All of the following statements are true about sickle cell disease except (AI 2010)
|A||Patient may require frequent blood transfusions|
|B||Acute infection is the most common cause of mortality before 3 years of age.|
|C||There is positive correlation between conc HBS and polymerization of HBS.|
|D||Patient presents early in life before 6 months of age.|
Patient presents early in life before 6 months of age.
(Ref. Nelson. 18th ed., Chapter 462)
1. Infants with sickle cell anemia have abnormal immune function.
2. As early as 6 month of age, some children, and by 5 yr of age, most children have functional asplenia.
3. Bacterial sepsis is one of the greatest causes of morbidity and mortality in this patient population.
4. Children with sickle cell anemia also have deficient levels of serum opsonins of the alternate complement pathway against pneumococci.
5. Regardless of age, all patients with sickle cell anemia are at increased risk for infection and death as a result of bacterial infection, particularly with encapsulated organisms, such as Streptococcus pneumoniae and Haemophilus influenzae type B.
6. Children with sickle cell anemia should receive prophylactic oral penicillin at least until 5 yr of age. No established guidelines exist for penicillin prophylaxis beyond 5 yr of age.
Thus sickle cell anemia can present before 6 Months of age but usually presents after 6 Months. So option D is answer of exclusion here.
Sequence of events in sickle cell anemia:
1. Mild hemolytic anemia – appear by 10-12 week of age [HbF (α2 2) is protective.]
2. Splenomegaly – after 6 month. Later on autosplenectomy
3. Veno occlusive crisis – between 8-12 month of age
4. Aplastic crisis – Any time offer 6 month.