Search results for: sickle cell trait

Authors: Mahfoodha Al-Kitani, Dylan Thompson, Keith Stokes

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Sickle cell disease (SCD) and sickle cell trait (SCT) are the most common hematological diseases in Oman according to the national survey of genetic blood disorders. The aim of this study was to determine markers of physical fitness and anthropometrics indices in children with sickle cell disease and children with sickle cell trait and compare them with normal healthy children of the same age. One hundred and twenty male children participated in the present study divided to three groups: 40 with sickle disease (SCD; age, 13.3(.80), height, 131.9(3.5), mass, 29.2(3.1)); 40 with sickle cell trait (SCT; age, 12.2(.80), height, 141.0(9.9), mass, 38.0(4.4)); and 40 controls with normal hemoglobin (Con; age, 12.8(.80), height, 139.4(8.7), mass, 37.2(4.3)). All children completed a 5-min running exercise test on a treadmill at speed corresponding to 5 km/hr. Heart rate and was recorded during exercise and during 10-min of recovery. Blood lactate was measured before and 5 min after the completion of exercise. Children with SCD exhibited a higher mean value (P < 0.05) for percent body fat and fat mass than the normal healthy subjects and SCT subjects. Resting values of hemoglobin were similar in SCT (11.04(.78)) and control (10.8(94)) groups, and lower in SCD (8.89(.54); P < 0.05). There was a strong correlation between peak heart rate and resting hemoglobin levels for the three groups (r= -.472. n= 120, p < .0005).The SCD group (175.2(10.3)) exhibited higher mean heart rate during exercise than those observed in the SCT (143.7(9.5)) and normal control children (144.5(22.4); P < 0.05). Additionally, SCD children showed higher serum lactate values before and after treadmill exercise compared to the other groups (P < 0.05). Children with sickle cell trait demonstrate similar physical fitness level and similar exercise responses to treadmill stress test to normal children. In contrast, SCD children have lower body mass, higher fat mass and lower physical fitness than children with SCT and healthy controls.

Keywords: sickle cell disease, sickle cell trait, children, exercise

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Authors: Charlie Jeffkins

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Sickle cell disease is a serious life-limiting condition which can reduce the quality of life for many patients. Public Health England (PHE), in partnership with the Sickle Cell Society (SCS), has created guidelines to prevent severe complications from sickle cell disease. Data was collected from Children’s Hospital for Wales between 15/03/21-26/03/21. Methods: A manual search of patient records for children under the care of Rocket Ward and a key term search of online records was used. Results: Penicillin prophylaxis was given at 90 days for 89%, 77% of TCDs scans were done at 2-3 years, and 72% have had a scan in the last year. 53% of patients have had discussions about hydroxycarbamide, whilst 65% have started it. PPV vaccination was documented for 19%. Conclusion: Overall, none of the four standards were reached; however, TCD uptake has improved. There is a need for better documentation of treatment and annual re-audits.

Keywords: paediatric, haematology, sickle cell, audit

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Authors: Agi Sunday

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A descriptive analysis of reported cases of sickle cell disease and the level of awareness about genetic counselling in 30 hospitals were carried out. Additionally, 150 individuals between ages 16-45 were randomly selected for evaluation of genetic counselling awareness. The main tools for this study were questionnaires which were taken to hospitals, and individuals completed the others. The numbers of reported cases of sickle cell disease recorded in private, public and teaching hospitals were 14 and 57; 143 and 89; 272 and 57 for the periods of 1995-2000 and 2001-2005, respectively. A general informal genetic counselling took place mostly in the hospitals visited. 122 (86%) individuals had the knowledge of genetic disease and only 43 (30.3%) individuals have been exposed to genetic counselling. 64% of individuals agreed that genetic counselling would help in the prevention of genetic disease.

Keywords: sickle disease, genetic counseling, genetic testing, advocacy

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Authors: Theresa Ukamaka Nwagha, Angela Ogechukwu Ugwu, Martins Nweke

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Background and Objectives: Blood transfusion is an effective and proven treatment for some severe complications of sickle cell disease. Recurrent transfusions have put patients with sickle cell disease at risk of developing antibodies against the various antigens they were exposed to. This study aims to investigate the frequency of red blood cell alloimmunization in patients with sickle disease in Africa. Materials and Methods: This is a systematic review of peer-reviewed literature published in English. The review was conducted consistent with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses checklist. Data sources for the review include MEDLINE, PubMed, CINAHL, and Academic Search Complete. Included in this review are articles that reported the frequency/prevalence of red blood cell alloimmunization in sickle cell disease patients in Africa. Eligible studies were subjected to independent full-text screening and data extraction. Risk of bias assessment was conducted with the aid of the mixed method appraisal tool. We employed a random-effects model of meta-analysis to estimate the pooled prevalence. We computed Cochrane’s Q statistics and I2 and prediction interval to quantify heterogeneity in effect size. Results: The prevalence estimates range from 2.6% to 29%. Pooled prevalence was estimated to be 10.4% (CI 7.7.–13.8); PI = 3.0 – 34.0%), with significant heterogeneity (I2 = 84.62; PI = 2.0-32.0%) and publication bias (Egger’s t-test = 1.744, p = 0.0965). Conclusion: The frequency of red cell alloantibody varies considerably in Africa. The alloantibodies appeared frequent in this order: the Rhesus, Kell, Lewis, Duffy, MNS, and Lutheran

Keywords: frequency, red blood cell, alloimmunization, sickle cell disease, Africa

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Authors: Fatima El Zahra Amin, Emine Efe

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This study was carried out to evaluate the effect of the education program for parents of children with Sickle Cell Anemia, on the knowledge level of parents and the reduction of pain relief by non-pharmacological methods used by parents at home. In Turkey, 54 parents and 109 from Chad agreed to participate in the survey. The data were collected by the researcher using a face-to-face interview method. Non-pharmacological treatment information form for parents, face expressions rating scale, and parent education program for non-pharmacological methods used in children with sickle cell anemia were used. It was determined that there was a statistically significant difference between the educational status, occupation, disease status, place of residence, family structure and age of parents of Chad and Turkey. According to the ratings of facial expressions scale, it was concluded that there was no significant difference between the children’s average degree of pain before and after administration of non-pharmacological methods by the groups of Chad and Turkey. It was determined that the educational programs prepared for parents of children with sickle cell anemia in both Turkey and Chad were effective in increasing the knowledge level of parents and also in reducing pain crisis with non-pharmacological methods parents used at home.

