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## Sickle Cell Disease (Sickle Cell Anemia) ### Sickle Cell Disease - Sickle Cell Anemia is an **autosomal recessive** disorder. ### Factors Affecting Sickling Process - **Degree of oxygenation: Hypoxia promotes sickling** - **pH: Low pH favors sickling** - **Dehydration of the patient: Dehydrat...

## Sickle Cell Disease (Sickle Cell Anemia) ### Sickle Cell Disease - Sickle Cell Anemia is an **autosomal recessive** disorder. ### Factors Affecting Sickling Process - **Degree of oxygenation: Hypoxia promotes sickling** - **pH: Low pH favors sickling** - **Dehydration of the patient: Dehydration promotes sickling** - **Concentration of other types of hemoglobins:** High Hb F decreases the severity of the disease. This is seen in newborns and when Hb S is combined with **hereditary persistence of fetal hemoglobin (HPFH)** ## Sickle Cell Crises - **Vasoocclusive crises:** Increase in blood viscosity restricts blood flow to an organ - **Hemolytic crises:** Acute accelerated drop in Hb levels and RBC's break down at a faster rate. - **Infectious crises:** - Abnormal splenic function, depressed immune function - _Streptococcus pneumoniae_ is the major infectious agent among children. - **Aplastic crises:** Caused by _Parvovirus B19_ resulting in infection and fever