted a cross-sectional study of 262 participants, aged 13 years and above, with sickle cell disease. Demographic and clinical data were collected using a structured questionnaire and standard physical examinations. Vitreo-retinal specialists performed eye examinations on all the participants. Hematological and biochemical assessments were conducted using standard methods. A multivariate stepwise forward logistic regression was performed to determine the predictors of retinopathy. The median age of the participants was 20 years (interquartile range: 17–25 years). Most of the participants had a homozygous Hb S (HBB: c.20A>T) genotype (96.9%), with 3.1% who carried a Hb S/Hb C (HBB: c.19G>A) genotype. The prevalence of non-proliferative sickle cell retinopathy was 24.4%. Only 1.9% had proliferative sickle cell retinopathy (PSCR). Elevated systolic blood pressure (BP) [odds ratio (OR): 6.85, 95% confidence interval (95% CI): 1.05–44.45, p = 0.059], moderate visual impairment (OR: 5.2, 95% CI: 1.39–19.63, p = 0.015), and anterior segment changes (OR: 2.21, 95% CI: 1.19–4.13, p = 0.012) were independently predictive of retinopathy. This study provides new insight into predictors of retinopathy in sickle cell disease, with implications on early screening and prevention.
... Despite being a monogenic disorder, SCD presents with extreme phenotypic variability [2,5,6]. While it is well known that the Central African Republic and Bantu haplotypes present the most severe clinical phenotype compared with the Senegalese and Arab ones, partially due to differences in fetal hemoglobin production, a detailed comparison of acute and chronic complications of SCD among different populations has not been performed [2]. ...
... While it is well known that the Central African Republic and Bantu haplotypes present the most severe clinical phenotype compared with the Senegalese and Arab ones, partially due to differences in fetal hemoglobin production, a detailed comparison of acute and chronic complications of SCD among different populations has not been performed [2]. Several studies did indeed compare mortality, acute events, alloimmunization rates, hemolysis, asthma, and psychosocial burden in SCD populations living in different countries [5,[7][8][9][10] but a detailed description of clinical manifestations of SCD in various ethnic groups and environments has not been performed. ...
... Unlike other reports combining results of previous clinical trials and cohort studies [6,15] which highlight differences in clinical presentation of SCD across continents, this paper's specific focus was the geographic distribution of patients of different ethnic origins in the USA, Europe, and Africa. Another study by Akingbola et al. [5] reported direct comparisons of SCD patients from Nigeria and USA highlighting differences in hydroxyurea use, body mass index, and blood pressure. The multi-national DOVE study revealed geographic differences in pain reporting and analgesic use among sub-Saharan African, North American, and European SCD patients [15,19]. ...
Millions are affected by sickle cell disease (SCD) worldwide with the greatest burden in sub-Saharan Africa. While its origin lies historically within the malaria belt, ongoing changes in migration patterns have shifted the burden of disease resulting in a global public health concern. We created the Consortium for the Advancement of Sickle Cell Research (CASiRe) to understand the different phenotypes of SCD across 4 countries (USA, UK, Italy, and Ghana). Here, we report the multi-generational ethnic and racial background of 877 SCD patients recruited in Ghana (n = 365, 41.6%), the USA (n = 254, 29%), Italy (n = 81, 9.2%), and the UK (n = 177, 20.2%). West Africa (including Benin Gulf) (N = 556, 63.4%) was the most common geographic region of origin, followed by North America (N = 184, 21%), Caribbean (N = 51, 5.8%), Europe (N = 27, 3.1%), Central Africa (N = 24, 2.7%), and West Africa (excluding Benin Gulf) (N = 21, 2.4%). SCD patients in Europe were primarily West African (73%), European (10%), Caribbean (8%), and Central African (8%). In the USA, patients were largely African American (71%), Caribbean (13%), or West African (10%). Most subjects identified themselves as Black or African American; the European cohort had the largest group of Caucasian SCD patients (8%), including 21% of the Italian patients. This is the first report of a comprehensive analysis of ethnicity within an international, transcontinental group of SCD patients. The diverse ethnic backgrounds observed in our cohort raises the possibility that genetic and environmental heterogeneity within each SCD population subgroup can affect the clinical phenotype and research outcomes.
link to Full-Text https://link.springer.com/article/10.1007%2Fs40615-020-00762-2
... In our study, we found that only 4% of patients had diagnosis codes indicating overweight or obesity. Local data for our own adult SCD patients at the University of Illinois at Chicago indicate prevalence of overweight and obesity to be 6% and 25% in paediatric and adult SCD cases, respectively (Akingbola et al, 2014). Nearly 2 of 3 US adults are either overweight or obese (Flegal et al, 2016). ...
