Anesthesia and Hemoglobinopathies

Firth, Paul G.

doi:10.1016/j.anclin.2009.05.001

Cited by 28 publications

(40 citation statements)

References 77 publications

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“…The cardiac manifestations vary depending on the severity of illness. Cardiac surgery can exacerbate these disease states due to surgical stress, use of cardiopulmonary bypass, hypothermia, and low flow states 5,6 . We review the literature on anesthetic considerations for sickle cell disease, thalassemia, and cardiac surgery.…”

Section: Discussionmentioning

confidence: 99%

Case 10—2016

Farooqui

Chaney

Staikou

et al. 2016

Journal of Cardiothoracic and Vascular Anesthesia

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Section: Discussionmentioning

confidence: 99%

Case 10—2016

Farooqui

Chaney

Staikou

et al. 2016

Journal of Cardiothoracic and Vascular Anesthesia

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“…It is inherited on chromosome 11 and patients are homozygous for two mutant alleles. 1 Anaesthetic management of these patients is dictated by the disease itself, the complications of its treatment (iron overload from multiple transfusions) and the use of preventive measures (chelating agents).…”

Section: Discussionmentioning

confidence: 99%

“…5 Major β-thalassaemia patients may present with pulmonary hypertension and vascular damage (vasculopathy), secondary to disturbed nitric oxide physiology. 1 Angina, conduction defects, pericarditis, arrhythmias and sudden death are all more likely in patients with thalassaemia and iron overload (> 2500 μg/L serum ferritin). A hyper-dynamic circulation with a high cardiac output, a compensatory mechanism to chronic anaemia, and decreased blood oxygen carrying capacity may also be present.…”

Section: Cardiovascularmentioning

confidence: 99%

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Anaesthesia for a patient with B-thalassaemia

Giannakikou

Karra

2015

Southern African Journal of Anaesthesia and Analgesia

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Β-Thalassaemia is a rare hereditary disease caused by partial or complete deficiency of β-haemoglobin chain synthesis. There is a lot of literature regarding anaesthetic management in other haemoglobinopathies (i.e. sickle cell disease), especially in the paediatric population, but there is scarce information regarding β-thalassaemia major in adults. With current medical management, β-thalassaemia major patients survive to adulthood and may present for a variety of surgical procedures, even unrelated to their disease process. It is important for the anaesthetist to be familiar with the pathophysiology of β-thalassaemia major and how the disease itself and its treatment or complications (iron deposition from multiple transfusions) can affect anaesthesia. A case of a 51-year-old woman with severe β-thalassaemia undergoing laparoscopic cholecystectomy is presented. The anaesthetic management and systematic review of the perioperative concerns in severe β-thalassaemia are discussed.

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“…Adequate hydration preoperatively combined with avoidance of perioperative hypoxia, hypothermia, and acidosis, the triggers for sickling, will reduce the sickle-induced complications. [14–16] Basic supportive care, including adequate analgesia, early mobilization, and oxygen supplementation to prevent hypoxemia, is the mainstay of postoperative management.…”

Section: Discussionmentioning

confidence: 99%

Anesthetic management of a patient with sickle β⁺ thalassemia

et al. 2011

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Sickle cell disease is a congenital condition and its most common clinical manifestation is anemia due to chronic hemolysis. Persistent and accelerated hemolysis associated with multiple transfusions is a recognized risk factor for the development of cholelithiasis. The occurrence of gallstones is one of the most important manifestations of sickle cell disease in the digestive tract. Most gallstones are pigmented and characteristically occur at younger ages and the prevalence of cholelithiasis increases progressively with age, affecting 50% of young adults. Cholecystectomy is the most common surgical procedure performed in sickle cell disease patients. Anesthesia in this population of patients for major surgeries deserves special attention due to various complications particularly silent infarctions of end organs are common. We are reporting a 14-year-old girl diagnosed with sickle cell anemia and β+ thalassemia with cholelithiasis went for cholecystectomy under general anesthesia. Although the patient has both β+ thalassemia and sickle cell disease component, the latter is of more concern for anesthesia.

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Anesthesia and Hemoglobinopathies

Cited by 28 publications

References 77 publications

Case 10—2016

Case 10—2016

Anaesthesia for a patient with B-thalassaemia

Anesthetic management of a patient with sickle β⁺ thalassemia

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Anesthesia and Hemoglobinopathies

Cited by 28 publications

References 77 publications

Case 10—2016

Case 10—2016

Anaesthesia for a patient with B-thalassaemia

Anesthetic management of a patient with sickle β+ thalassemia

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Anesthetic management of a patient with sickle β⁺ thalassemia