Keywords: Chad, child, non-pharmacological treatment methods, nurse, sickle cell anemia, Turkey

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Authors: Basma Hassabo, Sarah Ahmed, Aisha Hameed

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Aims and Objectives: To determine the incidence of maternal mortality amongst pregnant women with sickle cell disease (SCD) in the United Kingdom and to determine exact cause of death in these women. Background: SCD is caused by the ‘sickle’ gene and is characterized by episodes of severe bone pain and other complications like acute chest syndrome, chronic pulmonary hypertension, stroke, retinopathy, chronic renal failure, hepato-splenic crises, avascular bone necrosis, sepsis and leg ulcers. SCD is a continual cause of maternal mortality and fetal complications, and it comprises 1.5% of all Direct and Indirect deaths in the UK. Sepsis following premature rupture of membranes with ascending infection, post-partum infection and pre-labour overwhelming septic shock is one of its leading causes of death. Over the last fifty years of maternal mortality reports in UK, between 1 to 4 pregnant women died in each triennium. Material and Method: This is a retrospective study that involves pregnant women who died from SCD complications in the UK between 1952-2012. Data were collected from the UK Confidential Enquiries into Maternal Death and its causes between 1952–2012. Prior to 1985, exact cause of death in this cohort was not recorded. Results: 33 deaths reported between 1964 and 1984. 17 deaths were reported due to sickle cell disease between 1985 and 2012. Five women in this group died of sickle cell crisis, one woman had liver sequestration crisis, two women died of venous thromboembolism, two had myocardial fibrosis and three died of sepsis. Remaining women died of amniotic fluid embolism, SUDEP, myocardial ischemia and intracranial haemorrhage. Conclusion: The leading causes of death in sickle cell sick pregnant women are sickle cell crises, sepsis, venous thrombosis and thromboembolism. Prenatal care for women with SCD should be managed by a multidisciplinary team that includes an obstetrician, nutritionist, primary care physician, and haematologist. In every sick Sickle Cell woman Sickle Cell crises should be on the top of the list of differential diagnosis. Aggressive treatment of complications with low threshold to commence broad-spectrum antibiotics and LMWH contribute to better outcomes.

Keywords: incidence, maternal mortality, sickle cell disease (SCD), uk

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Authors: Surekha Rathod

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Sickle cell disease is a vascular disorder characterized by chronic, ongoing organ damage that is punctuated by episodes of acutely painful vascular complications.1 It is the most common genetic blood disorder in the United States, with about 2000 infants being identified through routine blood screenings annually, and an estimated 104,000-138,000 affected individuals living in the United States. Approximately 0.3%-1.3% of African American are affected by Sickle Cell Diseases (SCD).3 The aim of this paper is to present oral health status of patients with SCD. A total of 200 subjects of both sexes in the age group 18- 40 years were included in this study. The subjects were examined and the following indices were recorded • Oral hygiene index – Simplified (OHI-S). • Probing depths (PD). • Clinical Attachment Levels (CAL). • Gingival Index - Loe and Sillness. • Turesky Gillmore Glickman Modification of the Quigley Hein Plaque Index. (1970) • DMFT index. • Sickle Cell Disease Severity Index. A total of 1478 patients were screened of which 200 subjects were found to be diagnosed with SCD by electrophoresis. The study thus, included 200 subjects (111 females & 89 males) diagnosed with Sickle Cell Disease in the age group of 18-40 years. The probing pocket depths (PPD) were measured in millimeters. 36% had PPD in the range of 2-4mm, 48% had PPD in the range of 4-6mm while 16% had PPD of more than 6mm. Similar results were obtained for the Clinical Attachment Levels (CAL). 29.5 % subjects had CAL 2-4mm, 44.5% had 4-6mm & 26% had CAL 6mm & above. We can thus conclude that although oral health is not a priority for patients with SCD, it is supported by increased plaque accumulation. Because of the chronic anemic state of the patients with SCD, they should be encouraged to pay strict attention to oral hygiene instructions and practice.

Keywords: chronic, genetic, oral, sickle cell disease, vascular

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Authors: Anthara Vivek, Manisha Shukla, Mahesh Narayan, Prakash Narayan

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Sickle cell disease disproportionately affects sub-Saharan Africa and certain tribal populaces in India and has consequently drawn little intertest from Pharma. In sickle cell patients, adhesion of erythrocytes or reticulocytes to one another and the vessel wall results in painful ischemic episodes with few, if any, effective treatments for vaso-occlusive crises. Identification of disease-associated adhesion markers on erythrocytes or reticulocytes might inform the use of more effective therapies against vaso-occlusive crises. Increased expression of one or more of bcam, itga4, cd44, cd47, rap1a, vcam1, or icam4 has been reported in sickle cell subjects. Using the miRNet ontology knowledgebase, peripheral blood interactomes were generated by seeding various combinations of the afore-referenced mRNA. These interactomes yielded an array of miR targets. As examples, targeting hsa-miR-155-5p can potentially neutralize the rap1a-bcam-cd44-itga4-vcam1 erythrocyte/reticulocyte adhesion interactome whereas targeting hsa-miRs-103a-3p or 107 can potentially neutralize adhesion in cells overexpressing icam4-cd47-bcam-itga4-cd36. AM3380 (MIRacle™) is an off-the shelf hsa-miR-155-5p agomiR that can potentially neutralize the rap1a-bcam-cd44-itga4-vcam1 signaling axis. Phlebotomy coupled with transcriptomics represents a potentially feasible and effective precision medicine strategy to mitigate vaso-occlusive crises in sickle cell patients.

