Adams RJ, McKie VC, Hsu L, et al.

Prevention of a first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial Doppler ultrasonography.
N Engl J Med. 1998;339:5-11.
Description: A prospective randomized clinical trial demonstrating that a chronic transfusion program can reduce the risk of stroke in children with abnormal transcranial Doppler results.
PubMed citation number: 9647873

Bellet PS, Kalinyak KA, Shukla R, et al.

Incentive spirometry to prevent acute pulmonary complications in sickle cell disease.
N Engl J Med. 1995;333:699-703.
Description: A prospective randomized trial demonstrating that incentive spirometry can reduce the incidence of acute pulmonary complications in patients with thoracic bone infarctions.
PubMed citation number: 7637747

Charache S, Terrin ML, Moore RD, et al.

Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. Investigators of the MultiCenter Study of Hydroxyurea in Sickle Cell Anemia.
N Engl J Med. 1995;332:1317-22.
Description: A multicenter phase III randomized study that established the efficacy of hydroxyurea in the treatment of adults with sickle cell disease.
PubMed citation number: 7715639

Fixler J and Styles L.

Sickle Cell Disease.
Pediatr Clin N Am. 2002;49:1193-1210.
Description: A comprehensive review of the clinical manifestations and complications of sickle cell disease.
PubMed citation number: 12580362

Miller ST, Wright E, Abboud M, et al.

Impact of chronic transfusion on incidence of pain and acute chest syndrome during the Stroke Prevention Trial (STOP) in sickle cell anemia.
J Pediatr. 2001;139:785-89.
Description: A randomized trial designed for the prevention of stroke but also demonstrated protective effect of chronic transfusion for pain crisis and acute chest syndrome.
PubMed citation number: 11743502

Platt OS, Brambilla DJ, Rosse WF, et al.

Mortality in sickle cell disease. Life expectancy and risk factors for early death. N Engl J Med. 1994;330:1639-44.
Description: A landmark prospective study of over 3000 patients addressing survival and risk factors for mortality in sickle cell disease.
PubMed citation number: 7993409

Platt OS, Thorington BD, Brambilla DJ, et al.

Pain in sickle cell disease. Rates and risk factors.
N Engl J Med. 1991;325:11-6. Description: A prospective study of the pain crises and its relationship to the risk of death in over 3000 patients.
PubMed citation number: 1710777

Steinberg MH.

Management of sickle cell disease.
N Engl J Med. 1999;340:1021-30.
Description: A concise review of major issues and therapies for the management of patients with sickle cell disease.
PubMed citation number: 10099145

Steinberg MH, Barton F, Castro O, et al.

Effect of hydroxyurea on mortality and morbidity in adult sickle cell anemia. Risks and benefits up to 9 years of treatment.
JAMA. 2003;289:1645-51.
Description: Follow up analysis of study reported by Charache et al. demonstrating reduced mortality for patients taking hydroxyurea.
PubMed citation number: 12672732

Vichinsky EP, Neumayr LD, Earles AN, et al.

Causes and outcomes of acute chest syndrome in sickle cell disease. National Acute Chest Syndrome Study Group.
N Engl J Med. 2000;342:1855-65.
Description: A prospective multicenter trial analyzing 671 episodes of acute chest syndrome. Clinical presentation, causes and outcomes are addressed.
PubMed citation number: 10861320

Vichinsky EP, Haberkern CM, Neumayr L, et al.

A comparison of conservative and aggressive transfusion regimens in the perioperative management of sickle cell disease. The Preoperative Transfusion in Sickle Cell Disease Study Group.
N Engl J Med. 1995;333:206-213.
Description: A multicenter randomized clinical trial comparing simple transfusion with a regimen to reduce hemoglobin S below 30% in more than 600 surgical procedures.
PubMed citation number: 7791837

Walters MC, Storb R, Patience M, et al.

Impact of bone marrow transplantation for symptomatic sickle cell disease: an interim report. Multicenter investigation of bone marrow transplantation for sickle cell disease.
Blood. 2000;95:1918-24.
PubMed citation number: 10706855

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