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1

National Heart, Lung, and Blood Institute. Division of Blood Diseases and Resources. The management of sickle cell disease. 4th ed. Bethesda, MD: The Institute, 2002.

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2

Al-Jamʼa, Ali H. Management manual of sickel cell disease. [Saudi Arabia: Qatif Central Hospital], 1992.

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3

Rosse, Wendell F. Transfusion support for patients with sickle cell disease. Bethesda, Md: AABB Press, 1998.

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4

Anie, Kofi. Coping with sickle cell disease & pain: A self-help manual for children. London: Brent Sickle Cell & Thalassaemia Centre, 1998.

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5

Oni, L. Care and management of women with sickle cell disease in maternity care settings. London: Brent Sickle Cell & Thalassaemia Centre, 2002.

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6

Peak, Lizabeth. Sickle cell disease. Detroit: Lucent Books, 2008.

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7

Serjeant, Graham R. Sickle cell disease. 2nd ed. Oxford: Oxford University Press, 1992.

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8

Eboh, Winifred Oluchukwu. Sickle cell disease. (Birmingham): Birmingham Sickle Cell & Thalassaemia Centre, 1993.

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9

Sickle cell disease. 2nd ed. Oxford: Oxford University Press, 1992.

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10

Sickle cell disease. Oxford: Oxford University Press, 1985.

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11

Peak, Lizabeth. Sickle cell disease. Detroit: Lucent Books, 2008.

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12

Jones, Phill. Sickle cell disease. New York: Chelsea House, 2008.

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13

Understanding sickle cell disease. Jackson: University Press of Mississippi, 1995.

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14

McCormick, Marie, Henrietta Awo Osei-Anto, and Rose Marie Martinez, eds. Addressing Sickle Cell Disease. Washington, D.C.: National Academies Press, 2020. http://dx.doi.org/10.17226/25632.

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15

Meier, Emily Riehm, Allistair Abraham, and Ross M. Fasano, eds. Sickle Cell Disease and Hematopoietic Stem Cell Transplantation. Cham: Springer International Publishing, 2018. http://dx.doi.org/10.1007/978-3-319-62328-3.

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16

Midence, Kenny. Sickle cell disease: A psychosocial approach. Oxford: Radcliffe Medical Press, 1994.

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17

Howard, Jo, and Paul Telfer. Sickle Cell Disease in Clinical Practice. London: Springer London, 2015. http://dx.doi.org/10.1007/978-1-4471-2473-3.

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18

Ennis, Sylvia. The natural choice: Sickle cell disease/ thalassaemia. New Delhi: B.Jain Publishers, 1994.

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19

Anionwu, Elizabeth N. Sickle cell disease: A guide for families. Harlow: Longman, 1991.

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20

B, Jibril Harun, ed. Sickle cell disease: A guide for families. London: Collins, 1986.

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21

Renaissance of sickle cell disease research in the genome era. London: Imperial College Press, 2007.

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22

Managing sickle cell disease in low-income families. Philadelphia: Temple University Press, 1994.

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23

Alvin, Silverstein, Silverstein Virginia B, and Nunn Laura Silverstein, eds. What you can do about sickle cell disease. New York, NY: Enslow Publishing, 2016.

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24

Alan, Sacerdote, ed. Hope and destiny: The patient's and parent's guide to sickle cell disease and sickle cell trait. Roscoe, Ill: Hilton Pub. Co., 2002.

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25

Alan, Sacerdote, ed. Hope and destiny: The patient's and parent's guide to sickle cell disease and sickle cell trait. Roscoe, Ill: Hilton Pub. Co., 2006.

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26

Mayfield, Eleanor. New hope for people with sickle cell anemia. [Rockville, MD: Dept. of Health and Human Services, Public Health Service, Food and Drug Administration, 1996.

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27

Mayfield, Eleanor. New hope for people with sickle cell anemia. [Rockville, MD: Dept. of Health and Human Services, Public Health Service, Food and Drug Administration, 1997.