Keywords: adhesion, interactome, precision, medicine

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Authors: Nikhil A. Gavhane, Sachin Shah, Ishant S. Mahajan, Pawan D. Bahekar

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Introduction: Millions of people are affected with dengue fever every year, which drives up healthcare expenses in many low-income countries. Organ failure and other serious symptoms may result. Another worldwide public health problem is sickle cell anaemia, which is most prevalent in Africa, the Caribbean, and Europe. Dengue epidemics have reportedly occurred in locations with a high frequency of sickle cell disease, compounding the health problems in these areas. Aims and Objectives: This study examines dengue infection in sickle cell disease-afflicted pre-schoolers. Method:This Retrospective cohort study examined paediatric patients. Young people with sickle cell disease (SCD), dengue infection, and a control group without SCD or dengue were studied. Data on demographics, SCD consequences, medical treatments, and laboratory findings were gathered to analyse the influence of SCD on dengue severity and clinical outcomes, classified as severe or non-severe by the 2009 WHO classification. Using fever or admission symptoms, the research estimated acute illness duration. Result: Table 1 compares haemoglobin genotype-based dengue episode features in SS, SC, and controls. Table 2 shows that severe dengue cases are older, have longer admission delays, and have particular symptoms. Table 3's multivariate analysis indicates SS genotype's high connection with severe dengue, multiorgan failure, and acute pulmonary problems. Table 4 relates severe dengue to greater white blood cell counts, anaemia, liver enzymes, and reduced lactate dehydrogenase. Conclusion: This study is valuable but confined to hospitalised dengue patients with sickle cell illness. Small cohorts limit comparisons. Further study is needed since findings contradict predictions.

Keywords: dengue, chills, headache, severe myalgia, vomiting, nausea, prostration

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Authors: E. A. Alaswad, H. M. Choudhry, F. Z. Filimban

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Sickle Cell Anemia (SCA) is one type of blood diseases related to autosomal disorder. The sickle shaped red blood cells are the main cause of many problems in the blood vessels and capillaries. Aerva Javanica (J) and Ficus Palmata (P) are medicinal plants that have many popular uses and have been proved their efficacy. The aim of this study was to assess the antioxidants activity and the antisickling effect of J and P extractions. The period of this study, air-dried leaves of J, and P plants were ground and the active components were extracted by maceration in water (W) and methanol (M) as solvents. The antioxidants activity of JW, PW, JM, and PM were assessed by way of the radical scavenging method using 2,2-diphenyl-1-picrylhydrazyl (DPPH). To determine the antisickling effect of J and P extracts. 20 samples were collected from sickle cell anemia patients. Different concentrations of J and P extracts (200 and 110 μg/mL) were added on the sample and incubated. A drop of each sample was examined with light microscope. Normal and sickled RBCs were calculated and expressed as the percent of sickling. The stabilization effect of the extracts was measured by the osmotic fragility test for erythrocytes. The finding suggests as estimated by DPPH method, all the extracts showed an antioxidant activity with a significant inhibition of the DPPH radicals. PM has the least IC50% with 71.49 μg/ml while JM was the most with 408.49 μg/ml. Sickle cells treated with extracts at different concentrations significantly reduced the percentage of sickling compering to control samples. However, JM 200 μg/mL give the highest anti-sickling affect with 17.4% of sickling compared to control 67.5 of sickling while PM at 200 μg/mL showed the highest membrane cell stability. In a conclusion, the results showed that J and P extracts have antisickling effects. Therefore, the Aerva javanica and Ficus palmata may have a role in SCA management and a good impact on the patient's lives.

Keywords: Aerva javanica, antioxidant, antisickling, Ficus palmata, sickle cell anemia

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Authors: Mona Hamdy, Iman Shaheen, Hadeel Seif Eldin, Basma Ali, Omnia Abdeldayem

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Summary: Bone involvement of sickle cell disease (SCD) patients varies from acute clinical manifestations of painful vaso-occlusive crises or osteomyelitis to more chronic affection of bone mineral density (BMD) and debilitating osteonecrosis and osteoporosis. Secreted klotho protein is involved in calcium (Ca) reabsorption in the kidney. This study aimed to measure serum klotho levels in children with SCD to determine the possibility of using it as a marker of low BMD in children with SCD in correlation with a dual-energy radiograph absorptiometry scan. This study included 60 sickle disease patients and 30 age-matched and sex-matched control participants without SCD. A highly statistically significant difference was found between patients with normal BMD and those with low BMD, with serum Ca and klotho levels being lower in the latter group. Klotho serum level correlated positively with both serum Ca and BMD. Serum klotho level showed 94.9% sensitivity and 95.2% specificity in the detection of low BMD. Both serum Ca and klotho serum levels may be useful markers for detection of low BMD related to SCD with high sensitivity and specificity; however, klotho may be a better indicator as it is less affected by the nutritional and endocrinal status of patients or by intake of Ca supplements.

Keywords: sickle cell disease, BMD, osteoporosis, DEXA, klotho

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Authors: Vincent A. Adzika, Franklin N. Glozah, Collins S. K. Ahorlu

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Background: Sickle Cell Disease (SCD) is of major public-health concern globally, with majority of patients living in Africa. Despite its relevance, there is a dearth of research to determine the socio-demographic distribution and psychosocial impact of SCD in Africa. The objective of this study therefore was to examine the socio-demographic distribution and psychosocial consequences of SCD among patients in Ghana and to assess their quality of life and coping mechanisms. Methods: A cross-sectional research design was used, involving the completion of questionnaires on socio-demographic characteristics, quality of life of individuals, anxiety and depression. Participants were 387 male and female patients attending a sickle cell clinic in a public hospital. Results: Results showed no gender and marital status differences in anxiety and depression. However, there were age and level of education variances in depression but not in anxiety. In terms of quality of life, patients were more satisfied by the presence of love, friends, relatives as well as home, community and neighbourhood environment. While pains of varied nature and severity were the major reasons for attending hospital in SCD condition, going to the hospital as well as having Faith in God was the frequently reported mechanisms for coping with an unbearable SCD attacks. Multiple regression analysis showed that some socio-demographic and quality of life indicators had strong associations with anxiety and/or depression. Conclusion: It is recommended that a multi-dimensional intervention strategy incorporating psychosocial dimensions should be considered in the treatment and management of SCD.

