Body Composition and Physical Activity in Pediatric Intestinal Failure On and Off Parenteral Nutrition.

OBJECTIVES: Data on the relationship between body composition (BC) and physical activity (PA) in children with intestinal failure (IF) are lacking. The objectives were to collect data on PA and BC in children with IF, both parenterally and enterally fed, and to assess the relationship between PA and BC. METHODS: Cross-sectional study in children 5-18 years with IF including those receiving parenteral nutrition (PN) and those fully enterally fed. PA levels were measured using accelerometry. BC was measured by dual-energy X-ray absorptiometry. Data were compared to age- and sex-matched population norms using t tests. Regression analysis assessed the relationship between BC and PA. RESULTS: Fifty-eight children with IF (38 males), mean (SD) age of 10.0 (3.5) years, 20 dependent on PN were included. Patients with IF had significantly fewer steps per day ( P ≤ 0.001) compared with literature controls, with a mean (SD) of 7,972 (3,008) and 11,749 (1,106), respectively. There were no significant differences between patients receiving PN and those enterally fed, but both groups were significantly less active than literature controls ( P < 0.001). Patients with IF had higher fat mass and lower fat-free mass compared to literature controls ( P = 0.008). PA had a significant effect on BC ( r2 = 0.32, P < 0.001). CONCLUSIONS: Children with IF, those receiving PN and those fully enterally fed, are at risk of decreased PA and altered BC. PA should be part of ongoing rehabilitation and management to optimize outcomes.

Regular Voluntary Running is Associated with Increased Tumor Vascularization and Immune Cell Infiltration and Decreased Tumor Growth in Mice.

Tumors present dysfunctional vasculature that limits blood perfusion and hinders immune cells delivery. We aimed to investigate if regular voluntary running promotes tumor vascular remodelling, improves intratumoral immune cells infiltration and inhibits tumor growth. Tumors were induced in C57BL/6 male mice (n=28) by subcutaneous inoculation in the dorsal region with a suspension of RM1 cells (1.5×105 cells/500 µL PBS) and randomly allocated into two groups: sedentary (n=14) and voluntarily exercised on a wheel (n=14). Seven mice from each group were sacrificed 14 and 28 days after cells' inoculation to evaluate tumor weight, microvessel density, vessels' lumen regularity and the intratumoral quantity of NKG2D receptors, CD4+and CD8+T cells, by immunohistochemistry. The statistical inference was done through a two-way ANOVA. Exercised mice developed smaller tumors at 14 (0.17±0.1 g vs. 0.48±0.2 g, p<0.05) and 28 (0.92±0.7 g vs. 2.09±1.3 g, p<0.05) days, with higher microvessel density (21.20±3.2 vs. 15.86±4.0 vessels/field, p<0.05), more regular vessels' lumen (1.06±0.2 vs. 1.43±0.2, p<0.05), and higher CD8+T cells (464.95±48.0 vs. 364.70±49.4 cells/mm2, p<0.01), after 28 days. NKG2D expression was higher in exercised mice at 14 (263.27±25.8 cells/mm2, p<0.05) and 28 (295.06±56.2 cells/mm2, p<0.001) days. Regular voluntary running modulates tumor vasculature, increases immune cells infiltration and attenuates tumor growth, in mice.

Are Genetic Modifiers the Answer to Different Responses to Hydroxyurea Treatment?-A Pharmacogenetic Study in Sickle Cell Anemia Angolan Children.

Sickle cell anemia (SCA) is an inherited disease affecting the hemoglobin that is particularly common in sub-Saharan Africa. Although monogenic, phenotypes are markedly heterogeneous in terms of severity and life span. Hydroxyurea is still the most common treatment for these patients, and the response to treatment is highly variable and seems to be an inherited trait. Therefore, identifying the variants that might predict hydroxyurea response is important for identifying patients who will have a poorer or non-response to treatment, and the ones that are more prone to suffer from severe side effects. In the present pharmacogenetic study, we analyzed the exons of 77 genes described in the literature as potentially associated with hydroxyurea metabolism in Angolan children treated with hydroxyurea and evaluated the drug response considering fetal hemoglobin levels, other hematological and biochemical parameters, hemolysis, number of vaso-occlusive crises and hospitalizations. Thirty variants were identified in 18 of those genes as possibly associated with drug response, five of them in gene DCHS2. Other polymorphisms in this gene were also associated with hematological, biochemical and clinical parameters. Further research examining the maximum tolerated dose and fixed dose with a larger sample size is necessary to corroborate these findings.

