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Sickle cell disease
Published in Hung N. Winn, Frank A. Chervenak, Roberto Romero, Clinical Maternal-Fetal Medicine Online, 2021
Marc R. Parrish, John C. Morrison
These abnormalities of the red cells themselves do not, alone, explain the pathophysiology of the sickle cell crisis. It is currently felt that there exists a combination of the erythrocyte abnormalities, plasma flow alterations, and vessel wall interactions that are associated with sequestration and destruction of abnormal red cells in the microcirculation (Fig. 2) (27). This would explain patient variability noted in the clinical expression of a sickle crisis. Another factor involved in the pathophysiology of sickle cell crisis appears to be the large oscillation in blood flow in the microcirculation (28). With the use of laser-Doppler velocimetry, rather large changes in blood flow in factors of 7–10 s with peak/trough magnitudes measured at 50% of mean flow were noted. These findings would suggest that during sickle cell crisis, there is periodic flow in the microcirculation with synchronized rhythmic oscillations versus the continuous even-flow pattern observed in patients with normal hemoglobin.
The patient with acute cardiovascular problems
Published in Peate Ian, Dutton Helen, Acute Nursing Care, 2020
Whatever the cause of anaemia, there will be a reduced oxygen-carrying ability, and the potential for reduced oxygen delivery to the tissues. For example, sickle-cell disease results from an abnormality in the haemoglobin, causing the red blood cell to become fragile, with a tendency to change shape and become crescent- or ‘sickle’-shaped. A sickle-cell crisis occurs when large numbers of RBCs assume this sickle shape and is a medical emergency. Sickled cells have a reduced ability to carry oxygen, and due to their abnormal shape, they obstruct the capillaries, leading to reduced blood flow to organs and therefore ischaemia and organ damage. The most common sites for the action of sickled cells are the lungs, liver, brain, spleen and kidneys, although other parts of the body can be affected. The presenting symptoms are severe pain related to the affected organ(s) and extreme breathlessness. Immediate management includes oxygen and analgesia. A crisis may happen spontaneously with no apparent cause, however, precipitating factors can be hypoxia, hypovolaemia, hypothermia, stress and co-existing illness.
Obstetrics: Answers
Published in Euan Kevelighan, Jeremy Gasson, Makiya Ashraf, Get Through MRCOG Part 2: Short Answer Questions, 2020
Euan Kevelighan, Jeremy Gasson, Makiya Ashraf
Fetal risks.Maternal risks.Sickle cell crisis.
Impact of COVID-19 Infection on 24 Patients with Sickle Cell Disease. One Center Urban Experience, Detroit, MI, USA
Published in Hemoglobin, 2020
Nino Balanchivadze, Adam A. Kudirka, Sally Askar, Khaled Almadhoun, Philip Kuriakose, Raef Fadel, Vrushali Dabak
Sickle cell disease is a general term that describes an inherited group of disorders characterized by the presence of abnormal hemoglobin (Hb), Hb S (HBB: c.20A>T), caused by a point mutation at codon 6 of the β-globin gene. Hb S becomes poorly soluble when deoxygenated [8], causing red blood cells (RBCs) to distort into a crescent or sickle cell shape. Inciting events such as infection may cause hypoxia, which may lead to a sickle cell crisis resulting in vascular occlusion and major organ complications. This predisposition to major organ complications and susceptibility to hypoxia in patients with sickle cell disease was cited as one of the most frequent comorbidities seen in hospitalized patients during the H1N1 pandemic [9]. Note that sickle cell disease encompasses multiple genotypes, including a specific genetic profile called sickle trait. Collectively, the term sickle cell disease or trait (sickle cell disease/sickle cell trait) encompasses all individuals with at least one copy of the sickle cell disease Hb S gene.
Clinical variability and molecular characterization of Hbs/Gγ (Aγδβ)0-thal and Hbs/HPFH in Indian sickle cell disease patients: AIIMS experience
Published in Hematology, 2019
Hareram Pandey, Kanwaljeet Singh, Ravi Ranjan, Sanjay Kumar Pandey, Amit Sharma, Kamal Kishor, Tulika Seth, Manoranjan Mahapatra, Renu Saxena
Sickle cell disease (SCD) is a hemoglobinopathy, caused by point mutations in the β-hemoglobin gene (HBB), resulting in the substitution of adenine for thymine (GAG > GTG) at codon 6 of the β-globin gene. This results in change in amino acid (valine for glutamic acid) which leads to polymerization of the abnormal sickled hemoglobin i.e. HbS when deoxygenated. As the disease in progresses, there is deformation of the shape of initially reversible deformed red blood cells into the rigid sickle cells which cannot pass through the microcirculation efficiently, resulting anemia, cell adhesion, vaso-occlusion, and ultimately painful sickle cell crisis [1,2]. The sickle cell gene is widespread across in India and it is believed to represent an occurrence of the HbS mutation separate from those in Africa. This is known as the Asian haplotype [3].
Brachial plexitis: an unusual presentation in sickle cell disease
Published in Journal of Community Hospital Internal Medicine Perspectives, 2019
Mustafa Dawood, Justin McArthur, Abubakar Tauseef
MRI cervical spine and chest showed diffuse inflammation of the brachial plexus as shown in Figure 1. Electromyography and Nerve conduction studies confirmed the diagnosis of brachial plexitis. After ruling out sickle cell crisis, which was diagnosed clinically, patient was discharged home on tapering steroid treatment with outpatient follow-up. Patient returned for follow-up after multiple attempts of reaching her. On follow-up visit, her neurologic functions were back to normal as power of 5/5 in all muscle groups in the right upper extremity, sensations intact to pinprick, temperature, vibration, and deep tendon reflexes of 2+ in biceps, triceps, and brachioradialis.