Fostering Medical Compliance in Some Nigerian Sickle Cell Disease Patients

1992 ◽  
Vol 23 (1) ◽  
pp. 33-37
Author(s):  
O.B. Boroflice
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Positive Attitude ◽  
Cell Disease ◽  
Control Groups ◽  
College Hospital ◽  
Lecture Method ◽  
Medical Compliance ◽  
The University ◽  
Ibadan Nigeria

This study investigated the effect of an education program on the attitude toward service personnel and medication of sickle cell disease patients. Subjects were sixty registered sickle cell disease patients at the University College Hospital, Ibadan, Nigeria. The subjects were randomly assigned to two treatment and one control groups. The first treatment group was exposed to the lecture method while the second had in addition, exposure to group interaction. The education protocol used included lectures on the cause, prognosis, prevention of sickle cell disease, and self care skills. The results showed that both methods were effective in enhancing a positive attitude.

scholarly journals Prenatal Diagnosis in Sickle Cell Disease: In the Eyes of the Couple at Risk

2020 ◽  
pp. 65-71
Author(s):  
Okechukwu Chioma
Keyword(s):  
At Risk ◽  
Prenatal Diagnosis ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Health Care Professionals ◽  
Cell Disease ◽  
Hospital Data ◽  
Port Harcourt ◽  
Knowledge And Attitude ◽  
The University

Background: Prenatal diagnosis of sickle cell disorders provides a couple at risk the opportunity to make informed decisions whether or not to birth a child with Sickle Cell Disease (SCD). Aim: To explore the knowledge of prenatal diagnosis and its acceptability among parents at risk of having another child with SCD Methods: A self-administered structured questionnaire was used to obtain information on socio-demographics, knowledge and attitude towards prenatal diagnosis. The respondents were parents of SCD patients seen at the haematology consultant paediatric clinic of the University of Port Harcourt Teaching hospital. Data were analysed using SPSS version 20.0. Results: Forty-six parents were interviewed and they were all Christians. Thirty-two (69.6%) were females and fourteen (30.4%) were males. All the respondents were from the south-south region of Nigeria. Four (8.7%) had lost children from complications of SCD. Twenty-four (52.2%), had heard about prenatal diagnosis of sickle cell disease while twelve (50%) of them heard about it from health care professionals. Four (8.7%) had done prenatal diagnosis in previous pregnancies while 32 (69.6%) were willing to do it in their next pregnancy. The most common reasons given for not doing prenatal diagnosis were religious beliefs, personal beliefs and fear of the procedure. Conclusion: There is still a gap in knowledge and utilization of prenatal diagnosis by at risk couples. Appropriate information and regular counselling should be given to at risk parents as a key method of preventing SCD.

Red Cell Transport Abnormalities As Predictors of the Development of Renal Disease in Children with Sickle Cell Disease

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 1317-1317
Author(s):  
Sanjay Tewari ◽  
John Gibson ◽  
Stephan Menzel ◽  
Anke Hannemann ◽  
Swee Lay Thein ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Conflicts Of Interest ◽  
Cation Transport ◽  
Transport Systems ◽  
Common Finding ◽  
Early Event ◽  
Cell Disease ◽  
College Hospital ◽  
Transport Pathways

Abstract Background: Patients with sickle cell disease (SCD) have the abnormal haemoglobin (Hb) HbS in their red blood cells (RBCs). The complications of SCD may be extensive but are markedly variable between patients. Renal damage (sometimes called sickle cell nephropathy, SCN) is a common finding in about a third of children with SCD, a significant number of whom will proceed to renal failure. As yet, it is not possible to predict which children will develop SCN and who would therefore benefit from earlier and aggressive management. Various biomarkers have been investigated but none have proved efficacious. Changes in RBC membrane permeability are closely linked with polymerisation of HbS and, as such, may represent an important early event in pathogenesis. We postulated that certain RBC characteristics (sickling, K+ transport, PS exposure), which may be inherited independently of the HbS mutation, may correlate with renal pathology, but more importantly, may be identifiable in advance of damage, thereby providing prognostic markers to inform patient management. Participants and Methods: Children (>4 years old) with sickle cell anaemia (SCA) were recruited from the paediatric haematology clinic at King's College Hospital in South London, UK. Patients who had received blood transfusion in the preceding four months or who were on medications known to directly alter RBC cation transport (e.g. dipyridamole and Ca2+ channel blockers) were excluded. Patients on hydroxyurea were included. All patients were in steady state, with no acute symptoms requiring treatment for at least seven days. Clinical and laboratory parameters were collected prospectively at routine clinical attendance. Details of enuresis were collected using specifically developed proforma and patients were divided into two groups: those who stopped wetting their bed before the age of five years and those who were still enuretic after five years of age. RBC permeability was assessed using 86Rb+ as a congener for K+ to measure the activity of main cation transport systems involved in RBC dehydration. The activity of RBC transport pathways and percentage of sickling was measured at 100mmHg, 35mmHg, 15mmHg and 0mmHg. Statistical analysis was conducted using IBM-SPSS, New York. Results: 112 patients of HbSS genotype (SCA) consented to take part in the study. Their clinical profile is summarised Table 1. The percentage of children with enuresis, hypertension and significant albuminuria is shown in Table 2. There was significant correlation between activity of Gardos channel at O2 tensions of 35mmHg, 15mmHg and 0mmHg and enuresis after the age of five years. Psickle activity also showed significant correlation but only in fully deoxygenated RBCs (O2 of 0mmHg.) Table 3. Conclusion In conclusion, the present findings emphasise the significance of a thorough appreciation of RBC permeability to the pathogenesis of SCD. Whilst preliminary, these findings are exciting because they show that the activity of a particular transport pathways, abnormally elevated in SCA patients, are associated with enuresis. These findings suggest that the early identification of increased Gardos and Psickle activity may identify children who would benefit from early treatment for nocturnal enuresis. Disclosures No relevant conflicts of interest to declare.

