scholarly journals Growth Failure in Children with Sickle Cell Anaemia

2020 ◽  
Vol 5 (8) ◽  
pp. 1393-1399
Author(s):  
Ikegwuonu Charles ◽  
Manyike Chuka ◽  
Ezeanosike Obumneme ◽  
Ujunwa Fortune ◽  
Onoh Emmanuel ◽  
...  
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anaemia ◽  
Growth Failure ◽  
Physical Growth ◽  
Cell Disease ◽  
Anthropometric Indices ◽  
Common Causes ◽  
Chronic Anaemia ◽  
The Common ◽  
Haemoglobin Synthesis

Abstract:-  Background Sickle cell disease is a group of inherited sickling disorders consequent upon abnormalities of haemoglobin synthesis. It is one of the common causes of retardation in both physical growth and sexual maturation in children, as a result of chronic anaemia and consequent hypoxia that occur in several organs.  Objectives To determine the anthropometric indices (weight, height and Body Mass Index) of children with sickle cell anaemia (SCA) and compare them with that of children with normal haemoglobin; haemoglobin AA (HbAA).

Anemias

2019 ◽  
pp. 21-27
Author(s):  
Kevin B. Hoover
Keyword(s):  
Iron Deficiency ◽  
Sickle Cell ◽  
Blood Cells ◽  
Imaging Study ◽  
Deficiency Anemia ◽  
Imaging Findings ◽  
Common Causes ◽  
Characteristic Imaging ◽  
The Common ◽  
Initial Imaging

Chapter 74 discusses anemia conditions including sickle cell disease and thalassemia. Anemia is a condition of decreased oxygen delivery to organs caused by a deficit of functional red blood cells. It is a very common clinical condition and has a wide variety of causes. Of the common causes, the hemoglobinopathies have the most characteristic imaging findings. There are common imaging findings secondary to the compensatory increase in red blood cell and precursor number. Radiographs are a standard initial imaging study. MRI is the most sensitive imaging study for the sequelae of anemia. Iron deficiency anemia is treated with iron supplementation, aplastic anemia is treated by removal of the inciting agent, and painful crises of sickle cell are treated symptomatically.

scholarly journals The Fc Receptor Polymorphisms and Expression of Neutrophil Activation Markers in Patients with Sickle Cell Disease from Western India

2013 ◽  
Vol 2013 ◽  
pp. 1-7 ◽  
Author(s):  
Harshada K. Kangne ◽  
Farah F. Jijina ◽  
Yazdi M. Italia ◽  
Dipti L. Jain ◽  
Anita H. Nadkarni ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Clinical Manifestations ◽  
Western India ◽  
Genotypic Frequency ◽  
Cell Disease ◽  
Activation Markers ◽  
Initiation And Propagation ◽  
Significant Difference ◽  
The Common

Objective. Sickle cell disease has variable clinical manifestations. Activation of neutrophils plays an important role in the initiation and propagation of vaso occlusive crises which can be analysed by determining the expression of neutrophil antigens such as CD16, CD32, and CD62L. The common FcγR polymorphisms (FcγRIIA and FcγRIIIB) are considered to influence clinical presentation. This study focuses on distribution of FcγR polymorphisms and their association with neutrophil activity among the patients from western India.Methods. In this paper 127 sickle cell anemia patients and 58 patients with sickle-β-thalassemia (median age12±8.58 years) with variable clinical phenotypes along with 175 normals were investigated. FcγRs polymorphisms were analysed by RFLP and AS-PCR. Activation of neutrophils was measured by flow cytometry.Results. The genotypic frequency of the H/R genotype of FcγRIIA and the NA1/NA1 genotype of FcγRIIIB was significantly decreased in patients compared to normals (P-0.0074,P-0.0471, resp.). We found a significant difference in the expression of CD32 and CD62L among the patients as against normals. A significantly higher expression of CD32 was seen in the milder patients with the H/H genotype (P-0.0231), whereas the expression of CD16 was higher in severe patients with the NA2/NA2 genotype (P-0.0312).Conclusion. The two FcγR polymorphisms had significant association with variable phenotypes of sickle cell disease. The expression of CD62L decreased in our patients indicating activation of neutrophils.

