Recent data on prevalence and incidence have been obtained from the. This student paper was written as an assignment in the. Strokes are an occurrence one expects to see in older people, but in children with sickle cell disease scd, they occur most commonly at age 5. Children with sickle cell disease, a chronic hemolytic anemia, present with a wide variety of neurological syndromes, including ischemic and hemorrhagic stroke, transient ischemic attacks, soft. Since the discovery of sickle cell disease scd in 1910, enormous strides have been made in the elucidation of the pathogenesis of its protean complications, which has inspired recent advances in. Major complications of sickle cell disease and nursing implications. Overt strokes are typically due to largeartery vasculopathy affecting the intracranial internal carotid arteries and proximal middle cerebral arteries, whereas silent strokes typically occur in the territory of penetrating arteries. Sickle cell disease sickle cell anemia medlineplus. Hillery and others published pathophysiology of stroke in sickle cell disease find, read and cite all the research you. Sickle cell disease american academy of pediatrics. A 24yearold man with sickle cell disease scd was non compliant with. Sickle cell disease scd, a geneticallydetermined pathology due to an amino acid substitution i. Background about sickle cell disease and making the diagnosis sickle cell disease is the most common inherited hematologic disorder in the world, primarily affecting people of african, mediterranean, east indian or latin american descent.
Cerebral ischemia may manifest in children as both silent cerebral infarcts and overt ischemic stroke. Pathophysiology and treatment of stroke in sicklecell disease. Cerebrovascular disease, particularly stroke, is one of the most severe clinical complications associated with sickle cell disease and is a significant cause of morbidity in both children and adults. Stroke affects both motor and cognitive function in patients with sickle cell disease scd. The pathophysiology of scd involves not only red blood cells rbcs but also the. Sickle cell disease scd is a group of inherited red blood cell disorders. The most common stroke is ischemic, but hemorrhagic stroke can be seen in up to 2% of patients, particularly in early adulthood. This publication, which was developed by physicians, nurses, psychologists, and social workers who specialize in the care of children and adults with sickle cell disease, describes the current approach to counseling and also to management of many of the medical complications of sickle cell disease. Although the precise pathophysiology of stroke in scd is still an area of. Individuals living with scd suffer from both acute and chronic complications that require close contact with the medical system. Stroke prevention requires intensive risk assessment and closely monitored interventions for those with increased risk. The pathophysiology, prevention, and treatment of stroke. While the problem of stroke in the patients with sickle cell disease scd has been known for more than 75 years, adequate preventive and treatment strategies are just now being tested. This leads to a rigid, sickle like shape under certain circumstances.
Challenges in management include distinguishing acute stroke from other central nervous system complications of the disease such as seizures, meningitis, complicated migraine and cerebral malaria in endemic areas. This student paper was written as an assignment in the graduate course free radicals in biology and medicine 77. Stroke in sickle cell patients is fairly common, with an incidence ranging between 4% and 8%. The most common type is known as sickle cell anaemia sca. Symptomatic management and prevention of these events using the fetal hemoglobinreactivating agent hydroxyurea are currently the mainstay of treatment. Review pathophysiology and treatment of stroke in sicklecell disease. Adults with history of stroke or abnormal tcd as children. Healthy red blood cells are round and they move through small blood vessels carrying oxygen to all parts of the body. Sickle cell diseasegenetics, pathophysiology, clinical. Sickle cell disease scd is a powerful risk factor for neurovascular and other neurological conditions due to vasculopathy, thrombus formation, and thromboembolic phenomenon. Help patients to understand potential triggers and avoidance strategies. Stroke management is largely extrapolated from pediatric studies. Intravascular hemolysis and the pathophysiology of sickle cell disease gregory j.
Sickle cell disease is the most common cause of childhood stroke. Cerebral infarction in sicklecell disease ranges from overt stroke with abrupt onset of neurological deficit to silent infarcts, which are not acutely. Hsu 2, neeraj kohli 3, anissa patel 4, kilali ominuevbota 5, ko. Review pathophysiology and treatment of stroke in sickle.
It is important to evaluate for and treat other potential causes of stroke. While the problem of stroke in the patients with sickle cell disease scd has been known for more than 75 years, adequate preventive and treatment strategies. It results in an abnormality in the oxygencarrying protein haemoglobin found in red blood cells. Both adult and pediatric patients with sickle cell disease face a higher risk of stroke than the general population. Pdf the epidemiology, evaluation and treatment of stroke in. Strokes are difficult to explain on the basis of the central pathological process in sickle cell disease, namely the occlusion of small vessels by deformed sickled cells. Several risk factors increase the likelihood for stroke.