scholarly journals Prevalence of sickle cell disease among Grenadian newborns

2017 ◽  
Vol 25 (1) ◽  
pp. 49-50 ◽  
Author(s):  
Magdalene Antoine ◽  
Ketty Lee ◽  
Tyhiesia Donald ◽  
Yonni Belfon ◽  
Ali Drigo ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Neonatal Screening ◽  
Screening Programme ◽  
Population Based ◽  
University Hospital ◽  
Cell Disease ◽  
Diagnostic Laboratory ◽  
Birth Prevalence ◽  
The University

Objective To establish the birth prevalence of sickle cell disease in Grenada, with a view to assess the requirement for a population-based neonatal screening programme. Methods A two-year pilot neonatal screening programme, involving the Ministry of Health of Grenada, the Sickle Cell Association of Grenada, and the diagnostic laboratory of hemoglobinopathies of the University Hospital of Guadeloupe, was implemented in 2014–2015 under the auspices of the Caribbean Network of Researchers on Sickle Cell Disease and Thalassemia. Results Analysis of 1914 samples processed identified the following abnormal phenotypes: 10 FS, 2 FSC, 183 FAS, 63 FAC. These data indicate βs and βc allele frequencies of 0.054 and 0.018, respectively. Conclusion Neonatal screening conducted in the framework of this Caribbean cooperation can allow rapid detection and earlier management of affected children.

Lung Function Tests in Sickle-Cell Patients in Benin City

2012 ◽  
Vol 2012 ◽  
pp. 1-5 ◽  
Author(s):  
J. N. Oko-Ose ◽  
V. Iyawe ◽  
E. Egbagbe ◽  
M. Ebomoyi
Keyword(s):  
Sickle Cell Disease ◽  
Lung Function ◽  
Sickle Cell ◽  
Pearson Correlation ◽  
Stable State ◽  
Forced Expiratory Volume ◽  
World Health ◽  
Cell Disease ◽  
Control Groups ◽  
Function Test

Background. Sickle-cell disease (SCD) is a life-long haematological disorder characterized by red blood cells that assume an abnormal, rigid, sickle shape and with high prevalence in West Africa. Sickle cell disease (SCD) is one of the most common genetic disorders among Africans as indicated in Akinyanju (1989). 61.4% of the children born annually with haemoglobinopathies worldwide have been reported to have SCD and they are from Africa as shown in World Health Organization Report (1987). Hypoxaemia is the hallmark of pulmonary abnormality in SCD patients of all age groups. This was first documented by Klinefelter (1942), and has since been corroborated by other workers. The hypoxaemia of stable SCD patients is said to result from the combined effects of perfusion and diffusion defect. Materials and Methods. This study was carried out to determine the changes in lung functions in relation to gender, age, and body mass index (BMI) of sickle-cell patients in a stable state. 60 subjects made up of 30 patients and 30 control groups were used in this study. Both test and control groups were 13 men and 17 women, aged 19–35 years. Lung Function Test was done with the aid of a digital spirometer (Spirolab II, Italy). Weight was measured using Avery scales (Avery Berkel, 2003, UK). Height was measured using a wall-mounted stadiometer. Data were collected and analyzed using Student's t-test and Pearson correlation. was statistically significant. Results. The results revealed that adults with SCD had a significantly lower mean forced expiratory volume in one second (FEV1) and forced vital capacity (FVC) compared to the control. The lung function indices were lower in females than males in the sickle cell patients. The FVC, FEV1, and FEV1/FVC correlated positively with BMI statistically in the patients. The lung function declined with age. This work has also shown that the most common pulmonary function test (PFT) abnormality was restrictive disease pattern (76.7%). Conclusion. The result of this work suggested that lung function differs significantly in subjects with SCD compared with matched controls of a similar age and gender.

