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They can tell you about genetic counseling and get you started with prenatal care early on. Carefully
and regularly inspect skin and assess pressure points for redness, warmth and swelling. The heme-
iron geometry of ferrous nitrosylated heme-serum lipoproteins, hemopexin, and albumin: a
comparative EPR study. SCD comes from the sickling or c-shaped mutation of red blood cells. In
addition to standard immunizations recommended by the Advisory Committee on Immunization
Practices, children with SCD should also be immunized against meningococcal disease and receive
the annual influenza vaccine. The liver may be adversely affected by hepatitis B and C, and HIV
infections ( 72 ). Platt OS, Brambilla DJ, Rosse WF, Milner PF, Castro O, Steinberg MH, et al.
Structure, expression and receptor-binding properties of placenta growth factor (PlGF). Also, it can
lead to other problems in the body such as heart problems. High doses of ESA and overcorrection of
anemia has been shown in studies to be associated with a higher risk of cardiovascular problems
(Derebail et al. 2014). Many times doctors must run other exams, monitoring heart ischemia during a
sickling crisis to make sure a crisis does not cause other issues (Wagdy et al. 2018). While being
extremely painful, a crisis can lead to other complications. This includes fetal hemoglobin, which
protects an infant from sickle cell disease for the first six months after birth. Document any changes
in the level of consciousness whether the patient is oriented to time, place, and situation. The
addition of inhaled nitric oxide may be helpful in supporting right heart function in the face of
elevated pulmonary vascular resistance. Reduced peripheral blood superoxide dismutase 2 expression
in sickle cell disease. Heme-induced oxidative stress exceeds the capacity of HMOX-1 to prevent
cellular and organ injury in transgenic murine model of SCD. The intensivist managing critically ill
SCD patients must be mindful to maintain hydration, intake and output, acid-base balance, and the
renal elimination of medications. This suggests that carriers of the gene for Rh-Negative blood might
be more common than expected because there is a positive selective advantage, greater resistance to
parasites, that outweighs the negative, possibly having a pregnancy where the mother’s blood
endangers the unborn child. A sudden, severe headache that is different from past headaches.
Support of patients with a central nervous system infection is similar to other patients with the added
concern for proclivity to stroke. Continuous formation of Hb-Hp complexes in diseases with severe
intravascular hemolysis including SCD and paroxysmal nocturnal hemoglobinuria results in depletion
of Hp to undetectable levels, leading to some accumulation in plasma of cell-free Hb ( 101, 102 ).
Although it has not been shown experimentally, sickle cell-related elevated PlGF levels could
possibly contribute to higher ET-1 levels ( 167, 314 ) driving renal dysfunction. Cardiac Dysfunction
Cardiac complications are common in SCD patients and along with the pulmonary complications
raise their morbidity and mortality risk ( 94, 345 ). Regulation of iron transport and the role of
transferrin. Higdon AN, Benavides GA, Chacko BK, Ouyang X, Johnson MS, Landar A, et al. Cell-
free hemoglobin limits nitric oxide bioavailability in sickle-cell disease. Hemolytic anemia is present
by the age of 6 months, although vascular occlusion symptoms may not appear until later. In
contrast, exchange transfusion using standard calculations can be performed by automated
erythrocytapheresis or manual exchange to quickly decrease the HbS percentage to 171 ). 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
). It also has the potential to be harmful to SCD patients long term. Oxidized Mutant Human
Hemoglobins S and E Induce Oxidative Stress and Bioenergetic Dysfunction in Human Pulmonary
Endothelial Cells. Nausea or vomiting. Pain, pressure, or a strange feeling in the back, neck, jaw, or
upper belly or in one or both shoulders or arms. Light-headedness or sudden weakness.
Multiple studies now demonstrate that the degree of anemia does not correlate with fetal outcome.