Keywords: anxiety, depression, sickle cell disease, socio-demographic quality of life, characteristics, Ghana

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Authors: Z. Nurzaireena, K. Azalea, T. Azirawaty, S. Jameela, G. Muralitharan

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The aim of this study is the review of the management practices of sickle cell disease patients during pregnancy, as well as the maternal and neonatal outcome at Ampang Hospital, Selangor. The study consisted of a review of pregnant patients with sickle cell disease under follow up at the Hematology Clinic, Ampang Hospital over the last seven years to assess their management and maternal-fetal outcome. The results of the review show that Ampang Hospital is considered the public hematology centre for sickle cell disease and had successfully managed three pregnancies throughout the last seven years. Patients’ presentations, managements and maternal-fetal outcome were compared and reviewed for academic improvements. All three patients were seen very early in their pregnancy and had been given a regime of folic acid, antibiotics and thrombo-prophylactic drugs. Close monitoring of maternal and fetal well being was done by the hematologists and obstetricians. Among the patients, there were multiple admissions during the pregnancy for either a painful sickle cell bone crisis, haemolysis following an infection and anemia requiring phenotype- matched blood and exchange transfusions. Broad spectrum antibiotics coverage during and infection, hydration, pain management and venous-thrombolism prophylaxis were mandatory. The pregnancies managed to reach near term in the third trimester but all required emergency caesarean section for obstetric indications. All pregnancies resulted in live births with good fetal outcome. During post partum all were nursed closely in the high dependency units for further complications and were discharged well. Post partum follow up and contraception counseling was comprehensively given for future pregnancies. Sickle cell disease is uncommonly seen in the East, especially in the South East Asian region, yet more cases are seen in the current decade due to improved medical expertise and advance medical laboratory technologies. Pregnancy itself is a risk factor for sickle cell patients as increased thrombosis event and risk of infections can lead to multiple crisis, haemolysis, anemia and vaso-occlusive complications including eclampsia, cerebrovasular accidents and acute bone pain. Patients mostly require multiple blood product transfusions thus phenotype-matched blood is required to reduce the risk of alloimmunozation. Emphasizing the risks and complications in preconception counseling and establishing an ultimate pregnancy plan would probably reduce the risk of morbidity and mortality to the mother and unborn child. Early management for risk of infection, thromboembolic events and adequate hydration is mandatory. A holistic approach involving multidisciplinary team care between the hematologist, obstetricians, anesthetist, neonatologist and close nursing care for both mother and baby would ensure the best outcome. In conclusion, sickle cell disease by itself is a high risk medical condition and pregnancy would further amplify the risk. Thus, close monitoring with combine multidisciplinary care, counseling and educating the patients are crucial in achieving the safe outcome.

Keywords: anaemia, haemoglobinopathies, pregnancy, sickle cell disease

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Authors: V. A. Adzika, D. Ayim-Aboagye, T. Gordh

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Background and aims: Prevalence of Sickle Cell Disease (SCD) is high in Ghana but not much is known in terms of research into non-medical strategies for managing and coping with the pain associated with SCD. This study was carried out to examine effective non-medical related strategies patients use to cope and manage their SCD condition. Methods: SCD patients (387) consisting of 180 males and 204 females between 18-65 years old years participated in the study. A cross-sectional research design was used in which participants completed questionnaires on pain, non-medical coping and management strategies, anxiety, and depression. Results of multiple regression analysis showed that socio-demographic characteristics contributed to the variance in the pain associated with SCD. Results: Over 90% of participants reported that pains associated with SCD were the main reason for seeking treatment in SCD crisis. In terms of non-medical related coping strategies, attending a place of worship and praying were the main coping strategies used in SCD crises, suggesting that patients’ beliefs, particularly in a supernatural being, served as a mitigating factor in the process of coping with the pain associated with SCD crisis. Also, avoidance and withdrawal from people and social activities were reported to be strategies used to cope effectively with the pain associated with SCD crisis. Conclusion: This indicates that it is imperative to incorporate non-medical related coping and management strategies, especially religious beliefs and psychosocial factors, to coping and management of the pain associated with SCD.

Keywords: anxiety, depression, sickle cell disease, quality of life, socio-demographic characteristics, Ghana

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Authors: Anita Paddy, Millicent Obodai, Lebbaeus Asamani

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The management of sickle cell disease requires a multidisciplinary team for better outcomes. Thus, literature on the application of the biopsychosocial model for the management and explanation of chronic pain in sickle cell disease (SCD) and other chronic diseases abound. However, there is limited research on the use of the biopsychosocial model, together with a spiritual component (biopsychosocial-spiritual model). The study investigated the extent to which healthcare providers utilized the biopsychosocial-spiritual model in the management of chronic pain to improve the quality of life (QoL) of patients with SCD. This study employed the descriptive survey design involving a consecutive sampling of 261 patients with SCD who were between the ages of 18 to 79 years and were accessing hematological services at the Clinical Genetics Department of the Korle Bu Teaching Hospital. These patients willingly consented to participate in the study by appending their signatures. The theory of integrated quality of life, the gate control theory of pain and the biopsychosocial(spiritual) model were tested. An instrument for the biopsychosocial-spiritual model was developed, with a basis from the literature reviewed, while the World Health Organisation Quality of Life BREF (WHOQoLBref) and the spirituality rating scale were adapted and used for data collection. Data were analyzed using descriptive statistics (means, standard deviations, frequencies, and percentages) and partial least square structural equation modeling. The study revealed that healthcare providers had a great leaning toward the biological domain of the model compared to the other domains. Hence, participants’ QoL was not fully improved as suggested by the biopsychosocial(spiritual) model. Again, the QoL and spirituality of patients with SCD were quite high. A significant negative impact of spirituality on QoL was also found. Finally, the biosocial domain of the biopsychosocial-spiritual model was the most significant predictor of QoL. It was recommended that policymakers train healthcare providers to integrate the psychosocial-spiritual component in health services. Also, education on SCD and its resultant impact from the domains of the model should be intensified while health practitioners consider utilizing these components fully in the management of the condition.

