Sickle cell disease

Sickle cell disease Dr Moutasem Almashour

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What is Sickle Cell Anemia (SCA)? ,[object Object],[object Object],[object Object]

[object Object],[object Object],Haemoglobin A two alpha two beta A2 two alpha two delta F two alpha two gamma

C ommon types of Sickle Cell Disorders Type of anaemia Hemoglobin variation comment Sickle Cell Anemia Sickle haemoglobin (HbS) + Sickle haemoglobin Most Severe – No HbA Hemoglobin S-Beta thalassemia ,[object Object],Mild form of Sickle Cell Disorder Hemoglobin S-C disease ,[object Object],Mild form of Sickle Cell Disorder Sickle Cell Trait Sickle haemoglobin (S) + Normal haemoglobin (A)

[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],*Gupta, Kirchner, Nicholson, Adams, Schechter, Noguchi, Steinberg, JCI 1991

Epidemiology ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

Features of sickle-cell disease in Bahrain ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

Pathophysiology ,[object Object],[object Object],[object Object],[object Object]

Pathophysiology cont. ,[object Object],[object Object],[object Object],[object Object]

Red Blood Cells from Sickle Cell Anemia ,[object Object],OXY-STATE DEOXY-STATE

Clinical Features of Sickle Cell Disease ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

Clinical Features of Sickle Cell Disease ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

Clinical Features of Sickle Cell Disease ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

Defective Urine Concentrating Ability in Sickle Cell anaemia ,[object Object],[object Object],[object Object],Gupta, Kirchner, Nicholson, Adams, Schechter, Noguchi, Steinberg, JCI 1991

Clinical Features of Sickle Cell Disease in infants ,[object Object],[object Object],[object Object],[object Object],[object Object]

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Factors That Can Cause Sickle Cell Crises ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

Measures for Preventing Pain Crises in Patients with Sickle Cell Disease ,[object Object],[object Object],[object Object],[object Object]

Preoperative anesthesia considerations for SCD ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

Preoperative Preparation ,[object Object],[object Object],[object Object],[object Object],[object Object]

Preoperative Preparation ,[object Object],[object Object],[object Object],[object Object]

Investigations ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

Preoperative Transfusion ,[object Object],[object Object],[object Object]

Indications for transfusion therapy in sickle-cell disease ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

[object Object],[object Object],[object Object],[object Object],Preoperative Transfusion

[object Object],[object Object],[object Object],Preoperative Transfusion

General vs. regional ,[object Object],[object Object],[object Object],[object Object]

Predictors of Postoperative SCD complications ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

Predictors of Postoperative SCD complications in the first two years of life ,[object Object],[object Object],[object Object],[object Object]

Mortality/Morbidity ,[object Object],[object Object],[object Object],[object Object]

Postoperative Management ,[object Object],[object Object],[object Object]

Pregnant women with sickle cell disease ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

Websites http://www.sicklecellsociety.org/: Another Great Site information, Counselling and Caring for those with Sickle Cell Disorders and their families: UK based http://www.sicklecelldisease.org/: Sickle Cell Disease Association of America The Human Genome Project Sickle Cell Education Site at http://www.massinteraction.org/html/genome/ http://www.ascaa.org/ American Sickle Cell Anemia Association ASCAA was founded in 1971 and is the oldest sickle cell research, education, and social services organization in the United States. http://www.ncd.gov/ http://www.painfoundation.org/

Sickle cell disease

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Sickle cell disease