"Mãos de Conforto" (Hands of Comfort): A novel non-pharmacological intervention to ease agitation in elderly persons with dementia.

Behavioral symptoms associated with dementia, such as agitation, are frequent and associated with well-known negative consequences for patients, their carers, and their environment. Pharmacological treatments for agitation using sedatives and antipsychotics are known to have several undesirable side effects and modest efficacy. Non-pharmacological alternatives are recommended as first-line options for agitation in persons with dementia with few side effects, but there is limited evidence of efficacy. We developed a novel and simple non-pharmacological alternative for agitation in dementia residents based on a Brazilian intervention using warm water surgical gloves used in patients with COVID-19 in intensive care units during the pandemic. We coined it "Mãos de Conforto" - Hands of Comfort. We report a series of 7 cases in 3 residents with dementia who whore Hands of Comfort.

A qualitative analysis of the needs and wishes of people with type 2 diabetes and healthcare professionals for optimal diabetes care.

AIM: Globally, type 2 diabetes care is often fragmented and still organised in a provider-centred way, resulting in suboptimal care for many individuals. As healthcare systems seek to implement digital care innovations, it is timely to reassess stakeholders' priorities to guide the redesign of diabetes care. This study aimed to identify the needs and wishes of people with type 2 diabetes, and specialist and primary care teams regarding optimal diabetes care to explore how to better support people with diabetes in a metropolitan healthcare service in Australia. METHODS: Our project was guided by a Participatory Design approach and this paper reports part of the first step, identification of needs. We conducted four focus groups and 16 interviews (November 2019-January 2020) with 17 adults with type 2 diabetes and seven specialist clinicians from a diabetes outpatient clinic in Brisbane, Australia, and seven primary care professionals from different clinics in Brisbane. Data were analysed using reflexive thematic analysis, building on the Capability, Opportunity, Motivation and Behaviour model. RESULTS: People with diabetes expressed the wish to be equipped, supported and recognised for their efforts in a holistic way, receive personalised care at the right time and improved access to connected services. Healthcare professionals agreed and expressed their own burden regarding their challenging work. Overall, both groups desired holistic, personalised, supportive, proactive and coordinated care pathways. CONCLUSIONS: We conclude that there is an alignment of the perceived needs and wishes for improved diabetes care among key stakeholders, however, important gaps remain in the healthcare system.

Electrocardiographic findings in pregnant women in Angola.

BACKGROUND: Studies on the electrocardiogram findings in African pregnant women are limited. There is no information available in the literature on the electrocardiographic parameters of pregnant Angolan women. OBJECTIVES: The aim of this study was to describe electrocardiographic findings in women with normal pregnancies in Bengo Province, Angola. METHODS: This is a community-based study with a cross-sectional design conducted between September 2013 and March 2014 in Bengo. The study involved 114 black pregnant women, compared with a paired control group comprising of 120 black non-pregnant women, aged 15 to 42 years. A 12-lead electrocardiogram and a rhythm strip were recorded for all participants. RESULTS: In this study, the mean age was 26.2 ± 7.3 years. Comparing pregnant women vs. non-pregnant, we found the following mean values: Heart rate (83 bpm vs. 74 bpm, p < .001), PR interval (146 ms vs. 151 ms, p = .034), QT interval (360 ms vs. 378 ms, p < .001), QTIc Fridericia (398 ms vs. 403, p = .017), QTIc Framingham (399 ms vs. 404 ms, p = .013) and T-wave axis (340 vs. 410 , p = .001).The main electrocardiographic changes found were: Sinus tachycardia (4.4% vs. 2.5%), T-wave inversion (14.9% vs. 1.7%), Minor ST segment depression (4.5% vs. 0%) and left ventricular hypertrophy (11.4% vs. 11.7%, p = .726). CONCLUSIONS: Pregnant Angolan women compared with controls, had several significantly higher values for heart rate, and significantly lower values of systolic blood pressure and diastolic blood pressure, PR interval, QT interval, QTc interval by Fridericia and Framingham and T-wave axis. Sinus tachycardia, T-wave inversion, and left ventricular hypertrophy, were the main electrocardiographic changes found.

Schools reopening and the COVID-19 pandemic: a case study from Macaé, Rio de Janeiro, Brazil.