Keywords: biopsychosocial (spritual), sickle cell disease, quality of life, healthcare, accra

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Authors: Wafa Al Jabri

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Sickle cell disease (SCD) is a genetic blood disorder that is characterized by a severe painful crisis. SCD among children requires long term dependencies and high caregiving demands that increase the overall family burdens. It is, therefore, essential to examine, support, and promote the well-being of families of children with SCD. Although there has been considerable progress in the international research on family quality of life (FQOL) in recent years; however, research in this field is relatively recent and diverse. Oman is a country in which family quality of life has definitely been under-researched. Therefore, the purpose of the study is to describe the FQOL in families of children with SCD in Oman. The study will also examine the relationships between child, mother, and family-related factors that may influence the overall FQOL. Theoretical Framework: The study is guided by the unified theory of family quality of life to help in understanding the concept of FQOL and the factors that shape it. Method:A convenience sample of 98 mothers of children with SCD will be recruited from the pediatric hematology clinic at Sultan Qaboos University Hospital in Oman to participate in this descriptive, cross sectional, correlational study. Data will be obtained using a self-administered questionnaire that includes child and mother socio-demographic data, questions about the number of visits and admissions to health care facilities for vaso- occlusive crises (VOCs), the Perceived Stress Scale-10, and the Beachcenter-FQOL scale. Anticipated Results: It is expected to find an association among frequency of VOCs, mother’s perceived stress level, and FQOL in families of children with SCD in Oman. Family type, socio-economic status, and number of SCD children in the family are also expected to influence the overall FQOL. Conclusion: The findings of the study might be pivotal in designing and implementing tailored family-based interventions to improve families’ wellbeing.

Keywords: family quality of life, sickle cell disaes, children, family well-being

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Authors: Lindy Adoma Dampare, Marina Aferiba Tandoh

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Sickle cell disease (SCD) is an inherited blood disorder that mostly affects individuals in sub-Saharan Africa. Nutritional deficiencies have been well established in SCD patients. In Ghana, studies have revealed the prevalence of malnutrition, especially amongst children with SCD and hence the need to develop an evidence-based comprehensive nutritional therapy for SCD to improve their nutritional status. The aim of the study was to develop and assess the effect of a nutrition education material on the nutritional status of SCD patients in Ghana. This was a pre-post interventional study. Patients between the ages of 2 to 60 years were recruited from the Tema General Hospital. Following a baseline nutrition knowledge (NK), beliefs, sanitary practice and dietary consumption pattern assessment, a twice-monthly nutrition education was carried out for 3 months, followed by a post-intervention assessment. Nutritional status of SCD patients was assessed using a 3-days dietary recall and anthropometric measurements. Nutrition education (NE) was given to SCD adults and caregivers of SCD children. Majority of the caregivers (69%) and SCD adult (82%) at baseline had low NK. The level of NK improved significantly in SCD adults (4.18±1.83 vs. 10.00±1.00, p<0.001) and caregivers (5.58 ± 2.25 vs.10.44± 0.846, p<0.001) after NE. Increase in NK improved dietary intake and dietary consumption pattern of SCD patients. Significant increase in weight (23.2±11.6 vs. 25.9±12.1, p=0.036) and height (118.5±21.9 vs. 123.5±22.2, p=0.011) was observed in SCD children at post intervention. Stunting (10.5% vs. 8.6%, p=0.62) and wasting (22.1% vs. 14.4%, p=0.30) reduced in SCD children after NE although not statistically significant. Reduction (18.2% vs. 9.1%) in underweight and an increase (18.2% vs. 27.3%) in overweight SCD adults was recorded at post intervention. Fat mass remained the same while high muscle mass increased (18.2% vs. 27.3%) at post intervention in SCD adult. Anaemic status of SCD patients improved at post intervention and the improvement was statistically significant amongst SCD children. Nutrition education improved the NK of SCD caregivers and adults hence, improving the dietary consumption pattern and nutrient intake of SCD patients. Overall, NE improved the nutritional status of SCD patients. This study shows the potential of nutrition education in improving the nutritional knowledge, dietary consumption pattern, dietary intake and nutritional status of SCD patients, and should be further explored.

Keywords: sickle cell disease, nutrition education, dietary intake, nutritional status

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Authors: Gehan Hussein, Hala Agha, Rasha Abdelraof, Marina George, Antoine Fakhri

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Background: β-Thalassemia major (TM) and sickle cell disease (SCD) are inherited hemoglobin disorders resulting in chronic hemolytic anemia. Cardiovascular involvement is an important cause of morbidity and mortality in these groups of patients. The narrow border is between overt myocardial dysfunction and clinically silent left ventricular (LV) and / or right ventricular (RV) dysfunction in those patients. 3 D Speckle tracking echocardiography (3D STE) is a novel method for the detection of subclinical myocardial involvement. We aimed to study myocardial affection in SCD and TM using 3D STE, comparing it with conventional echocardiography, correlate it with serum ferritin level and lactate dehydrogenase (LDH). Methodology: Thirty SCD and thirty β TM patients, age range 4-18 years, were compared to 30 healthy age and sex matched control group. Cases were subjected to clinical examination, laboratory measurement of hemoglobin level, serum ferritin, and LDH. Transthoracic color Doppler echocardiography, 3D STE, tissue Doppler echocardiography, and aortic speckle tracking were performed. Results: significant reduction in global longitudinal strain (GLS), global circumferential strain (GCS), and global area strain (GAS) in SCD and TM than control (P value <0.001) there was significantly lower aortic speckle tracking in patients with TM and SCD than control (P value< 0.001). LDH was significantly higher in SCD than both TM and control and it correlated significantly positive mitral inflow E, (p value:0.022 and 0.072. r: 0.416 and -0.333 respectively) lateral E/E’ (p value.<0.001and 0.818. r. 0.618 and -0. 044.respectively) and septal E/E’ (p value 0.007 and 0.753& r value 0.485 and -0.060 respectively) in SCD but not TM and significant negative correlation between LDH and aortic root speckle tracking (value 0.681& r. -0.078.). The potential diagnostic accuracy of LDH in predicting vascular dysfunction as represented by aortic root GCS with a sensitivity 74% and aortic root GCS was predictive of LV dysfunction in SCD patients with sensitivity 100% Conclusion: 3D STE LV and RV systolic dysfunction in spite of their normal values by conventional echocardiography. SCD showed significantly lower right ventricular dysfunction and aortic root GCS than TM and control. LDH can be used to screen patients for cardiac dysfunction in SCD, not in TM