Processing of the lipocalin alpha(1)-microglobulin by hemoglobin induces heme-binding and heme-
degradation properties. It should be noted that, while blood transfusion remains an important
therapeutic option in SCD, evidence for its role in management of acute or chronic complications is
lacking except for prevention of primary and secondary strokes ( Howard, 2016 ). Heme binding to
albuminoid proteins is the result of recent evolution. HLA-haploidentical HSCT following RIC has
been reported to show promising results with prolonged and stable engraftment, but for both
unrelated umbilical cord blood (UCB) and haploidentical HSCT, rejection remains a major obstacle
in the context of RIC ( Bolanos-Meade et al., 2012; Angelucci et al., 2014; Fitzhugh et al., 2014;
Saraf et al., 2018; Bolanos-Meade et al., 2019 ). The gene therapy used was an erythroid-specific
gathering of anti-sickling proteins that would make up more than half of hemoglobin and almost all
of the red blood cells in the body. The spatial epidemiology of sickle-cell anaemia in India. In
contrast, a higher percentage of mature RBCs from SCD patients and mice retain their mitochondria
leading to excessive ROS accumulation and oxidative stress ( 25, 33, 34 ). Airway
hyperresponsiveness in children with sickle cell anemia. We present issues related to (a)
hemodynamic assessment and support, (b) respiratory considerations, (c) the role of transfusion
therapy in critically ill persons with SCD, and (d) precautions related to granulocyte colony-
stimulating factor and coagulation factor replacement. Note the size, shape and other characteristics
of ischemic areas, cuts, and bruises. Crizanlizumab (or Novartis’ Adakveo) has been recommended
by the National Institute for Health and Care Excellence (NICE) as a treatment option for
preventing recurrent sickle cell crises in people aged 16 or over. The additional financial and
infrastructural costs of caring for SCD in specific geographies need to inform state and regional
policy and practice guidelines in order to ensure adequate health systems response, given increased
costs of care on primary health centres and local governments with high proportion of patients with
SCD. The NLRP3 inflammasome and bruton’s tyrosine kinase in platelets co-regulate platelet
activation, aggregation, and in vitro thrombus formation. The size of the obstructed vascular area and
the amount and duration of the ischemia determine the degree of pain or organ damage. Their
genome might not even be based on DNA - but something else such as RNA, or some exotic form of
genetic storage that never evolved on Earth. Endothelin-1 contributes to the progression of renal
injury in sickle cell disease via reactive oxygen species. Lanzkron, MD, MHS; Richard Lottenberg,
MD; William J. Oxidative red blood cell membrane injury in the pathophysiology of severe mouse
beta-thalassemia. Dimerization of the receptor complex activates Janus kinases (JAKs) resulting in
phosphorylation of the tyrosine residues in the cytoplasmic domain of gp130 ( 364, 371 ). Lu M,
Zhang Q, Deng M, Miao J, Guo Y, Gao W, et al. The prevalence of sickle-cell haemoglobin, the
result of an amino acid substitution due to a point mutation in the gene coding for one of the
constituent proteins of haemoglobin, was first examined in India by Australian and British
pathologists. It has been reported to inhibit sickle RBC adhesion to the endothelial cells and to
reduce tumor necrosis factor-induced vasocclusion. Long-term malaria chemoprophylaxis has been
shown to lower the incidence of crisis and to reduce mortality, but few studies have evaluated its
benefit, particularly in SCD ( 106 ). OpenUrl ? Payyappallimana U, Venkatasubramanian P. A special
emphasis is given to heme-induced PlGF and IL-6 related mechanisms and their role in SCD disease
progression. Gao JL, Lu Y, Browne G, Yap BC, Trewhella J, Hunter N, et al. Immenschuh S, Vijayan
V, Janciauskiene S, Gueler F. Hsieh et al. (2009) developed a protocol for non-myeloablative HSCT
with low dose total body radiation, alemtuzumab, and sirolimus. PlGF’s pleiotropic nature in evident
in its angiogenic role where it exerts a paracrine or autocrine effect on endothelial cells, smooth-
muscle cells, fibroblasts, bone marrow progenitor cells and monocytes, to orchestrate vessel growth
and maturation ( 304 ).
Amelioration of inflammation and tissue damage in sickle cell model mice by Nrf2 activation.
Magnetic resonance detection of kidney iron deposition in sickle cell disease: a marker of chronic
hemolysis. Singla S, Sysol JR, Dille B, Jones N, Chen J, Machado RF. Your doctor can help with
patches, pills, and support. Thomas DD, Espey MG, Vitek MP, Miranda KM, Wink DA.
