Hi, this is Tom, and in this episode I'm going to be going through hereditary spherocytosis.

And you can find notes at 0.0.5 and in the 0 to finals pediatrics book.

And you can find flashcards and questions at members.0tofinals.com. So let's jump straight in. Hereditary

spherocytosis is a genetic condition in which the red blood cells are sphere-shaped. This makes

them fragile and more easily destroyed as they pass through the spleen. It's usually

autosomal dominant in its inheritance,

and it's the most common inherited hemolytic anemia

in people of Northern European descent.

Let's talk about the presentation.

The key presenting features of hereditary spheroocytosis are jaundice because red blood cells

release bilirubin when they're destroyed, anemia due to red blood cell destruction or

hemolysis, spleenomegaly with an enlarged spleen, and gallstones. Patients can have episodes of hemolytic crisis, sometimes triggered by infections,

with increased hemolysis or destruction of red blood cells, leading to acutely worsening

anemia and jaundice.

They can also develop a plastic crisis,

which is often triggered by parvovirus B-19 infection.

During an aplastic crisis,

red blood cell production in the bone marrow decreases,

and this leads to acute worsening of the anemia.

Atompt it for you, parvovirus B-19 causes slapped cheek syndrome in children,

which features nonspecific viral symptoms that are followed two to five days later

by a bright red rash on both cheeks, which gives the slapped cheek appearance,

...