Florida's Experience With Newborn Screening

PEDIATRICS ◽  
1989 ◽  
Vol 83 (5) ◽  
pp. 861-863
Author(s):  
Astrid K. Mack
Keyword(s):  
At Risk ◽  
Informed Consent ◽  
Sickle Cell Disease ◽  
Cord Blood ◽  
Sickle Cell ◽  
Fiscal Year ◽  
Cell Disease ◽  
University Of Miami ◽  
National Cooperative ◽  
The University

Cord blood screening of newborns for sickle cell disease was initiated in Florida at the Miami Sickle Cell Clinical Center in 1979 as a part of its participation in the national Cooperative Study of Sickle Cell Disease. Funding for newborn sickle cell screening in Florida began as a pilot project with a line-item appropriation in fiscal years 1983/84, and this funding for the three medical schools (University of Florida, University of Miami, and University of South Florida) continues to the present. During fiscal year 1984/85, a contract was negotiated between the University Hospital at Jacksonville and the local Children's Medical Services' office for the provision of newborn sickle cell screening. This contract has been renewed each year to the present. The primary objective in each of these programs is to identify babies with clinically significant hemoglobinopathies and to offer the services of the institution's pediatric hematology division. Additionally, education, genetic counseling, family studies, and appropriate referrals are provided for all babies found to have any hemoglobinopathy and for their family members. Initially, at the University of Miami, cord blood specimens were obtained at the time of delivery from at-risk (mostly black) mothers from whom informed consent had been obtained during a visit to one of several prenatal clinics associated with the University of Miami/Jackson Memorial (UM/ JM) Medical Center. It was soon discovered that many at-risk expectant mothers did not attend a prenatal clinic before their babies were born. Our internal review board did not consider consent "at delivery" informed consent.

Role of Hypoxia Induced Factor (HIF-1 alpha) in Pulmonary Hypertension in Sickle Cell Disease

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 2493-2493
Author(s):  
Mukta Kumar ◽  
Xuhui Zhu ◽  
Shilpa Buch
Keyword(s):  
Pulmonary Hypertension ◽  
Growth Factor ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Target Genes ◽  
Rna Isolation ◽  
Rna Expression ◽  
Cell Disease ◽  
Control Groups

Abstract Background. Pulmonary hypertension is a leading cause of mortality in sickle cell disease (SCD), however, the mechanisms leading to the development of pulmonary hypertension remain poorly understood. The pathogenesis of SCD revolves around chronic hypoxia and hypoxic events that are mediated through the upregulation of hypoxia-inducible factor-1alpha (HIF-1alpha), a transcriptional factor, that is involved in regulation of several redox sensitive genes including vascular endothelial growth factor (VEGF), platelet derived growth factor (PDGF) and ApoE. The objective of this study was to examine the role of HIF-1 and its target genes in SCD-associated pulmonary lung disease. Methods. In this study, 3 groups of age-matched homo and hemizygous sickle and control C57/Bl6 mice (Jackson Labs) were sacrificed and organs harvested for RNA isolation in Trizol. Using quantitative RT-PCR we assessed the RNA expression of HIF-1 and its target genes, VEGF and PDGF-B from the lungs of homozygous sickle mice and compared these with the C57/BL6 and the hemizygous sickle controls. HPRT was used as the housekeeping gene control. Results. Homozygous sickle mice demonstrated an upregulation of HIF-1 alpha RNA (almost 2.3 fold) compared with the hemizygous sickle and the C57/Bl6 controls. VEGF RNA expression was elevated ~8.3 to 10.9 fold in sickle mice compared to the two control groups. Interestingly there was a down regulation of PDGF-B expression (0.3 fold) in the sickle mice group compared to the control groups. In contrast, expression of all the HIF-related genes tested was down-regulated in the spleens and brains of sickle mice compared with the respective control tissues. Discussion. These findings support the possibility that complex molecular pathways, including dysregulation of HIF pathway, may contribute to the development of pulmonary hypertension in sickle cell disease and underscore role of oxidative stress pathways in the onset of disease pathogenesis.

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