Keywords: thalassemia major, sickle cell disease, 3d speckle tracking echocardiography, LDH

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Authors: H. Analuie, M. Faruque, S. Saha, H. Hashim, M. Muzaimi

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Though mental toughness has long been explored in sport psychology, much of our understanding on the topic remains largely unexplored. The concept is used widely, but empirical evidence is required to fully understand the construct and its related variables. This research investigated the correlation between mental toughness and trait anxiety to determine whether mentally tough athletes generally experience more or less anxiety. A sample of 57 men (M age = 25.4 years, s=4.66) and 45 women (M age = 23.5 years, s=5.73) participated in a variety of sports were recruited, where mental toughness was measured using MTQ48. Levels of trait anxiety were assessed using the State-Trait Anxiety Inventory (STAI). Series of Pearson correlations between trait anxiety, overall mental toughness, and the six subscales of mental toughness showed significant (p> .05) relationships. As predicted, greater mental toughness was associated with less reported trait anxiety. Independent t-tests found significant differences (p> .05) in overall mental toughness, the mental toughness subscales or trait anxiety between men and women. More research is required to understand how mentally tough athletes experience less anxiety in comparison to those who are not as mentally tough. Our findings suggest that relationships observed in this study emphasize the need for the inclusion of trait anxiety in mental toughness interventions.

Keywords: mental toughness, trait anxiety, MTQ48, sport psychology

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Authors: Caleb B. Sagwa, Tobias O. Okeno, Alexander K. Kahi

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Dairy cattle production plays an important role in the Kenyan economy. However, high incidences of mastitis is a major setback to the productivity in this industry. The current dairy cattle breeding objective in Kenya does not include mastitis resistance, mainly because the economic value of mastitis resistance has not been determined. Therefore this study aimed at estimating the economic value of mastitis resistance in dairy cattle in Kenya. Initial input parameters were obtained from literature on dairy cattle production systems in the tropics. Selection index methodology was used to derive the economic value of mastitis resistance. Somatic cell count (SCC) was used an indicator trait for mastitis resistance. The economic value was estimated relative to milk yield (MY). Economic values were assigned to SCC in a selection index such that the overall gain in the breeding goal trait was maximized. The option of estimating the economic value for SCC by equating the response in the trait of interest to its index response was considered. The economic value of mastitis resistance was US $23.64 while maximum response to selection for MY was US $66.01. The findings of this study provide vital information that is a pre-requisite for the inclusion of mastitis resistance in the current dairy cattle breeding goal in Kenya.

Keywords: somatic cell count, milk quality, payment system, breeding goal

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Authors: Jacob Hornik, Matti Rachamim, Ori Grossman

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Schadenfreude, a bittersweet phenomenon, is considered atypical and complicated state that might reflect ambivalent types of sentiments -a mixed of both positive and negative reactions towards others misfortunes. This brief note reports a study that examined the association between trait ambivalence, using the Trait Mixed Emotions Scale (TMES), and four different consumer schadenfreude affairs. Results propose that trait ambivalence offers a novel explanation for schadenfreude responses. Showing that trait ambivalence enhances schadenfreude, when consumers encounter misfortune type of information about a disliked or rival marketplace entity.

Keywords: schadenfreude, consumer behavior, mixed emotions, sentiments, ambivalence

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Authors: Himali Gunasekara, Baddika Jayaratne

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Haematological abnormalities are known to cause Ischemic Stroke or Transient Ischemic Attack (TIA). The identification of haematological correlates plays an important role in a management and secondary prevention. The objective of this study was to describe haematological correlates of stroke and their association between stroke profile. The haematological correlates screened were Lupus Anticoagulant, Dysfibroginemia, Paroxysmal nocturnal haemoglobinurea (PNH), Sickle cell disease, Systemic Lupus Erythematosis (SLE) and Myeloploriferative Neoplasms (MPN). A cross sectional descriptive study was conducted in a sample of 152 stroke patients referred to haematology department of National Hospital of Sri Lanka for thrombophilia screening. Different tests were performed to assess each hematological correlate. Diluted Russels Viper Venom Test and Kaolin clotting time were done to assess Lupus anticoagulant. Full blood count (FBC), blood picture, Sickling test and High Performance Liquid Chromatography were the tests used for detection of Sickle cell disease. Paroxysmal nocturnal haemoglobinurea was assessed by FBC, blood picture, Ham test and Flowcytometry. FBC, blood picture, Janus Kinase 2 (V617F) mutation analysis, erythropoietin level and bone marrow examination were done to look for the Myeloproliferative neoplasms. Dysfibrinogenaemia was assessed by TT, fibrinogen antigen test, clot observation and clauss test. Anti nuclear antibody test was done to look for systemic lupus erythematosis. Among study sample, 134 patients had strokes and only 18 had TIA. The recurrence of stroke/TIA was observed in 13.2% of patients. The majority of patients (94.7%) have had radiological evidence of thrombotic event. One fourth of patients had past thrombotic events while 12.5% had family history of thrombosis. Out of haematological correlates screened, Lupus anticoagulant was the commonest haematological correlate (n=16 ) and dysfibrigonaemia(n=11 ) had the next high prevalence. One patient was diagnosed with Essential thrombocythaemia and one with SLE. None of the patients were positive for screening tests done for sickle cell disease and PNH. The Haematological correlates were identified in 19% of our study sample. Among stroke profile only presence of past thrombotic history was statistically significantly associated with haematological disorders (P= 0.04). Therefore, hematological disorders appear to be an important factor in etiological work-up of stroke patients particularly in patients with past thrombotic events.