Stroke and other complications hematology fellows conference july, 2012 john j. The majority of strokes in this population occur between ages 3 and 14. Most patients experience both chronic and episodic pain, and acute pain crisis is the most common reason for emergency department use by patients with. In the united states, sickle cell trait is carried by 7% to 8% of people of african ancestry, and the sickle hemoglobinopathies are estimated to affect 90,000 to 100,000 people. Pdf stroke is a frequent and severe complication in adults with sickle cell disease. In this article we plan to discuss the clinical features of the disorder and describe current concepts. Basic pathophysiology and new therapeutic options dimitris loukopoulos, md foundation for biomedical research of the academy of athens, greece.
Stroke is a common and potentially devastating manifestation of sickle cell disease scd that can affect children and adults. Pathophysiology of stroke in sickle cell disease request pdf. In predicting the risk of stroke in scd children without a previous event, studies have revealed signs or symptoms that often occur before a stroke. Pathophysiology and treatment of stroke in sicklecell. Symptomatic stroke is associated with intimal disease of the large cerebral arteries. Major complications of sickle cell disease and nursing. By far, the strongest predictor of stroke in children with scd is a previous stroke. Stroke in children with sickle cell disease internet. Sickle cell disease scd is a protean disorder caused by elevations of intraerythrocyte and total blood viscosity. Stroke prevention and treatment in sickle cell disease. Predictors of stroke in sickle cell disease children.
Sicklecell anaemia is the most common cause of stroke in children, and stroke is one of the most devastating complications of sicklecell disease. Sickle cell anaemia is the most common cause of stroke in children, and stroke is one of the most devastating complications of sickle cell disease. Hemoglobin is a protein in red blood cells that carries oxygen throughout the body. Acute ischemic and hemorrhagic stroke in sickle cell disease.
Ensure patients understand how to take pain medicines to manage acute pain. Sicklecell disease is one of the most common inherited anemias of the hematopoietic system. Hypoxiainduced gelation of hemoglobin s hbs deforms the erythrocyte and its membrane and causes massive cation loss as well as increased erythrocyte surface expression of adhesion molecule receptors. Approximately 8% of the africanamerican population carries the sickle cell trait. Expert panel report epr, 2014 is to synthesize the available scientific evidence on sickle cell disease and offer guidance to busy primary care clinicians. Recent findings new concepts in the pathophysiology of central nervous system events in. The role of exchange transfusion in patients with stroke with sickle cell disease is based on pediatric studies. Introduction stroke is a devastating and potentially fatal complication to sickle cell disease. Sickle cell disease scd is an inherited blood disorder characterized by chronic anemia. Silent stroke, defined as cerebral infarction in the absence of overt clinical neurologic symptoms, is often due to microinfarcts suggestive of microvascular disease.
Sickle cell disease scd and its variants are genetic disorders resulting from the presence of a mutated form of hemoglobin, hemoglobin s hbsref1. Ischemic stroke often causes physical and cognitive disability. Discontinuingprophylactic transfusions used to prevent stroke in sickle cell disease. The origin of scd lies in the malarial regions of the tropics where carriers are protected against death from malaria and hence enjoy an evolutionary. If you have scd, there is a problem with your hemoglobin. Pathophysiology and treatment of stroke in sicklecell disease ncbi. A manual or automated exchange transfusion should be performed with a goal. Stroke or reversion to abnormal tcd velocities adams rj, brambilla d.
Anie 6 and wale atoyebi 7 1 paediatric haematology, evelina london childrens hospital, guys and st thomas nhs trust. Recurrent and unpredictable episodes of vasoocclusion are the hallmark of sickle cell disease. Stroke risk and its management in patients with sickle. Exchange blood transfusion compared with simple transfusion for first overt stroke is associated with a lower risk of subsequent stroke. Overt stroke in sicklecell disease occurrence of stroke in children with sicklecell disease is clinically identi. Challenges in management include distinguishing acute stroke from other central nervous system complications of the disease such as seizures, meningitis. Sickle cell disease scd is a group of blood disorders typically inherited from a persons parents. April 23, 2020 sickle cell disease scd affects 100,000 individuals in the united states and millions globally. Chronic inherited autosomal recessive condition commonest genetic condition worldwide first described in 1910 the pathophysiology of scd arises from haemolytic anaemia and acute vasoocclusion.
Sickle cell disease scd is a single gene disorder causing a debilitating systemic syndrome characterised by chronic anaemia, acute painful episodes, organ infarction and chronic organ damage and by a significant reduction in life expectancy. Pathophysiology of stroke in sickle cell disease hillery. Optimizing primary stroke prevention in sickle cell anemia stop 2 trial investigators. Stroke as a complication of sickle cell disease internet. Expert panel report, 2014 ix foreword the purpose of the evidence based management of sickle cell disease. The figure depicts some of the pathophysiologic components of the disorder in simplified form. With scd, the hemoglobin forms into stiff rods within the red blood cells.
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