Hydroxyurea lowers transcranial Doppler flow velocities in children with sickle cell anaemia in a
Nigerian cohort. This would explain why the gene occurs in high frequency in populations that are
not really connected other than the fact that they are Homo Sapiens, such as Basques and certain
Jewish populations. Interleukin-6 and soluble interleukin-6 receptor: direct stimulation of gp130 and
hematopoiesis. Detterich JA, Liu H, Suriany S, Kato RM, Chalacheva P, Tedla B, et al. To create a
baseline set of observations for the patient, and to monitor any changes in the vital signs as the
patient receives medical treatment. It was hypothesized that because of the impact that it has on the
body it must also play a role in something as important as proper sleep. More than 175 mutations
producing beta thalassemia have been described. Antiviral, antimalarial, and other therapies may be
added based on risk factors, lab testing, and infectious disease consultant recommendations.
Heterotypic interactions enabled by polarized neutrophil microdomains mediate
thromboinflammatory injury. This aids in determining individualized therapy requirements. This in
turn will inhibit another function of the spleen, which is the production the infection-fighting
leukocytes. Table 1 summarizes the hemoglobin composition in normals, in the common forms of
sickle cell disease, and in the carrier state, sickle cell trait. Winterbourn CC. Toxicity of iron and
hydrogen peroxide: the Fenton reaction. Crises and infections are less frequent than in SCA,
although occasional patients have severe disease (Platt et al., 1991). Bone disease (aseptic necrosis)
and retinal vessel involvement (sickle retinopathy) are more common than in SCA, whereas other
types of organ damage are less common. Methods We followed a scoping review protocol, using
Google Scholar and PubMed for published literature. Renal iron load in sickle cell disease is
influenced by severity of haemolysis. Heparin inhibits the flow adhesion of sickle red blood cells to
P-selectin. Cellular basis for the acute inhibitory effects of IL-6 and TNF- alpha on excitation-
contraction coupling. This was regardless of background treatment with hydroxyurea. Gaps in
coverage Studies on rehabilitative care, quality of care and cultural aspects of SCD were completely
absent, whereas studies on governance, financing and community engagement were poor in terms of
coverage of different geographical regions from where SCD is reported. Demonstrate to the client
and explain the importance of always cleaning the open wound ulcers as indicated with hydrogen
peroxide or povidone iodine solutions. Revascularization of ischemic tissues by PlGF treatment, and
inhibition of tumor angiogenesis, arthritis and atherosclerosis by anti-Flt1. Autiero M, Waltenberger
J, Communi D, Kranz A, Moons L, Lambrechts D, et al. Increased Heme Levels in the Heart Lead to
Exacerbated Ischemic Injury. Finally, other blood cells such as neutrophils and macrophages also
release ROS into the plasma which are neutralized by anti-oxidants such as superoxide dismutase
before they can be taken up by RBCs ( 52 ).
Epstein-Barr Virus infection can cause splenic rupture, thrombocytopenia, agranulocytosis, hemolytic
anemia, and hemophagocytic lymphohistiocytosis in SCD. Targeted disruption of the housekeeping
gene encoding glucose 6-phosphate dehydrogenase (G6PD): G6PD is dispensable for pentose
synthesis but essential for defense against oxidative stress. Burden and spectrum of bacterial
infections among sickle cell disease children living in Cameroon. Many underestimate the number of
people who face this disorder and how challenging it can be on the body. It is essential to continue to
bring awareness about this disease to the public along with continually looking into how it affects
the body beyond just the sickling of cells. Gaps in coverage Studies on rehabilitative care, quality of
care and cultural aspects of SCD were completely absent, whereas studies on governance, financing
and community engagement were poor in terms of coverage of different geographical regions from
where SCD is reported. Crizanlizumab (or Novartis’ Adakveo) has been recommended by the
National Institute for Health and Care Excellence (NICE) as a treatment option for preventing
recurrent sickle cell crises in people aged 16 or over. Augmented NRF2 activation protects adult