Conflicting evidence exists on the epidemiology of type 2 diabetes mellitus (T2DM) among patients with sickle cell disease (SCD). This study measured the prevalence, incidence and clinical outcomes associated with T2DM in a large US population of commercially‐insured adults aged ≥20 years with SCD between 2009 and 2014. Among 7070 patients with SCD, the mean age (median) was 39 (37) years and 60·8% were female. The standardized prevalence of T2DM among patients with SCD showed a modest increase, from 15·7% to 16·5% (P trend = 0·026), and was comparable to African‐American respondents to the National Health and Nutrition Examination Survey (18·2%). Over 17 024 person‐years, the crude incidence rate for T2DM was 25·4 per 1000 person‐years. Incident T2DM was associated with comorbid hypertension (hazard ratio [HR] = 1·45, 95% confidence interval [CI] 1·14–1·83), and dyslipidaemia (HR = 1·43, 95%CI 1·04–1·96). Compared to SCD patients without T2DM, more SCD patients with T2DM had diagnoses of nephropathy (28·0% vs. 9·5%; P < 0·001), neuropathy (17·7% vs. 5·2%; P < 0·001) and stroke (24·1% vs. 9·2%; P < 0·001). Prevalence of T2DM in SCD patients is similar to the general African American population with an increasing trend in recent years. These trends support routine screening for T2DM in aging patients with SCD, especially those with comorbid hypertension and/or dyslipidaemia.
... A dearth of national newborn haemoglobinopathy screening across African countries prevents many affected children from being identified early and prioritised for these prophylactic measures. 11 Required childhood immunisations in Uganda are available at no cost from government-sponsored vaccination clinics. 12 Parents are issued vaccine cards for documenting immunisations. ...
... None of the patients was on hydroxyurea during the duration of this study and this may be due to poor knowledge and perception of physicians towards its use and the issue of cost and its side effects. A recent comparative study among sickle cell patients from Nigeria and the USA showed significant lower use of hydroxyurea among the Nigerian cohort 50 . Also the locally developed anti-sickling agent, Ciklavit®, which has been found to be effective in reducing sickling episodes, was not prescribed for any of the patients during the study 51 . ...
Background:
Sickle cell disease (SCD) is a genetic haematological disorder that affects millions of people around the world especially people of African heritage. The treatment of the symptoms of SCD includes the use of analgesics, antibiotics, and anti-malarial drugs. Studying the pattern of drug prescription is a veritable tool for establishing the current practice and how it conforms to existing guidelines.
Objectives:
The main objective of this study was to assess the pattern of drug prescription in children with sickle cell disease (SCD) attending the paediatric outpatients' clinic of a tertiary care centre in Ado-Ekiti, South-West Nigeria.
Methods:
This was a cross-sectional retrospective study carried out using the medical records of all patients with SCD who attended the paediatric outpatient clinic of the teaching hospital between January 1 and December 31, 2014. The information retrieved from the case notes included the bio-demographic data, associated co-morbid conditions and the list of prescribed drugs.
Results:
A total of 202 SCD patients aged below 18 years were seen in the clinic during the study period with males accounting for 61.9% of them. The mean age of all patients was 6.9 ±3.8 yrs. A total of 1015 medications were prescribed during the study period giving a mean of 5.02 ± 1.9. Vitamins/micronutrients, anti-malarial drugs, antibiotics and analgesics accounted for 41.4%, 29.0%, 15.7% and 13.9% of all prescribed drugs respectively. Antibiotics from the penicillin group were the most commonly prescribed followed by macrolides and cephalosporins while Ibuprofen (60.3%) and Acetaminophen (32.6%) were the commonly prescribed analgesics.
Conclusion:
High rate of antibiotic prescription, low use of opioid analgesics and non-prescription of prophylactic penicillin/pneumococcal vaccination were the main findings in this study. There is need for the introduction of standard treatment protocols for this group of patients.
