All of the following statements are true about sickle cell disease EXCEPT:
- Patients of Sickle cell anemia rarely presents before 6 months age as abundance of HbF inhibits polymerisation of HbS. CLINICAL FEATURES OF SICKLE CELL ANEMIA: Hand foot syndrome / dactylitis of bones of hands, feet Priapism & erectile dysfunction Stroke & retinopathy- loss of visual acuity & blindness Auto splenectomy d/t splenic INFARCTS (Spleen becomes non palpable) Chronic hemolysis - pt may require frequent blood transfusions Renal Involvement- papillary necrosis & hyposthenuria cardiomegaly Infections mainly by encapsulated bacteria . - Fever in a child with sickle cell anemia is a medical emergency, require prompt medical evaluation and delivery of antibiotics because of increased risk of bacterial infection and subsequent high moality rate. - Positive correlation is seen b/w concentration of HbS & polymerization of HbS
Patient may require frequent blood transfusions
Acute infection is the most common cause of moality before 3 years of age
There is positive correlation between concentration of HbS and polymerisation of HbS
Patient presents early in life before 6 months of life
Hemolytic anemia