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28

Acuña-Castroviejo, Darío, and Iryna Rusanova. Sickle cell disease: A new vision for an old problem. Hauppauge] New York: Nova Biomedical, 2013.

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29

Uncertain suffering: Racial healthcare disparities and sickle cell disease. Berkeley: University of California Press, 2009.

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30

Jones, P. Allen. I only cry at night: Living with sickle cell disease. [Charleston, S.C.?: P. Allen Jones], 2011.

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31

Jones, P. Allen. I only cry at night: Living with sickle cell disease. Charleston, S.C., USA: P. Allen Jones, 2011.

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32

James, Eckman, and Hsu Lewis, eds. Hope and destiny: The patient and parent's guide to sickle cell disease and sickle cell trait / Allan F. Platt Jr., James Eckman, and Lewis Hsu. 3rd ed. Indianapolis, Ind: Hilton Pub. Co., 2011.

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33

Szabova, Alexandra, and Kenneth R. Goldschneider. Sickle Cell Disease. Oxford University Press, 2013. http://dx.doi.org/10.1093/med/9780199764495.003.0040.

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0.2% of African-Americans have sickle cell anemia while, with 8% to 10% have sickle cell trait. This chapter provides an overiew of the etiology, pathophysiology, and treatment of sickle cell anemia as they affect anesthetic management—before, during, and after surgery.
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34

Franklin, Andrew. Sickle Cell Disease. Edited by Matthew D. McEvoy and Cory M. Furse. Oxford University Press, 2017. http://dx.doi.org/10.1093/med/9780190226459.003.0084.

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Sickle cell disease, a hemoglobinopathy that affects multiple organ systems, is a complex disease entity that presents unique challenges during the perioperative period. The hallmark of sickle cell disease, vaso-occlusion, results from sickling of erythrocytes containing hemoglobin of abnormal conformation due to genetically mutated beta globin genes. The perioperative clinician must properly care for acute sickle cell crises including acute painful episodes and acute chest syndrome, and safely care for the sickle cell patient through the preoperative, intraoperative, and postoperative phases of surgical treatment. Both acute painful episodes and acute chest syndrome result from vaso-occlusive crises, and early stabilization of these emergencies is crucial to ensuring a positive patient outcome. The singular perioperative objective for the care of sickle cell disease patients is both simple and daunting: to achieve physiologic homeostasis in patients with preexisting multiorgan dysfunction undergoing a series of physiologic insults during and after surgery.
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35

Hydroxyurea for the treatment of sickle cell disease. Rockville, MD: Agency for Healthcare Research and Quality, 2008.

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36

Lance, Eboni I., and Andrew W. Zimmerman. Sickle Cell Anemia. Oxford University Press, 2017. http://dx.doi.org/10.1093/med/9780199937837.003.0079.

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Sickle cell disease is a genetic hematological disorder involving red blood cells that become deformed when stressed. Patients with homozygous hemoglobin SS disease often have multiple systemic and neurologic complications, particularly stroke. Intellectual disability is commonly seen in the population, in patients with and without a history of stroke, attributed to different underlying mechanisms of brain injury. Autism is rare and not described in sickle cell disease in the literature to date. Many treatments (chronic transfusion therapy, hydroxyurea, bone marrow transplant) are in trials at this time to see if risk of stroke and other neurologic complications can be reduced (ClinicalTrials.gov identifiers: NCT01425307, NCT01389024, NCT00152113).
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37

Brown, Ronald T., ed. Comprehensive Handbook of Childhood Cancer and Sickle Cell Disease. Oxford University Press, 2006. http://dx.doi.org/10.1093/oso/9780195169850.001.0001.

