a brief explanation of the most important anemia in childhood references: nelson pediatrics, O.P.Ghai, UpToDate

1. Anemia In Childhood
-Ladi Anudeep
ISM-IUK

6. • Anemia is present when the hemoglobin level is more than two
standard deviations below the mean for the child’s age and sex

9. Hemolytic anemias

10. Clinical Features of hemolytic anemia
• Pallor
• fatigue
• Weakness
• Jaundice
• Hemolytic facies

15. Aplastic Anemia

16. Hemoglobinopathies
Sickle cell Disease
• Sickle cell anemia is an autosomal recessive disease that results from the
substitution of valine for glutamic acid at position 6 of the beta-globin
gene.
• Patients who are homozygous for the HbS gene have sickle cell disease.
Patients who are heterozygous for the HbS gene have sickle trait
• Crisis in sickle cell disease:
1. Vaso-occlusive crisis
2. Acute Chest Syndrome
3. Sequestration crisis
4. Aplastic Crisis

18. • Thalassemia
• caused by defects in the globin gene, are the most common
monogenic disease

20. G6PD deficiency Anemia
• Glucose-6- phosphate dehydrogenase (G6PD) deficiency is the most common red cell enzyme
deficiency. It is an X-linked recessive disease with full expression in affected males
• After an oxidant exposure, hemoglobin is oxidized to methemoglobin and denatured to form
intracellular inclusions also known as Heinz bodies.
• These Heinz bodies get attached to the red cell membrane and aggregate intrinsic membrane
proteins such as band 3.
• Reticuloendothelial cells detect these membrane changes as antigenic sites and ingest a part of
the red cell.
• This partly phagocytosed cell, called 'bite’ cell, has a shortened half-life.
• Drugs that cause oxidant formation are:
• Sulfonamides
• Antimalarials: primaquine, quinine
• Aspirin
• dapsone.