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Article types Author guidelines Editor guidelines Publishing fees Submission checklist Contact
editorial office. Nonetheless, use of HU therapy in SCD has expanded substantially in recent years.
Such haemoglobinopathies mainly thalassaemias and sickle-cell anaemia are globally widespread.
These damaged (typically sickled shaped) RBCs are not only less flexible compared to normal
RBCs, but also highly adhesive. As a group, patients with SCA incur large numbers of hospital
admissions, emergency department visits, and outpatient visits, often at substantial costs, hence,
obtaining adequate health insurance is a problem for many patients. Berthaut et al. (2008) measured
the semen quality of 4 patients with SCA at baseline and 4 years after starting hydroxyurea. Iron
deficiency causes insufficient hemoglobin production which, in turn, causes anemia and anemia
symptoms. The most common method of DSB repair is non-homologous end joining, often resulting
in gene disruption or knockout. Multiple factors affect the development of GVHD in patients
undergoing transplant, including the source of the stem cells, the intensity of immunosuppression in
the conditioning regime (dose of anti-thymoglobulin) and the mismatch status of the donor to the
recipient ( Shenoy, 2013; Inamoto et al., 2016; Bernaudin et al., 2020 ). Again, doctors are
eventually waking up to the consequences and ramifications of this data. This results in hemolytic
anemia and recurrent vaso-occlusion in the microvasculature due to increased red blood cell adhesion
and retention. Patients with SCD have increased platelet levels at baseline that are further increased
during acute VOC. Sickle cell disease SCD is a group of inherited red blood cell disorders.
Unfortunately, a phase 3 study failed to reduce the mean duration of VOC in patients with SCD
compared to placebo ( Adams-Graves et al., 1997 ). The differences that exist between different
ethnicities have been a source of conflicts in some regions, which can be traced to ancestral times but
they have existed up to this day. Prevalence of the sickle cell trait in adults of Charleston County,
SC: an epidemiological study. The decrease in irreversible sickling of RBCs was not statistically
significant but a downward trend was observed ( Pace et al., 2003; Nur et al., 2012 ). Further
studies have shown decreased red cell membrane expression of phosphatidylserine which seems to
reflect overall reduced oxidative stress. A clinical trial exploring antibody-mediated non-
chemotherapy conditioning is being evaluated in patients with severe combined immunodeficiency,
in an attempt to reduce the exposure to chemotherapy and its toxicities is currently recruiting
patients ( ClincialTrials.gov Identifier: NCT02963064). This study was funded, in part, by the
Healthy South Carolina Initiative within the Medical University of South Carolina. Characterized by
sever chronic hemolysis, crisis and short life expectancy. NA replication takes place in the interphase
of the cell cycle. A novel, potent and selective PDE9 inhibitor (IMR-687) has been shown to
increase levels of cGMP and HbF without signs of myelosuppression in cell lines of patients with
SCD. So there are many medical doctors who truly care about their patients and yet would probably
just laugh at you if you asked them how to breathe for health. Of the hip and joints occur as a result
of ischemia. Simvastatin was found to reduce adhesion of white blood cells and in combination with
hydroxyurea, was found to decrease the number of pain crisis and markers of inflammation ( Hoppe
et al., 2017 ). Currently, there is an active clinical trial to assess the effect of simvastatin on central
nervous system vasculature in patients with SCD ( ClinicalTrials.gov Identifier: NCT03599609).
Sickle-cell anemia is the name of a specific form of sickle-cell disease in which. We use cookies to
create the best experience for you. Vaso-occlusive crisis involving organs such as the penis or. Over
the last couple of decades, there has been a spectacular growth of such strategies, setting the scene
for developing therapies that could precisely genetically correct a single base mutation in patient with
SCD. This paper is a reflective essay and discusses leadership management on the caring of a patient
with sickle cell disorder by applying the theoretical concept from Gibbs framework of reflection.