scholarly journals Counselling Needs of Sickle-Cell Anaemia Adolescents in Ekiti State, Nigeria

2020 ◽  
Vol 30 (6) ◽  
Author(s):  
Lateef Omotosho Adegboyega
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Anaemia ◽  
Rank Order ◽  
Genetic Disorder ◽  
Research Question ◽  
Sampling Technique ◽  
Cell Disease ◽  
Significant Difference ◽  
Counselling Needs

BACKGROUND፡ Sickle-cell disease is an autosomal recessive genetic disorder of hemoglobin (Hb) structure and the most common of the hemoglobinopathies. Hence, this study investigated counseling needs of sickle-cell anaemia adolescents in Ekiti State.METHODS: Descriptive survey design was adopted for this study. Purposive sampling technique was adopted to draw a total of 121 respondents. A questionnaire was used to collect data for the study. Mean and rank order were used to answer the research question while chi-square and Analysis of Variance (ANOVA) were used to test the hypotheses at 0.05 level of significance.RESULTS: The findings revealed that counselling needs of adolescents with sickle-cell anaemia include counsellors are expected to encourage adolescents with sickle-cell anaemia to have confidence in self among others. The findings also revealed that there was a significant difference in the counselling needs of adolescents with sickle-cell anaemia based on gender while there was no significant difference in the counselling needs of adolescents with sickle-cell anaemia based on religion.CONCLUSION: The counselling needs of adolescents with sickle-cell anaemia include adolescents with sickle-cell anaemia easily comprehend the counselling therapy of counselloramong others. It was recommended that Government should offer standard health care for all adolescents with Sickle-cell disease.

scholarly journals Intima-media thickness of the common carotid arteries as a marker of retinopathy and nephropathy in sickle cell disease

2020 ◽  
Vol 39 (1) ◽  
pp. 79-84
Author(s):  
Oluwagbemiga O. Ayoola ◽  
Rahman A. Bolarinwa ◽  
Oluwatoyin H. Onakpoya ◽  
Stephen O. Onigbinde ◽  
Christianah M. Asaleye ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Carotid Arteries ◽  
Intima Media Thickness ◽  
Cell Disease ◽  
Media Thickness ◽  
The Common ◽  
Common Carotid Arteries

scholarly journals Association of sickle cell disease with nutritional status among under-five children and mediating role of hemoglobin level: secondary analysis of data from 2018 Nigeria Demographic and Health Survey

2020 ◽  
Author(s):  
Mohammad Redwanul Islam ◽  
Md. Moinuddin ◽  
Ayeda Ahmed ◽  
Syed Moshfiqur Rahman
Keyword(s):  
Sickle Cell Disease ◽  
Nutritional Status ◽  
Sickle Cell ◽  
Hemoglobin Level ◽  
Cell Disease ◽  
Anthropometric Indices ◽  
Under Five ◽  
Under Five Children ◽  
Mediating Role