Since the first reported case of COVID-19 in Brazil, the public and private educational system started to close. Up to November 2020, scientific discussions about the return of schooling activities have been rarely performed by the national scientific community and police-makers. The great delay of school returning in Brazil contrasts with successful international strategies of school reopening worldwide and seems counterintuitive with the reopening of non-essential activities. Here, important issues to be considered before and during school reopening are reviewed and discussed. COVID-19 testing is essential to avoid disease spreading, but high cost of individual RT-qPCRs impairs an extensive testing strategy for school returning. To reduce costs and increase the speed of diagnosis, we tested the efficiency of a pooled-sample PCR strategy in a cohort of the educational staff in the city of Macaé/RJ, finding five asymptomatic individuals (0,66%) among the 754 people tested. Thus, a polled-sample PCR testing strategy of the educational staff might prevent infection spreading in schools at a reasonable cost. We discuss how our test strategy could be coupled with internationally recognized safety rules to allow for a safe school return and how countries from different world regions are dealing with educational activities during COVID-19 pandemic.

How Hydroxyurea Alters the Gut Microbiome: A Longitudinal Study Involving Angolan Children with Sickle Cell Anemia.

Sickle cell anemia (SCA) is an inherited hematological disorder and a serious global health problem, especially in Sub-Saharan Africa. Although hydroxyurea (HU) is the leading treatment for patients with SCA, its effects on the gut microbiome have not yet been explored. In this context, the aim of this study was to investigate this association by characterizing the gut microbiome of an Angolan SCA pediatric population before and after 6 months of HU treatment. A total of 66 stool samples were obtained and sequenced for the 16S rRNA gene (V3-V4 regions). Significant associations were observed in alpha and beta-diversity, with higher values of species richness for the children naïve for HU. We also noticed that children after HU had higher proportions of several beneficial bacteria, mostly short-chain fatty acids (SCFAs) producing species, such as Blautia luti, Roseburia inulinivorans, Eubacterium halli, Faecalibacterium, Ruminococcus, Lactobacillus rogosae, among others. In addition, before HU there was a higher abundance of Clostridium_g24, which includes C. bolteae and C. clostridioforme, both considered pathogenic. This study provides the first evidence of the HU effect on the gut microbiome and unravels several microorganisms that could be considered candidate biomarkers for disease severity and HU efficacy.

Review of educational tools for skin self-examination: A qualitative analysis of laypeople's preferences.

ISSUE ADDRESSED: Skin self-examination (SSE) empowers individuals to be aware of their skin and to identify and present to a doctor with any suspicious lesions; however evidence-based, clear guidance for SSE is lacking. METHODS: A new acronym entitled SCAN (Sore, Changing, Abnormal, New) was compiled to help laypeople recognise early signs of skin cancer. This study undertook a qualitative investigation on how well laypeople understand SCAN and other commonly used educational tools (ABCDE, AC, and "ugly duckling"), whether they have a preferred SSE tool, and whether pictorial presentations enhance their understanding. RESULTS: Seven focus groups were conducted with 28 participants (age range 19-78 years), including 6 participants with a previous melanoma diagnosis. Among all the educational tools presented, SCAN was commonly described as the favoured to guide SSE. SCAN was perceived as patient-oriented due to its simplicity and use of easily understood words, while other tools, although perceived as positive in some aspects, were often identified as complex, difficult to understand and aimed towards clinician use. Pictorial presentations were desired as part of an educational tool; however, many also raised their potential disadvantages. CONCLUSION: This was the first study to determine the patient perspective of SCAN and established a diverse range of potential quality indicators for SSE educational tools. SO WHAT?: The findings of this study suggest that current widely used SSE tools may not be the most effective to promote SSE. SCAN may be preferred, as it simplifies SSE by using lay language and is applicable to all skin cancers.

What Can Twitter Tell Us about Skin Cancer Communication and Prevention on Social Media?

BACKGROUND: Skin cancer places a substantial burden on the health system in Australia. The modernisation of skin cancer prevention interventions by using social media may enhance their contemporary reach and relevance, especially among the youth. This study aimed to analyse Twitter posts in Australia to establish a baseline of skin cancer and sun-related communication trends. METHODS: Tweets posted over the summer of 2018/2019 relating to relevant keywords and hashtags such as "slipslopslap" and "sunscreen" were analysed using quantitative and qualitative methods. The number of tweets, related communication patterns, and possible factors for sudden changes in tweet volume were studied. RESULTS: Results showed that Australians are using Twitter to communicate about their sun-related and skin cancer experiences and to share advice and information on this matter. Overall, Australians use Twitter more frequently to talk about sunscreen (number of tweets between December 2018 and February 2019 = 5,842) and/or skin cancer (n = 3,936), but not so often to communicate about other sun-protective behaviours (n = 1,972) or skin cancer prevention campaigns (n = 108). The number of tweets is greatly affected by the increase in temperature. Celebrities, non-health-related organisations with a high number of followers, and individuals sharing their own skin cancer experiences achieve a substantial number of likes and retweets and thus influence on Twitter regarding skin cancer-related communication. CONCLUSIONS: The results allow deeper understanding of important determinants for effective social media use for health promotion and public health messaging in the future. Linking tweets with temperature, sharing by celebrities or non-health-related organisations and individuals with a high following can all contribute to greater spread of skin cancer and sun-related messages.