This blockage account for the pain that patients suffering encounter and the lack of oxygen supply
brings about serious fatigue. Sickle cell disease SCD which causes a wide range of severe and even
life-threatening consequences is caused by a single misspelling in the DNA instructions for
hemoglobin a protein vital for carrying oxygen in the blood. Sickle cell diseasespecific protocol
whenever possible to promote rapid effective and safe analgesic management and resolution of
vasoocclusive crises in children and adults. Report Back from San Antonio Breast Cancer
Symposium (SABCS) 2023: Spotlight. Hydroxyurea, however, is only partially successful because
the increase in fetal hemoglobin is uneven and not present in all cells. This may cure sickle cell
anemia, however, this is only available to a small subset of affected patients, because of either the
lack of a compatible donor or because severe organ damage is a contraindication. Memantine is a
NMDAR inhibitor which has shown to improve hydration of RBCs of patients with SCD in vitro
and to reduce sickling in the setting of deoxygenation. Sickling decreases the cells' flexibility and
results in a risk of various. The deformed erythrocytes also rupture easily and they tend to. Can be
define as hemolytic anemia with hemoglobinopathy. She was cured of her leukemia and at the same
time, her sickle cell complications also resolved ( Johnson et al., 1984; Johnson, 1985 ). Multiple
factors affect the development of GVHD in patients undergoing transplant, including the source of
the stem cells, the intensity of immunosuppression in the conditioning regime (dose of anti-
thymoglobulin) and the mismatch status of the donor to the recipient ( Shenoy, 2013; Inamoto et al.,
2016; Bernaudin et al., 2020 ). Sickle cell anaemia is an inherited blood disorder that causes a change
in haemoglobin characteristic. Bone marrow transplants have proven to be effective in children. The
differences that exist between different ethnicities have been a source of conflicts in some regions,
which can be traced to ancestral times but they have existed up to this day. In: Embury SH, Hebbel
RP, Mohandas N, et al., editors. Sickle cell disease: basic principles and clinical practice. As
described by Walters et al. (2010), HSCT can establish donor-derived erythropoiesis, but even more
importantly, can stabilize or even restore function in affected organs of patients with SCD when
performed in time. Since these are the patient’s own stem cells, there is no need for
immunosuppression, thus eliminating the risks of GVHD and immune-mediated graft rejection (
Esrick and Bauer, 2018; Orkin and Bauer, 2019 ). Of the hip and joints occur as a result of ischemia.
There is also conflicting evidence of the effects of HU on male fertility ( DeBaun, 2014 ). Valine (an
amino acid) takes the place of the normally. Sickle-cell disease, most commonly occurs in tropical
and sub-tropical regions. Adult hemoglobin contains a beta chain (HBB) while a. Autosplenectomy
increases the risk of infection from encapsulated organisms. Initially, this disease was referred to as '
Sickle Cell Anemia ' but in recent times the word disease has replaced the word anemia. These
concerns are being addressed in a current phase 3, double-blind, randomized, placebo-controlled,
multicenter study of Voxelotor ( ClinicalTrials.gov Identifier: NCT03036813) ( Vichinsky et al.,
2019 ). The average doctor only receives a mere 20 hours of nutrition training in his entire 8-year
program. In 1904, an African-American patient of Dr. James Herrick was. Disorders of the
haematopoietic system and immune system use the haematopoietic stem cell transplant procedure.
Alpha thalassaemia in Yemeni children with sickle cell disease.
Elucidation of its molecular basis prompted numerous biochemical and genetic studies that have
contributed to a better understanding of its pathophysiology. Effect of hydroxyurea on the frequency
of painful crises in sickle cell anemia: investigators of the multicenter study of hydroxyurea in sickle
cell anemia. Most people affected are of African or African-Caribbean origin although the sickle
gene is found in all ethnic groups. The patient received HSCT for the acute myeloid leukemia from
an HLA-matched sister who was a carrier for HbS (HbAS). A phase 3 study was terminated for lack
of efficacy ( ClinicalTrials.gov Identifier: NCT00294541) ( Ataga et al., 2008; Ataga and Stocker,
2009 ). This is 100% legal. You may not submit downloaded papers as your own, that is cheating.