sickle mice from lethal acute chest syndrome. Altered levels of pro-inflammatory cytokines in sickle
cell disease patients during vaso-occlusive crises and the steady state condition. Although, elevated
plasma IL-6 have been reported in human and mice with SCD ( 168, 387, 388 ), and hemolysis is a
major comorbidity of SCD ( 94 ), however, there has been no direct link between these two
processes. Preventing pneumococcal disease among infants and young children; recommendations of
the Advisory Committee on Immunization Practices (ACIP). Elucidation of its molecular basis
prompted numerous biochemical and genetic studies that have contributed to a better understanding
of its pathophysiology. Identification of the receptor scavenging hemopexin-heme complexes. Nath
KA, Belcher JD, Nath MC, Grande JP, Croatt AJ, Ackerman AW, et al. Certain populations (like
tribal people) and regions in India are disproportionately affected by sickle cell disease (SCD) but
significant knowledge and action gaps persist in developing comprehensive programmes for care for
SCD. The implantation of stem cells, cells that have yet to become a specific cell type, has excellent
potential to create healthy red blood cells(Pawliuk et al. 2001). In this condition, the spleen rapidly
entraps blood leading to sudden onset of severe anaemia, circulatory collapse and death in few hours
if not promptly detected and treated. Vasoocclusion in the sickling disorders is the result of a
complex interaction involving the sickle RBC, the endothelial cells lining the blood vessels,
leukocytes, and platelets, along with coagulation factors and other plasma proteins. Impact of
intravascular hemolysis in malaria on liver dysfunction: involvement of hepatic free heme overload,
NF-kappaB activation, and neutrophil infiltration. Control of Oxidative Stress and Inflammation in
Sickle Cell Disease with the Nrf2 Activator Dimethyl Fumarate. Endothelin-1 production during the
acute chest syndrome in sickle cell disease. Plewes K, Kingston HWF, Ghose A, Maude RJ,
Herdman MT, Leopold SJ, et al. Heme-induced oxidative stress exceeds the capacity of HMOX-1 to
prevent cellular and organ injury in transgenic murine model of SCD. Altered amount and activity of
superoxide dismutase in sickle cell anemia. There is no cure for sickle cell anemia yet; however, a
variety of treatments are available to control pain and to mitigate complications of the disease. Chi L,
Li Y, Stehno-Bittel L, Gao J, Morrison DC, Stechschulte DJ, et al. The depletion of NO is associated
with pathological platelet activation and tissue factor expression. Both MCP-1 and IL-8 are elevated
in SCD patients ( 321 ) supporting the PlGF-ET-1 synergy as another contributing factor to the
development of PH in SCD. SCD may confer protection against HIV infection because of
upregulation of inflammation, iron metabolism, and auto-splenectomy, which are not favorable for
HIV replication ( 98 ). When this happens, oxygen can't reach nearby tissues.
Not all heme and Hb are bound to proteins or other macromolecules. This would make sense if the
reason for the prevalence of the gene was because both the ancestral Basque and Jewish populations
originated in an environment with similar selective factors rather than being of a common lineage.
Seeing that my RH- paternal grandparents met each other in a small town here in Arkansas in 1941,
leads me to believe that RH- folks are not that rare. Complementary DNA for a novel human
interleukin (BSF-2) that induces B lymphocytes to produce immunoglobulin. The human Beta A
globin gene is what causes the mutation in those with SCD. Following hemolysis, Hb is oxidized to
unstable methemoglobin resulting in release of free heme ( 139 ), which can intercalate into cell
membrane and alter cellular structures or taken up by cells ( 150, 151 ). Thomas AM, Gerogianni A,
McAdam MB, Floisand Y, Lau C, Espevik T, et al. In the presence of appropriate risk factors,
geographic location, and travel history, further testing for tuberculous meningitis, cerebral malaria,
HIV and HIV-associated central nervous system infections (HIV, Cryptococcus neoformans,
Toxoplasma gondii ), fungal pathogens, roundworm infections, and amoeba should be considered.
Dialysis and renal transplantation are sometimes necessary in adulthood. The clinical sequelae of
intravascular hemolysis and extracellular plasma hemoglobin: a novel mechanism of human disease.