Normal limits of the electrocardiogram in Angolans.

INTRODUCTION: Studies on the normal electrocardiogram limits in African populations are limited, especially in sub-Saharan Africa. There is no literature describing normal ECG limits in Angolans. OBJECTIVES: The aim of this study is to establish the normal ECG limits for adult Angolans, without established heart disease, stratified by gender and age. METHODS: A cross-sectional study was performed, involving 2179 participants from a population in northern Angola, without established heart disease, aged between 15 and 74 years. A 12­lead ECG and a rhythm strip were recorded for all participants and analysed and processed by the University of Glasgow software and encoded by the Minnesota Code. The normal range of the electrocardiographic parameters were established as the 2nd and 98th percentiles of the measurement distribution per age group and gender. Mann-Whitney and Kruskal-Wallis tests were used for two independent groups and Bonferroni adjustments were used for multiple testing. GAMLSS models were used to obtain the continuous age-dependent percentile curves. RESULTS: The normal range of the ECG differed between men and women: heart rate 49 to 100 bpm vs. 55 to 108 bpm, P wave duration 81 to 130 ms vs. 84 to 130 ms, PR interval 119 to 210 ms vs. 120 to 202 ms, QRS duration 74 to 110 ms vs. 70 to 104 ms, QT interval 324 to 441 ms vs. 314 and 438 ms, P-wave axis - 29 to 850 vs. -18 to 810, QRS-wave axis - 13 to 850 vs. -180 and 820, T-wave axis 0 to 720 vs. -8 to 730, Sokolow-Lyon index 2.13 to 6.21 mV vs. 1.60 to 4.87 mV, Cornell index 0.17 to 6.24 mV vs. 0.14 mV to 4.35 mV. CONCLUSIONS: The values described for the electrocardiographic measurements above can act as a reference framework for Angolan adults without established heart disease. Our study suggests that the normal range of most ECG parameters vary according to age and sex and the ECG diagnostic criteria must therefore be specific for these demographic measures.

The Role of Pref-1 during Adipogenic Differentiation: An Overview of Suggested Mechanisms.

Obesity contributes significantly to the global health burden. A better understanding of adipogenesis, the process of fat formation, may lead to the discovery of novel treatment strategies. However, it is of concern that the regulation of adipocyte differentiation has predominantly been studied using the murine 3T3-L1 preadipocyte cell line and murine experimental animal models. Translation of these findings to the human setting requires confirmation using experimental models of human origin. The ability of mesenchymal stromal/stem cells (MSCs) to differentiate into adipocytes is an attractive model to study adipogenesis in vitro. Differences in the ability of MSCs isolated from different sources to undergo adipogenic differentiation, may be useful in investigating elements responsible for regulating adipogenic differentiation potential. Genes involved may be divided into three broad categories: early, intermediate and late-stage regulators. Preadipocyte factor-1 (Pref-1) is an early negative regulator of adipogenic differentiation. In this review, we briefly discuss the adipogenic differentiation potential of MSCs derived from two different sources, namely adipose-derived stromal/stem cells (ASCs) and Wharton's Jelly derived stromal/stem cells (WJSCs). We then discuss the function and suggested mechanisms of action of Pref-1 in regulating adipogenesis, as well as current findings regarding Pref-1's role in human adipogenesis.

Oral acute toxicity and impact of neonicotinoids on Apis mellifera L. and Scaptotrigona postica Latreille (Hymenoptera: Apidae).