Also you. In sickle cell anaemia the red blood cells are deformed; rather than the usual disc shape
they have a crescent or “sickle” shape, these sickle cells are also rigid as opposed to flexible like
regular red blood cells. Introduction Endocrine -1.pptx Introduction Endocrine -1.pptx Seminario
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anaemia 1. ?????????? ???? ?????? Page 1. Nietert View author publications You can also search for
this author in. And ?exible which lets them travel through small blood. Sickle-cell disease (SCD), or
sickle-cell anemia (or anemia, SCA) or. Result from excessive bilirubin production and precipitation
due to. Seminario biologia molecular-Universidad Pontificia Bolivariana. Barbie - Brand Strategy
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can trigger serious problems like stroke and organ failure. In which the person has only one copy of
the mutation that causes HbS and one. Prevalence of the sickle cell trait in adults of Charleston
County, SC: an epidemiological study. The bone marrow of individuals suffering from Sickle Cell
Anemia cannot produce red blood cells fast enough to meet the bodys demand. A detailed analysis
of charges for hematopoietic stem cell transplantation at a children’s hospital. Health insurance status
and the use of emergency and other outpatient services by adults with sickle cell disease. She was
cured of her leukemia and at the same time, her sickle cell complications also resolved ( Johnson et
al., 1984; Johnson, 1985 ). Well-designed studies are still needed to clarify its role in the
management of patients with SCD and to assess the safety of this approach ( van Zuuren and
Fedorowicz, 2015 ). Autosomal recessive (or co-dominant) genetic disease result from sub situation
of. Use of hydroxyurea in children ages 2 to 5 years with sickle cell disease. Hsieh et al. (2009)
developed a protocol for non-myeloablative HSCT with low dose total body radiation, alemtuzumab,
and sirolimus. Normal red blood cells RBCs are biconcave disc shaped and move smoothly through
the blood capillaries. Patients with identical sickle genotype still display extreme clinical course;
both acquired and inherited factors contribute to this clinical complexity of SCD ( Gardner and
Thein, 2016 ). It is regarded as another type of anaemia disorder in which cells necessary for carrying
oxygen are lacking or few in number. Sickle cell disease: diagnosis, management, education, and
research. Megaloblastic Anaemia: Symptoms, causes, diagnosis, treatment and preventions.
An ongoing clinical trial will compare 2-year overall survival and outcomes related to SCD in
patients that undergo transplant compared with current standard of care (ClinicalTrail.gov Identifier:
NCT02766465). Such molecules; histone deacetylase (HDAC), DNA methyltransferase 1 (DNMT1),
BCL11A and SOX6 modifying HbF expression have been explored as possible therapeutic options.
Further understanding of this technology could represent a new option for patients with SCD. As
nurses, our commitment to patient advocacy and holistic care makes us indispensable in supporting
patients with sickle cell anemia throughout their journey. However, researchers in Maryland have
modified the transplant procedure to ascertain it’s potential in treating adults with sickle cell disease.
And ?exible which lets them travel through small blood. Keep on browsing if you are OK with that,
or find out how to manage cookies. There are potentially less expensive pharmaceutical formulations
of L -glutamine available off the counter, but purity of the effective agents in these compounds have
not been validated. Sickle cell disease. 2nd ed. New York (NY): Oxford Press, 1992. Effect of
hydroxyurea on the frequency of painful crises in sickle cell anemia: investigators of the multicenter
study of hydroxyurea in sickle cell anemia. University of Minnesota Rochester MN Members Araba
N. Psychobiology There are several differences between Mendelian and Non-Mendelian inheritance
as given by Gregor Mendel to advance this subject matter in the field of Psychobiology. Article
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office Frontiers in Physiology. We use cookies to create the best experience for you. The costs of
children with sickle cell anemia: preparing for managed care. Palo Alto Software 9 Tips for a Work-
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Future of Work - Clark Boyd Getting into the tech field. As we move forward, we have to continue
focus our therapeutic approaches so that they can be accessed by those that suffer the most. Article
types Author guidelines Editor guidelines Publishing fees Submission checklist Contact editorial
office. Sickle cell anaemia is an inherited blood disorder that causes a change in haemoglobin
characteristic. Sickle cell anaemia is considered is a blood cell disorder passed from parent to child.
Use of hydroxyurea in children ages 2 to 5 years with sickle cell disease. Sickle cell disease SCD is
an umbrella term that defines a group of inherited diseases including sickle cell anaemia SCA HbSC
and HbS?-thalassaemia see below characterized by. Comparison of costs to the health sector of
comprehensive and episodic health care for sickle cell disease patients. Elucidation of its molecular
basis prompted numerous biochemical and genetic studies that have contributed to a better
understanding of its pathophysiology. While some chronic diseases require a significant amount of
breathing retraining, sickle cell anemia responds especially positively and very quickly to naturally
increasing CO2 and oxygen in the body cells. Patients with SCD have increased rates of venous and
arterial thrombotic events ( Brunson et al., 2017 ). This is an open-access article distributed under the
terms of the Creative Commons Attribution License (CC BY). Sickle cell disease SCD is an
autosomal recessive condition in which red blood cells become sickle-shaped and fragile. Read more
Doctors hope a recent medical breakthrough could lead to a cure for sickle cell anemia, an inherited
condition that can cause crippling pain and shortened lifespans for the estimated quarter million
babies born with it each year. Upload Read for free FAQ and support Language (EN) Sign in Skip
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