Selvaraj SK, Giri RK, Perelman N, Johnson C, Malik P, Kalra VK. Kato G, McGowan V, Machado
R, Little J, Taylor J, Morris C, et al. Ordinarily, 96% of the normal adult hemoglobin composition is
Hb A (two a and two b chains). Hemolysis, Interleukin-6, and Cardiovascular Dysfunction IL-6 is a
ubiquitous and pleiotropic proinflammatory cytokine produced by many cells including macrophages
( 356, 357 ), neutrophils ( 358, 359 ), endothelial and smooth muscle cells ( 360, 361 ),
cardiomyocytes ( 362 ) and fibroblasts ( 363 ), when stimulated by ligands for toll-like receptors or
other pattern recognition receptors. Children under the age of five are at highest risk for these
infections. Allow adequate room sunlight by opening the window curtains. PlGF exists as a
homodimer or as a heterodimer with VEGF. Cardiac abnormalities in children with sickle cell
anemia. Demonstrate to the client and explain the importance of always cleaning the open wound
ulcers as indicated with hydrogen peroxide or povidone iodine solutions. The United States Centers
for Disease Control ( ) provides a website and book for travel medicine specialists that offers
guidance for vaccination and medical prophylaxis for prevalent and endemic infections based on the
location of rural or urban destination, recent outbreaks, length of stay, age and immune competency
of the traveler, and other variables. This increases the danger of infection and delays recovery in the
case of a weakened immune system. Oxidized Ferric and Ferryl Forms of Hemoglobin Trigger
Mitochondrial Dysfunction and Injury in Alveolar Type I Cells. Zinc deficiency develops as a result
of poor dietary intake, high protein turnover, and increased losses from the kidneys due to
inadequate reabsorption ( 44 ). This suggests that carriers of the gene for Rh-Negative blood might
be more common than expected because there is a positive selective advantage, greater resistance to
parasites, that outweighs the negative, possibly having a pregnancy where the mother’s blood
endangers the unborn child. Albuminuria Is Associated with Endothelial Dysfunction and Elevated
Plasma Endothelin-1 in Sickle Cell Anemia. Thomas DD, Espey MG, Vitek MP, Miranda KM, Wink
DA. Gaps in evidence at the health policy and systems level also limit solutions at that level and
unfairly push the locus of action to individuals and communities. Sickle Cell Anemia Nursing
Interventions Rationales Assess the patient’s vital signs, especially the respiratory rate and depth, as
well as the use of accessory muscles. They can tell you about genetic counseling and get you started
with prenatal care early on. Managing the pain during this time can be extremely challenging as
some blockages can feel unbearable.
Septicemia, meningitis, osteomyelitis, pneumonia, and pyelonephritis are all more common in the
sickle syndromes. Aft RL, Mueller GC. Hemin-mediated DNA strand scission. This has led to
speculation that Rh-Negative blood is related to Cro-Magnon ancestry going back to the upper
Paleolithic period in Europe. Pandolfi PP, Sonati F, Rivi R, Mason P, Grosveld F, Luzzatto L.
Goldsmith, MD; Eduardo Ortiz, MD, MPH; Robinson Fulwood, PhD, MSPH; Ann Horton, MS;
Joylene John-Sowah, MD, MPH. In a Nigerian study of 100 SCD patients, 27% were found to be
infected with intestinal parasites that included four helminths ( Ascaris lumbricoides, Ancylostoma
duodenale, Trichuris trichiura, and Strongyloides stercoralis ) and three protozoa ( Entamoeba
histolytica, Entamoeba coli, and Giardia lamblia ) ( 109 ). Much useful information on the clinical
course of this disease has been the result of reports from the Cooperative Study of Sickle Cell
Disease in the United States (Platt et al., 1991, 1994). Recurrent vasoocclusion leads to ischemic
destruction of the spleen, which predisposes the patient to severe infections. Usually, the patient may
experience pain in the extremities, back, and ribs. Likewise, ROS production becomes markedly
amplified in low antioxidant microenvironments, as found in SCD, resulting in damage of
macromolecules including lipids ( 60, 61 ), DNA ( 62, 63 ), and proteins ( 64, 65 ). If you have SCD
and are pregnant, you should receive extra care to monitor your SCD and treat problems early.