Wild and managed bees are essential for crop pollination and food production. However, the widespread use of insecticides such as neonicotinoids may affect the survival, development, behavior, and maintenance of bee colonies. Therefore, in this study we evaluated the impacts of three neonicotinoid insecticides on the survival and walking abilities of the Africanized honeybee A. mellifera and stingless bee S. postica. A. mellifera was more susceptible than S. postica to all neonicotinoids tested. The median lethal concentrations LC50 values estimated for acetamiprid, imidacloprid, and thiacloprid were 189.62, 22.78, and 142.31 ng µL-1 of diet for A. mellifera, and 475.94, 89.11, and 218.21 ng µL-1 of diet for S. postica, respectively. All tested neonicotinoids affected the speed, distance traveled, duration and frequency of resting, and continuous mobility of both bee species. The results showed that in spite of the different susceptibility to compounds with cyano and nitro radicals, the behavioral variables showed different levels of commitment according to the molecule insecticide and bee species. These results contribute not only to the understanding of the effects of neonicotinoid insecticides on A. mellifera and S. postica, but also to help in the development of protocols that aim to reduce the impact of these insecticides in Neotropical environments.

Exercise Training-induced Modulation in Microenvironment of Rat Mammary Neoplasms.

Despite the importance attributed to exercise training in the breast cancer (BC) continuum, the underlying mechanisms modulating tumor behavior are unknown. We evaluated the effects of long-term moderate-exercise in the development of mammary tumors, and studied the microenvironment of infiltrative lesions, the amount of connective tissue, and balance between cellular proliferation/death.Fifty Sprague-Dawley rats, randomly assigned into four groups: two control groups (sedentary and exercised) and two models of BC groups (sedentary and exercised) induced by N-methyl-N-nitrosoureia (MNU), were sacrificed after 35 weeks of moderate-exercise, and all perceptible tumors were removed for histological and immunohistochemistry analysis.The median number of infiltrative-lesions per animal was lower in the MNU exercised animals (p=0.02). More than one histological pattern was identified, and papillary carcinoma was the most frequent in both groups. Within infiltrative-lesions, the number of immunopositive cells per µm2 of Ki67 was lower in exercised animals (p=0.002). This presents increased cell death per µm2 (p=0.019). Tumors from sedentary animals had a higher expression of collagen deposition (p=0.027).Long-term moderate-exercise has beneficial effects in tumor development with a diminished prevalence of malignancy. Within infiltrative-lesions, moderate-exercise improves the balance between cell-proliferation and cell-death with decreased connective tissue that suggests lower tumor aggressiveness.

Thidiazuron (TDZ) increases fruit set and yield of 'Hosui' and 'Packham's Triumph' pear trees.

The low fruit set is one of the main factors leading to poor yield of pear orchards in Brazil. The exogenous application of thidiazuron (TDZ) and aminoethoxyvinilglycine (AVG) has shown promising results in some pear cultivars and other temperate fruit trees. The objective of this study was to evaluate the effect of TDZ and AVG on fruit set, yield, and fruit quality of 'Hosui' and 'Packham's Triumph' pears. The study was performed in a commercial orchard located in São Joaquim, SC. Plant material consisted of 'Hosui' and 'Packham's Triumph' pear trees grafted on Pyrus calleryana. Treatments consisted on different rates of TDZ (0 mg L-1, 20 mg L-1, 40 mg L-1 and 60 mg L-1) sprayed at full bloom for both cultivars. An additional treatment of AVG 60 mg L-1 was sprayed one week after full bloom in 'Hosui'. The fruit set, number of fruit per tree, yield, fruit weight, seed number, and fruit quality attributes were assessed. Fruit set and yield of both cultivars are consistently increased by TDZ, within the rates of 20 to 60 mg L-1. Besides, its application increased fruit size of 'Hosui' and did not negatively affect fruit quality attributes of both cultivars.

Off-Label Use of Bevacizumab in Patients Diagnosed with Age-Related Macular Degeneration: A Systematic Review and Meta-Analysis.

BACKGROUND: Age-related macular degeneration (AMD) is the leading cause of vision loss in elderly people. Current pharmacological treatment in vascular AMD includes anti-VEGF agents, such as ranibizumab and aflibercept. Additionally, the off-label use of bevacizumab has been shown to be effective and has a lower cost, making it an interesting pharmacological approach; however, there is no consensus about its use. Therefore, this systematic review and meta-analysis aims to evaluate the efficacy, safety, and efficiency of bevacizumab in AMD patients. METHODS: This review only focused on randomized controlled clinical trials published in 2010 in the MEDLINE database that compared the effect of bevacizumab with ranibizumab. The risk of bias in each included study was assessed using the CASP Randomised Clinical Trials checklist. RESULTS: Twelve studies were included for qualitative synthesis, and nine of them were considered for meta-analysis. Bevacizumab-treated patients showed a significantly reduced neovascularization in a longer spectrum of time; however, they had a higher incidence of endophthalmitis than those treated with ranibizumab. Regarding efficiency, the mean number of administrations was reduced in the treatment with bevacizumab in comparison to ranibizumab. CONCLUSIONS: Clinical evidence demonstrates that bevacizumab has efficacy and safety profiles comparable with ranibizumab; however, it is relatively more efficient.