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Over recent decades, tremendous advances in the prevention, medical treatment, and quality of life issues in children and adolescents surviving cancer have spawned a host of research on pediatric psychosocial oncology. This important volume fulfills the clear need for an up-to-date, comprehensive handbook for practitioners that delineates the most recent research in the field--the first of its kind in over a decade. Over 60 renowned authors have been assembled to provide a thorough presentation of the state-of-the art research and literature, with topics including: -Neuropsychological effects of chemotherapy and radiation therapy -Bone marrow transplantation -Important issues about quality of life during and following treatment -Collaborative research among child-focused psychologists -Standards of psychological care for children and adolescents -Stress and coping in the pediatric cancer experience -The role of family and peer relationships The Comprehensive Handbook of Childhood Cancer and Sickle Cell Disease represents both multidisciplinary and international efforts, an alliance between physicians and parents, and a combination of research and service. With a wealth of information of great interest to patients and their families, this volume will also be a welcome resource to the psychologists, psychiatrists, pediatricians, oncologists, nurses, and social workers who confront these issues as they help children and their families through the treatment, recovery, and grieving processes.
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38

Haymann, Jean-Philippe, and Francois Lionnet. The patient with sickle cell anaemia. Edited by Giuseppe Remuzzi. Oxford University Press, 2015. http://dx.doi.org/10.1093/med/9780199592548.003.0167.

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In sickle cell anaemia (SCA) a single mutation in the haemoglobin beta-globin gene is responsible for a pleomorphic phenotype leading to acute and chronic life-threatening complications. Healthcare management programmes, patient and family education, infection prophylaxis (especially in childhood), and long-term treatment for some patients (such as hydroxyurea) have significantly improved survival, giving rise to some new long-term issues.Sickle cell-associated nephropathy (SCAN) leads in some cases to chronic renal failure with a significant impact on survival. SCAN is characterized by an increased effective plasma renal flow and glomerular filtration rate, glomerular hypertrophy, and damaged vasa recta system leading to albuminuria and impaired urinary concentration.Early onset of hyperfiltration occurs in 60% of SCA patients often associated with microalbuminuria. SCAN risk factors are still under investigation, but may be related to chronic haemolysis at an early time point. Other lesions in patients with sickle cell anaemia include papillary necrosis, and recurrent acute kidney injury in association with crises or infections.ACEI are recommended if there is proteinuria. There is no current agreement on whether angiotensin-converting enzyme inhibitors (ACEI) should be introduced earlier, but systematic screening for microalbuminuria and hypertension, and avoidance of nephrotoxic agents are strongly advised.Patients with sickle cell trait (carriers for sickle cell anaemia) are prone to microscopic haematuria and abnormalities of the vasa recta have been described. A very rare tumour, renal medullary carcinoma, is largely restricted to this group (in whom it is still extremely rare). Increased risk of other renal problems is still largely hypothetical rather than proven.The prevalence of nephropathies in other sickle cell diseases (in particular haemoglobin SC disease) is much lower.
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39

Jain, Shilpa, and Mark T. Gladwin. Sickle crisis in the critically ill. Oxford University Press, 2016. http://dx.doi.org/10.1093/med/9780199600830.003.0275.

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Sickle cell disease crises are precipitated by an acute occlusion of microvessels, which can lead to end organ ischaemia reperfusion injury and acute haemolysis. Acute fat emboli syndrome, acute lung injury (the acute chest syndrome), acute pulmonary hypertension, and cor pulmonale, haemorrhagic and occlusive stroke, and systemic infection represent the most common life-threatening complications observed in current ICU practice. General principles of management in all patients admitted to the critical care unit are hydration, antibiotics, pain control, and maintenance of oxygenation and ventilation. Red blood cell transfusion therapy is the treatment of choice for most complications of sickle cell disease requiring intensive care management. Transfusion of sickle negative, leukoreduced red blood cells, phenotypically matched for Rhesus and Kell antigens is the minimum standard of care in sickle cell disease patients as they have a high incidence of red blood cell alloimmunization.
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40

F, Whitten Charles, Bertles John F. 1925-, National Association for Sickle Cell Disease (U.S.), and New York Academy of Sciences., eds. Sickle cell disease. New York, N.Y: New York Academy of Sciences, 1989.