Keywords: stroke, transient ischemic attack, hematological correlates, hematological disorders

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Authors: Gabrielle Veloso, Welison Ty

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Trait anxiety is a pervasive tendency to attend to and experience fears and worries to a disproportionate degree, across various situations. This study sought to determine if participants who undergo emotional working memory training will have significantly lower scores on the trait anxiety scales post-intervention. The study also sought to determine if emotional regulation mediated the relationship between working memory training and trait anxiety. Forty-nine participants underwent 20 days of computerized emotional working memory training called Emotional Dual n-back, which involves viewing a continuous stream of emotional content on a grid, and then remembering the location and color of items presented on the grid. Participants of the treatment group had significantly lower trait anxiety compared to controls post-intervention. Mediation analysis determined that working memory training had no significant relationship to anxiety as measured by the Beck’s Anxiety Inventory-Trait (BAIT), but was significantly related to anxiety as measured by form Y2 of the Spielberger State-Trait Anxiety Inventory (STAI-Y2). Emotion regulation, as measured by the Emotional Regulation Questionnaire (ERQ), was found not to mediate between working memory training and trait anxiety reduction. Results suggest that working memory training may be useful in reducing psychoemotional symptoms rather than somatic symptoms of trait anxiety. Moreover, it proposes for future research to further look into the mediating role of emotion regulation via neuroimaging and the development of more comprehensive measures of emotion regulation.

Keywords: anxiety, emotion regulation, working-memory, working-memory training

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Authors: Marialena Kostouli, Georgia Tsoulfa

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The aim of this study is to investigate the relationship between general self-efficacy, perfectionism, and gifted students’ trait anxiety. One hundred fifty three students, who were all selected and enrolled at the Center for Talented Youth (CTY) - Greece summer program, participated in the study. The sample consisted of 78 males (51%) and 75 females (49%), with a mean age of 14.96 years (SD = 1.16 years). Three self-report questionnaires were used for the purposes of the current study, the Frost Multidimensional Perfectionism scale, the State-Trait anxiety inventory and the General Self-Efficacy scale. The results revealed a significant correlation between trait anxiety, general self-efficacy and the four sub-scales of perfectionism (concern over mistakes and doubts about actions, excessive concern with parents’ expectations and evaluation, excessively high personal standards and concern with precision, order, and organization). It was also found that the female CTY students experience greater levels of trait anxiety compared to the male CTYers. Moreover, a multiple regression analysis was conducted in order to determine the possible predictors of gifted students’ trait anxiety. The analysis showed that general self-efficacy and the concern over mistakes and doubts about actions significantly predicted the trait anxiety of the gifted children that we examined. Avenues of further research and implications for the development of interventions to help gifted students promote their general self-efficacy, reduce their concern over their actions and develop strategies in order to cope with their anxiety are discussed.

Keywords: general self-efficacy, gifted students, perfectionism, trait anxiety

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Authors: Mhaned Oubounyt, Jan Baumbach

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Differences in co-expression networks between two or multiple cells (sub)types across conditions is a pressing problem in single-cell RNA sequencing (scRNA-seq). A key challenge is to define those co-variations that differ between or among cell types and/or conditions and phenotypes to examine small regulatory networks that can explain mechanistic differences. To this end, we developed SCANet, an all-in-one Python package that uses state-of-the-art algorithms to facilitate the workflow of a combined single-cell GCN (Gene Correlation Network) and GRN (Gene Regulatory Networks) pipeline, including inference of gene co-expression modules from scRNA-seq, followed by trait and cell type associations, hub gene detection, co-regulatory networks, and drug-gene interactions. In an example case, we illustrate how SCANet can be applied to identify regulatory drivers behind a cytokine storm associated with mortality in patients with acute respiratory illness. SCANet is available as a free, open-source, and user-friendly Python package that can be easily integrated into systems biology pipelines.

Keywords: single-cell, co-expression networks, drug-gene interactions, co-regulatory networks

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Authors: Crystal Jewell

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Substantial evidence suggests a link exists between trait or dispositional mindfulness and creativity. While most studies on the mindfulness-creativity link focus on measures of divergent thinking, no study to date has explored the link through the lens of poetry writing. Thus, the present study sought to examine the relation between mindfulness and poetry through various linguistic elements, including word count, references to the self versus references to the collective, and frequency of past-, present-, and future-tense verb usage. Following a questionnaire on demographics, university undergraduates at a United States college completed a survey measuring trait mindfulness, then engaged in a two-part associated poetry-writing task intended to mimic writing tasks used to counter writer’s block. Results indicated no significant relations among any measures of poetry linguistic elements and trait mindfulness, as well as the facets of trait mindfulness. Limitations and future directions call for replication of results and further examination of different poetry linguistic elements.

Keywords: mindfulness, poetry, linguistics, psychology

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Authors: Shian-Ling Keng, Onn Siong Yim, Poh San Lai, Soo Chong Chew, Anne Chong, Richard Ebstein

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Research has demonstrated a positive association between mindfulness meditation and physical health. Little work, however, has examined the association between trait mindfulness and leukocyte telomere length (LTL), an emerging marker of cellular aging. The present study aimed to examine whether facets of trait mindfulness are correlated with longer LTL in a Singaporean Han Chinese sample and whether these facets may mediate the association between psychological symptoms and LTL. 158 adults (mean age = 27.24 years) completed measures assessing trait mindfulness and psychological symptoms (i.e., depression and stress) and provided blood samples for analyses of LTL using qPCR. Multiple regression analyses were conducted to assess the association between facets of trait mindfulness and LTL. Bootstrapping-based mediational analyses were run to examine the role of trait mindfulness as a mediator of the association between psychological symptoms and LTL. Of five facets of trait mindfulness (describe, act with awareness, observe, nonreactivity, and nonjudging), nonreactivity was significantly associated with LTL, after controlling for the effects of age, gender, and education, β = .21, p = .006. Further, there was a trend for overall trait mindfulness, β = .15, p = .06, and nonjudging, β = .13, p = .095, to each predict longer LTL. Nonreactivity significantly mediated the association between depression and LTL, BCa 95% CI [-.004, -.0004], p=.03, as well as the association between stress and LTL, BCa 95% CI [-.004, -.0004], p=.04. The results provide preliminary evidence for a positive association between selected facets of trait mindfulness and slower cellular aging, indexed by LTL. The findings suggest that individuals who are high on equanimity may experience slower aging at the cellular level, presumably through engaging in more effective coping mechanisms and modulation of stress. The findings also highlight the role of nonreactivity as a potential mechanism that underlies the association between LTL and psychological symptoms.

