Hereditary spherocytosis in children?

summary

Hereditary spherocytosis (HS) in children is a kind of hereditary hemolytic disease caused by congenital abnormality of erythrocyte membrane cytoskeleton protein. Its main feature is that there are many small spherocytes in peripheral blood. Clinically, anemia, jaundice, splenomegaly, increased spherical red blood cells in the blood, the course of chronic anemia, accompanied by repeated acute hemolysis are the main characteristics. Hereditary spherocytosis in children? Next, I'd like to share my views with you.

Hereditary spherocytosis in children?

The disease is more common in children or childhood, from asymptomatic to life-threatening anemia, more severe in the newborn or infant onset. The severity of clinical manifestations can be very different, and the severity of different patients in the same family is often consistent.

Most of the children had dominant inheritance, and the clinical manifestations were mild to moderate anemia; Very few children with recessive homozygous or allele mutations, clinical manifestations of severe HS. Anemia, jaundice and hepatosplenomegaly are the most common clinical manifestations of HS,

All of them exist at the same time or occur alone. Most HS had mild to moderate anemia, moderate splenomegaly and intermittent jaundice. A few HS symptoms are mild, although there is hemolysis, but due to bone marrow erythroid compensatory hyperplasia, generally no anemia, no or only mild jaundice, no or mild splenomegaly.

matters needing attention

After the appearance of hereditary spherocytosis, there are many harmful manifestations, and it is impossible for him to recover without treatment. Therefore, we should not wait blindly. We must pay attention to reasonable rest and diet conditioning to help patients recover as soon as possible. Folic acid should be added when it is serious, so as to prevent aplastic crisis.