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41

Anionwu, Elizabeth, and H. Jibril. Sickle Cell Disease. 2nd ed. Longman, 1992.

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42

Serjeant, Graham R. Sickle Cell Disease. Oxford University Press, USA, 1988.

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43

Midence, Kenny. Sickle Cell Disease. Scovill-Paterson, 1994.

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44

Anionwu, Elizabeth, and H. Jibril. Sickle Cell Disease. Longman, 1986.

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45

Ferrari, Lynne R. Sickle Cell Disease. Edited by Kirk Lalwani, Ira Todd Cohen, Ellen Y. Choi, and Vidya T. Raman. Oxford University Press, 2018. http://dx.doi.org/10.1093/med/9780190685157.003.0051.

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Sickle cell anemia is a disease that combines molecular biology, clinical features, biochemistry, pathology, natural selection, population genetics, gene expression, and genomics and is the world’s most common life-threatening monogenic disorder. Clinical features include anemia; painful crisis especially in fingers, chest, and long bones; hemolysis; splenic infarction resulting in functional asplenia; and microinfarction leading to neurologic and renal impairment. The maintenance of adequate body temperature with active warming devices and warmed intravenous fluids, monitoring hydration and urine output, providing supplemental oxygen, and limiting surgical and anesthesia times to reduce pulmonary complications constitute the best management for patients with sickle cell disease.
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46

Ng, Ann, and Erin S. Williams. Sickle Cell Disease. Edited by Erin S. Williams, Olutoyin A. Olutoye, Catherine P. Seipel, and Titilopemi A. O. Aina. Oxford University Press, 2018. http://dx.doi.org/10.1093/med/9780190678333.003.0033.

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Sickle cell anemia (sickle cell disease) is a common hemoglobinopathy with anywhere from 90,000 to 100,000 Americans affected. This chronic condition has a predominance in populations of African descent, occurring in approximately 1 out of 365 African American births, compared to 1 out of 16,300 Hispanic births. The sickle cell trait can be detected in 1 of 13 African American births. One of the most common complications associated with sickle cell anemia, vaso-occlusive crises by sickled cells, results in severe pain. Other issues associated with this condition include acute chest syndrome, lung infections, end organ damage, and stroke. With improvements in the management and prevention of pain crises, infection, and other systemic involvement, these patients are living longer, thus increasing the potential for surgical needs. Whether it is for routine surgeries or surgeries that are due to the natural history of the disease; the pediatric anesthesiologist must be knowledgeable of the management of these patients in order to prevent morbidity and mortality.
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47

Samuel, Charache, and Johnson Cage S, eds. Sickle cell disease. Philadelphia: W.B. Saunders, 1996.

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48

Sickle Cell Disease Patient. Tettah-A'Domeno Co, 1998.

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49

McCann, Shaun R. Molecules, genes, and gene therapy. Oxford University Press, 2016. http://dx.doi.org/10.1093/med/9780198717607.003.0009.

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The twenty-first century has brought many innovations in haematology, with improved diagnostic technology, which may inform treatment choices for malignant diseases, and a better understanding of the genetics and/or epigenetics underlying many diseases. Unfortunately, the aetiology of most of these diseases still eludes us, and some common diseases such as sickle cell disease await simple, inexpensive, and widely available curative treatment. For reasons that are often obscure, some diseases have become fashionable and attract large research financial backing, whereas some do not. With the advent of advanced technology and an improved understanding of disease mechanisms, most haematological malignancies should enjoy the same success as the treatment of childhood acute lymphoblastic leukaemia and chronic myeloid leukaemia.
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50

Sickle Cell Disease (Genes and Disease). Chelsea House Pub (L), 2008.

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