Management of Sickle Cell Disease: A Review for Physician Education in Nigeria (Sub-Saharan Africa)

Adewoyin, Ademola Samson

doi:10.1155/2015/791498

Cited by 139 publications

(150 citation statements)

References 165 publications

(221 reference statements)

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“…Therefore, patients can develop both conditions simultaneously. Patients who have sickle cell/β-thalassemia typically have symptoms similar to sickle cell anemia, though the intensity of the anemia can vary depending on the severity of the thalassemia [12]. Increased research in population genetics of hematological disorders over the past few years have enabled better understanding of the genetic factors responsible for progression and severity of anemia in Hb E/β-thalassemia [13].…”

Section: Discussionmentioning

confidence: 99%

Prenatal Screening for Co-Inheritance of Sickle Cell Anemia and β-Thalassemia Traits

Dhawan¹,

Chaudhary²,

Chandratre³

et al. 2016

Clin Med Biochemistry Open Access

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Co-inheritance of sickle cell anemia and β-thalassemia traits require medical attention. Individuals with sickle cell and β-thalassemia disorders produce abnormal form of hemoglobin or decreased synthesis or complete absence of the β-globin chains of hemoglobin. Therefore, affected individuals might require blood transfusions at regular intervals. Prenatal diagnosis of fetal hemoglobinopathy should be offered when the fetus is at risk of being affected. The aim of this study was to assess the applicability of the nucleotide sequencing method in the identification of both diseases and to identify and counsel asymptomatic parents to make reproductive choices. We demonstrate the ability to detect these traits in a family, which was suspected to be carrying β-thalassemia mutations as per the clinician.

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Section: Discussionmentioning

confidence: 99%

Prenatal Screening for Co-Inheritance of Sickle Cell Anemia and β-Thalassemia Traits

Dhawan¹,

Chaudhary²,

Chandratre³

et al. 2016

Clin Med Biochemistry Open Access

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“…In addition to the above factors, there is small evidence that the SCD genotype is associated with STA, since the frequency of this complication is high in patients who have hemoglobin SC and Hbs-ß [6,26]. Accordingly, studies suggest that the HLA haplotype of DRB1 *130101-DQB1* 060101 has been shown to be strongly associated with the development of STA when compared to other [27].…”

Section: Acute Thoracic Syndromementioning

confidence: 99%

“…As a result, red blood cells are prone to fail and agglutinate in states of low oxygen saturation. A range of complications arising from this condition may occur, such as chronic hemolytic anemia resulting from the premature removal of red blood cells from the circulation, vaso-occlusion caused by erythrocytic deposition in small vessels or bifurcations, increased blood viscosity, predisposition to primary and recurrent infections In addition to advanced stage organ failure [2,6,7]. The occurrence of such events is correlated with the propensity to develop a serious complication, the acute thoracic syndrome (STA).…”

Section: Introductionmentioning

confidence: 99%

Therapeutic Approaches of Acute Thoracic Syndrome in Patients with Falciform Disease

Ac¹,

Costa²,

Tec³

et al. 2017

J Blood Lymph

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Sickle cell disease (SCD) is an autosomal recessive inheritance disorder that affects the beta-globin gene and results in the replacement of the amino acid glutamic acid by valine in the β chain of the hemoglobin molecule, producing erythrocytes with defective forms and functions. A range of pathological conditions is associated with SCD, however, one in particular stands out by gravity. Acute thoracic syndrome (STA), characterized by the presence of pulmonary infiltrates associated with a clinical symptom such as chest pain, cough, wheezing, tachypnea and fever, is considered a leading cause of death in patients with sickle cell disease. Early diagnosis and introduction of an effective approach to complication are needed to improve outcomes and minimize associated morbidity and mortality. Risk factors associated with STA in the SCDAbdominal surgery for splenectomy and cholecystectomy [19] Hypersensitivity [20,21] Smoking [20,21] Asthma [22] Infections [9,[23][24][25] Post-Surgical Hypoventilation [9,[23][24][25] Fatty Embolism [9,[23][24][25]

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“…In corroboration of this, Alege [2] posited that Nigeria has the largest population of people with sickle cell disease in the world with over 150,000 babies born with serious condition every year. Nigeria bears the greatest burden of sickle cell in Sub-Saharan Africa (Adewoyin) [3] with forty million people carrying the gene for sickle cell disease in the country (Alhassan) [4]. Sickle cell disease can be in several forms which include sickle cell anaemia (when inherited in homozygous state), sickle cell beta plus thalassemia, sickle beta zero thalassemia and others (Adewoyin).…”

Section: Introductionmentioning

confidence: 99%

The Health Workers Perspectives in the Management of Sickle Cell Disease in an Urban Health Centre in Ile-Ife, Nigeria

Muoghalu¹,

Awolowo²

2017

J Hematol Thrombo Dis

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Management of sickle cell disease is key to improved quality of life of people living with the disease in Nigeria. This paper examined management strategies utilized by health workers in managing sickle cell patients in Obafemi Awolowo University health centre, in Ile-Ife, Nigeria. The specific objectives were to explore health workers definition of the disease, the observed symptoms by the health workers, drugs and non-drug strategies employed by these health workers in the management of the patients and the adequacy of health facilities for managing sickle cell patients. With structured in-depth interview schedule, fifteen health workers including nurses and doctors were interviewed. It was indicated that unanimously, health workers defined the disease as an inherited blood disorder which is highly symptomatic with a lot pains all over the body especially in joints and chest and that the sufferers are often admitted in the hospital. Also, the health workers use antibiotics, folic acid, analgesics, anti-malaria drugs, intravenous fluids and blood transfusion as drug regimens for managing the sufferers. The non-drug strategies employed by the health workers were counselling, education for self-care and psychotherapy. Furthermore, the health workers indicated that inadequate health facilities hamper their capacity to deliver effective management to people living with the disease. The paper concludes that health workers employ both drug and non-drug management strategies that promise improved quality of life for sickle cell patients in Nigeria but are constrained by inadequate health facilities and consumables.

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Management of Sickle Cell Disease: A Review for Physician Education in Nigeria (Sub-Saharan Africa)

Cited by 139 publications

References 165 publications

Prenatal Screening for Co-Inheritance of Sickle Cell Anemia and β-Thalassemia Traits

Prenatal Screening for Co-Inheritance of Sickle Cell Anemia and β-Thalassemia Traits

Therapeutic Approaches of Acute Thoracic Syndrome in Patients with Falciform Disease

The Health Workers Perspectives in the Management of Sickle Cell Disease in an Urban Health Centre in Ile-Ife, Nigeria

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