Pulmonary hypertension as a risk factor for death in patients with sickle cell disease. Altered levels of
pro-inflammatory cytokines in sickle cell disease patients during vaso-occlusive crises and the steady
state condition. Placental growth factor-1 potentiates hematopoietic progenitor cell mobilization
induced by granulocyte colony-stimulating factor in mice and nonhuman primates. They contain
hemoglobin (a protein), which carries oxygen in your blood. Abdominal pain, nausea, vomiting,
fever, and jaundice are common presenting symptoms of cholecystitis. Cell-free hemoglobin: a novel
mediator of acute lung injury. IL-6 is a glycoprotein composed of 184 amino acids and of 26 kDa in
molecular weight ( 364 ). The most common method of DSB repair is non-homologous end joining,
often resulting in gene disruption or knockout. This review focuses on heme-induced mechanisms
that are implicated in disease pathways, mainly in SCD. Sickle-shaped cells are not flexible and can
stick to vessel walls. Placenta Growth Factor (PlGF), a Novel Inducer of Plasminogen Activator
Inhibitor-1 (PAI-1) in Sickle Cell Disease (SCD). The role of the spleen in the immune response
following naturally acquired exposure to encapsulated bacteria. Hydroxyurea is the first clinically
acceptable drug that has been shown to deter painful crises in adults with sickle cell anemia
(Charache et al. 1995). Hydroxyurea differs from opioids because it does not have any effect during
a crisis (Charache et al. 1995). Its goal is to help decrease the number of crises and provide a long-
term health treatment (Charache et al. 1995). Westerman M, Pizzey A, Hirschman J, Cerino M, Weil-
Weiner Y, Ramotar P, et al. Molecular detection of respiratory pathogens among children aged
younger than 5 years hospitalized with febrile acute respiratory infections: s prospective hospital
based observational study in Niamey, Niger. Affected children present with acute onset of severe
pallor, shock and painful left sided abdominal distension (bloating) with an enlarged and often
massive spleen. Blood artery occlusion and circulatory stasis can cause edema in the limbs, increasing
the risk of tissue ischemia and necrosis. Its mechanism of action has not been fully understood and
its impact on HbF will decrease over time. Given the varied physical (painful SCD crises among
young adults, easy fatigability especially among disadvantaged populations that depend on daily
wage physical labour) and psychosocial manifestations of SCD, having access to counselling and
social services that can protect patients from socioeconomic consequences is important especially
among tribal and disadvantaged community settings. Complement Component C5 and TLR
Molecule CD14 Mediate Heme-Induced Thromboinflammation in Human Blood.
Gonsalves CS, Li C, Mpollo MS, Pullarkat V, Malik P, Tahara SM, et al. James, MD, MPH 6;
Lanetta Jordan, MD, MPH, MSPH 7; Sophie M. This increases the danger of infection and delays
recovery in the case of a weakened immune system. Liver injury is associated with mortality in sickle
cell disease. In vivo experiments using PlGF null and SS sickle mice as well as adenoviral
overexpression of PlGF, have confirmed that PlGF plays a significant role in PAI-1 regulation ( 326
). Sputum acid-fast staining and Mycobacterial cultures should be considered in the appropriate
clinical settings. This is due to progress in understanding the mechanisms and risk factors of several
complications of the disease, associated clinical findings and mouse models, approval of new
treatment therapies, multi-disciplinary approach to care, penicillin prophylaxis and high-tech
diagnostic tools ( 241 ). Call 911 anytime you think you may need emergency care. Accelerated
autoxidation and heme loss due to instability of sickle hemoglobin. CD163 binding to haptoglobin-
hemoglobin complexes involves a dual-point electrostatic receptor-ligand pairing. Splenomegaly or
hepatomegaly may indicate splenic or hepatic sequestration, malaria, or a variety of viral infections.
Always monitor the patient for proper hydration by encouraging adequate fluid intake and eating a
balanced diet. The mechanism is sickling and sludging of blood in the corpora cavernosa, with
failure of detumescence. One of the most prominent being that no set cure can be offered at this
time. They’re even more important for children with sickle cell disease because their infections can
be severe. As in the case of PAI-1, the induction is mediated by HIF-1a and NADPH oxidase ( 333
). Pulmonary artery hypertension develops for reasons that are multi-factorial and may reflect the
intrinsic elevation of pulmonary vascular resistance or left ventricular dysfunction with normal
pulmonary vascular resistance. Relative rates and features of musculoskeletal complications in adult
sicklers. P-selectin plays a role in haem-induced acute lung injury in sickle mice. Because of the
diverse perceptions of pain, different descriptions may be encountered with each patient. When they
are in the c-shape, they can latch onto each other, causing a block in the blood vessels. Xu H,
Wandersee NJ, Guo Y, Jones DW, Holzhauer SL, Hanson MS, et al. Control of Oxidative Stress and
Inflammation in Sickle Cell Disease with the Nrf2 Activator Dimethyl Fumarate. Molecules of
deoxygenated hemoglobin become polymerized, and this causes red blood cells to sickle in sickle cell
patients. It is often asymptomatic and affects mostly the spine. This scoping review seeks to identify
geographical and evidence gaps for action on SCD from a health systems lens. Erythrocyte
adherence to endothelium in sickle-cell anemia. New insights into the regulation of placental growth
factor gene expression by the transcription factors GCM1 and DLX3 in human placenta. Measure the
depth of the edema and gently press the skin to record time for the skin to go back to its original
form. Gao JL, Lu Y, Browne G, Yap BC, Trewhella J, Hunter N, et al.