Abstract Background: Malnutrition continues to affect under-five children in Africa to an overwhelming proportion. The situation is further compounded by the burden of sickle cell disease (SCD). However, association of SCD with stunting, wasting, and underweight in a nationally representative sample of under-five children remains unexplored. We aimed to describe prevalence of undernutrition by sickle cell status, to evaluate its association with growth faltering ascertained with anthropometric indices, and to explore mediating role of hemoglobin.Methods: We availed data from the 2018 Nigeria Demographic and Health Survey (DHS) and the sample comprised 11233 children aged 6–59 months who were successfully genotyped for SCD. The DHS employed a two-stage stratified sampling strategy. SickleSCAN rapid diagnostic test was used for SCD genotyping. Z-scores of length/height-for-age (HAZ), weight-for-height (WHZ), and weight-for-age (WAZ) were computed against the 2006 World Health Organization Child Growth Standards. We fitted logistic regression models to evaluate association of SCD with stunting, wasting, and underweight. Mediation analysis was performed to capture the indirect effect of, and proportion of total effect mediated through hemoglobin level in SCD-anthropometric indices association.Results: Prevalences of stunting, wasting, and underweight among children with SCD were 55.4% (54.5–56.4), 9.1% (8.6–9.7), and 38.9% (38.0-39.8), respectively. The odds of stunting were 2.39 times higher (adjusted odds ratio (aOR) 2.39, 95% CI: 1.26–4.54) among sickle children than those with normal hemoglobin. SCD was also significantly associated with underweight (aOR 2.64, 95% CI: 1.25–5.98), but not with wasting (aOR 1.60, 95% CI: 0.85–3.02). Hemoglobin level significantly mediated SCD-HAZ (adjusted indirect effect (aIE) -0.328, 95% CI: -0.387, -0.270), SCD-WHZ (aIE − 0.080, 95% CI: -0.114, -0.050), and WAZ (aIE − 0.245, 95% CI: -0.291, -0.200) associations. The extent of mediation was highest for SCD-HAZ association (adjusted proportion mediated 0.928, 95% CI: 0.535–2.770).Conclusion: We presented compelling evidence of the negative impact of SCD on nutritional status of under-five children. Integration of a nutrition-oriented approach into a definitive SCD care package and its nation-wide implementation could bring promising results by mitigating the nutritional vulnerability of children with SCD.

Platelet Aggregation, Malondialdehyde Generation and Production Time in Children with Sickle Cell Anaemia

1981 ◽  
Vol 46 (04) ◽  
pp. 690-693 ◽  
Author(s):  
George R Buchanan ◽  
Christine A Holtkamp
Keyword(s):  
Platelet Aggregation ◽  
Platelet Activation ◽  
Sickle Cell ◽  
Sickle Cell Anaemia ◽  
Adenosine Diphosphate ◽  
Cell Disease ◽  
Production Time ◽  
Platelet Production ◽  
White Control ◽  
Homozygous Sickle Cell Disease

SummaryIn view of prior reports of platelet activation in the sickling disorders, platelet aggregation, malondialdehyde (MDA) production following stimulation with N-ethylmaleimide, and/or production time (survival) measurements were examined in 44 children with homozygous sickle cell disease. Aggregation in response to epinephrine, collagen, and adenosine diphosphate was similar to or only slightly less than in normal black controls, rendering highly unlikely the circulation of a sizable population of refractory or “exhausted” platelets. The platelets from the normal blacks aggregated less in response to epinephrine than those from white control subjects. MDA generation in sickle cell platelets was not increased, and platelet production time was not shortened in 6 patients studied during crisis. These observations do not support the occurrence of a marked degree of platelet activation and consumption in sickle cell anaemia.

scholarly journals Prevalence of sickle cell disease and sickle cell trait among children admitted to Al Fashir Teaching Hospital North Darfur State, Sudan

2019 ◽  
Vol 12 (1) ◽  
Author(s):  
Mudathir A. Adam ◽  
Nassreldeen K. Adam ◽  
Babiker A. Mohamed
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Teaching Hospital ◽  
Sickle Cell Anaemia ◽  
Sickle Cell Trait ◽  
Sub Saharan Africa ◽  
Cell Disease ◽  
Normal Individuals ◽  
Sub Saharan ◽  
Hb Concentration

Abstract Objective It is estimated that 50% to 90% of infants born with (SCA) in sub-Saharan Africa die before 5 years old. Northern Darfur State at western Sudan region has a multiethnic population with a high frequency of sickle cell anaemia, but little about it is published. This study aimed to determine the prevalence of sickle cell anaemia among children admitted to Al Fashir Teaching Hospital in Al Fashir, Northern Darfur State, Sudan. Results The prevalence of sickle cell disease by haemoglobin electrophoresis among these 400 children patients was 59 (14.8%). Sickle cell trait patients were 11.3% and Sickle cell disease positive patients were 3.5%. Individuals with SCA have consistently low blood Hb concentration, normal MCV and high mean WBC’s. Individuals with sickle cell trait had haematological parameters near to those of normal individuals.

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