The efficient role of sodium alginate-based biodegradable dressings for skin wound healing application: a systematic review.

Injuries and damage to the skin can be caused by different reasons throughout human life. The use of sodium alginate in tissue dressing has been highly studied due to its intrinsic properties, including its degradation rate and biocompatibility, and the capacity of supporting tissue proliferation. The aim of this paper is to demonstrate evidences, through a systematic review method, to support the application of sodium alginate as a curative and as a potential accelerator in the healing of skin wounds. Four databases were used to develop this systematic review: Science Direct, PubMed, Scielo and Scopus. The time interval established for the search was from January 2016 to October 2023. After applying the exclusion and inclusion criteria, each selected article was evaluated and it was observed that the improvement of the mechanical properties of sodium alginate when correctly processed and crosslinked were evident. However, the increase of crosslinking affects as the wettability and the swelling of the biomaterials can cause limitations in mechanical properties and hidrophilic behavior. To achieve the ideal dressing, it is necessary to apply the optimal concentration of crosslinking and other substances, which can damage its hidrophilic characteristic. Thus, it was concluded that sodium alginate has every caracteristic desirable to develop an effective and safe dressing.

Influence of cigarette smoking on the Index of Cardiac Electrophysiological Balance in apparently healthy Angolans.

Background: Tobacco use accelerates atherosclerosis and is one of the predictors of death from ischemic heart disease, arrhythmias, heart failure, and sudden death. A new non-invasive parameter, the Index of Cardiac Electrophysiological Balance (iCEB) between depolarization and repolarization of the action potential, was considered a new biomarker for the identification of patients at increased arrhythmic risk. Objectives: We aimed to evaluate the iCEB in apparently healthy Angolans with habitual cigarette smoking compared to non-smokers. Subjects and methods: Data were obtained from the CardioBengo study, a cross-sectional community-based study in which a random sample of individuals aged between 15 and 84 years was selected. In total, 214 apparently healthy subjects, 102 smokers, and 112 non-smokers in the same age group were included in the final analysis. Results: The average age of the participants was 42.17 ± 13.04 years old and 26.6% of the sample was female. Smoking subjects had higher iCEB and corrected Index of Cardiac Electrophysiological Balance (iCEBc) values compared with non-smoking controls (4.39 vs. 4.25; p = 0.024, respectively), and (4.74 vs. 4.57; p = 0.030, respectively). Conclusions: In summary, iCEB and iCEBc were significantly higher in habitual smokers than in nonsmokers, which represents an increased risk of ventricular arrhythmogenesis in healthy habitual smokers. To the best of our knowledge, this is the first study performed in Africa to evaluate iCEB in smokers, making this type of study very important in low- and middle-income countries in the context of epidemiological transition.

Genetic Modifiers of Sickle Cell Anemia Phenotype in a Cohort of Angolan Children.

The aim of this study was to identify genetic markers in the HBB Cluster; HBS1L-MYB intergenic region; and BCL11A, KLF1, FOX3, and ZBTB7A genes associated with the heterogeneous phenotypes of Sickle Cell Anemia (SCA) using next-generation sequencing, as well as to assess their influence and prevalence in an Angolan population. Hematological, biochemical, and clinical data were considered to determine patients' severity phenotypes. Samples from 192 patients were sequenced, and 5,019,378 variants of high quality were registered. A catalog of candidate modifier genes that clustered in pathophysiological pathways important for SCA was generated, and candidate genes associated with increasing vaso-occlusive crises (VOC) and with lower fetal hemoglobin (HbF) were identified. These data support the polygenic view of the genetic architecture of SCA phenotypic variability. Two single nucleotide polymorphisms in the intronic region of 2q16.1, harboring the BCL11A gene, are genome-wide and significantly associated with decreasing HbF. A set of variants was identified to nominally be associated with increasing VOC and are potential genetic modifiers harboring phenotypic variation among patients. To the best of our knowledge, this is the first investigation of clinical variation in SCA in Angola using a well-customized and targeted sequencing approach.