Keywords: depression, mindfulness, stress, telomere length

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Authors: Nurul Azmawati Mohamed, Zarini Ismail, Shalinawati Ramli, Nurul Hayati Chamhuri, Nur Syahrina Rahim, K. Omar

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Choosing a career is one of the most important decisions in our life. A right career leads a person to grow with that career and achieve success through the decision. Thus, career suitability assessment is important to help individuals to understand how a variety of personal attributes can impact their potential success and satisfaction with different career options and work environments. Some career needs specific personality trait that relates to attributes of job requirements and commitments. For medicine, being caring, approachable, inquisitive, able to listen and understand patients’ pain, anxiety and sorrow are important. The aim of this study was to evaluate the career suitability of pre-clinical students. This was a cross sectional study conducted among pre-clinical medical students in Universiti Sains Islam Malaysia. 'Sidek Career Interest Inventory’ was used to assess the students’ suitability for the course. This instrument had been validated locally to suit the local social and cultural context. It assessed the students’ personality trait based on Holland’s theory and their interests. For students to pursue in the medical course, two main personality trait are believed to be essential namely investigative and social trait personalities. Some of the characteristics of investigative trait are analytical, rational, intellectual and curious, while the characteristics of social trait personality include empathy, friendly, understanding and accommodating. The score for each personality trait were categorized as low (0-3.99), moderate (4-6.99) and high (7-10). A total of 81 pre-clinical medical students were included in this study. About two third (93.8%) of them were female and all of them are from 20 to 21 of age. Approximately, half of the students (47.5%) scored high and another 46.3% scored moderate for investigative trait. For social trait, only 13.8% scored high while 31.3% scored moderate. Only 12.5% (10) students had high scores for both investigative and social traits. Most of the pre-clinical medical students scored high in the investigative sections, however their social values were inadequate (low scores). For them to become good medical doctors, they should be good in both investigative and social skills to enhance their suitability for this career. Therefore, there is a need to nurture these medical students with appropriate social values and soft skills.

Keywords: career suitability, career interest, medical students, personality trait

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Authors: Alexander N. Savostyanov, Tatiana A. Dolgorukova, Elena A. Esipenko, Mikhail S. Zaleshin, Margherita Malanchini, Anna V. Budakova, Alexander E. Saprygin, Tatiana A. Golovko, Yulia V. Kovas

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EEG correlates of mathematical and trait anxiety level were studied in 52 healthy Russian-speakers during execution of error-recognition tasks with lexical, arithmetic and algebraic conditions. Event-related spectral perturbations were used as a measure of brain activity. The ERSP plots revealed alpha/beta desynchronizations within a 500-3000 ms interval after task onset and slow-wave synchronization within an interval of 150-350 ms. Amplitudes of these intervals reflected the accuracy of error recognition, and were differently associated with the three conditions. The correlates of anxiety were found in theta (4-8 Hz) and beta2 (16-20 Hz) frequency bands. In theta band the effects of mathematical anxiety were stronger expressed in lexical, than in arithmetic and algebraic condition. The mathematical anxiety effects in theta band were associated with differences between anterior and posterior cortical areas, whereas the effects of trait anxiety were associated with inter-hemispherical differences. In beta1 and beta2 bands effects of trait and mathematical anxiety were directed oppositely. The trait anxiety was associated with increase of amplitude of desynchronization, whereas the mathematical anxiety was associated with decrease of this amplitude. The effect of mathematical anxiety in beta2 band was insignificant for lexical condition but was the strongest in algebraic condition. EEG correlates of anxiety in theta band could be interpreted as indexes of task emotionality, whereas the reaction in beta2 band is related to tension of intellectual resources.

Keywords: EEG, brain activity, lexical and numerical error-recognition tasks, mathematical and trait anxiety

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Authors: A. Nurgiartiningsih, G. Ciptadi, S. B. Siswijono

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This research was done to identify the performance and breeding potency of Local Buffalo in Kangean Island, Sumenep, East Java, Indonesia. Materials used were buffalo and farmer in Kangean Island. Method used was survey with purposive sampling method. Qualitative trait and existing breeding system including the type of production system were directly observed. Quantitative trait consisted of chest girth, body weight and wither height were measured and recorded. Data were analyzed using analysis of variance applying software GENSTAT 14. Results showed the purposes of buffalo breeding in Kangean Island were for production of calves, saving, religion tradition, and buffalo racing. The combination between grazing and cut and carry system were applied in Kangean Island. Forage, grass and agricultural waste product were available abundantly especially, during the wet season. Buffalo in Kangean Island was categorized as swamp buffalo with 48 chromosomes. Observation on qualitative trait indicated that there were three skin color types: gray (81.25%), red (10.42%) and white/albino (8.33%). Analysis on quantitative trait showed that there was no significant difference between male and female buffalo. The performance of male buffalo was 132.56 cm, 119.33 cm and 174.11 cm, for the mean of body length, whither height and chest girth, respectively. The performance of female buffalo were 129.8 cm, 114.0 cm and 166.2 cm, for mean of body length, wither height and chest girth (CG), respectively. The performance of local buffalo in Kangean Island was categorized well. Kangean Island could be promoted as center of buffalo breeding and conservation. For optimal improvement of population number and its genetics value, government policy in buffalo breeding program should be implemented.

Keywords: chromosome, qualitative trait, quantitative trait, swamp buffalo

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