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

And you can find notes at 0.0.5.com and in the 0 to Finals Pediatrics book.

And you can find flashcards and questions at members.0.0 tofinals.com.

So let's jump straight in. Complement disorders affect the complement

proteins that make up the complement system. The complement system is part of the immune system,

and it helps to clear pathogens through opsonization, inflammation and direct cell lysis.

Compliment proteins are particularly important in dealing with encapsulated organisms,

such as hemophilus influenza type B, streptococcus pneumonia and nyseria meningitis.

Let's talk about complement deficiencies.

Key complement deficiencies to be aware of include C2 or complement two deficiency,

which is the most common and is associated with respiratory tract, ear and throat

infections, C5 to C9 deficiency, which is particularly associated with recurrent

nyseria infections, for example meningoccal disease, and C4 deficiency, which is particularly associated with

systemic lupus erythematosis. Complement deficiencies, particularly C4 deficiency, are also associated

with immune complex disorders, such as systemic lupus erythematosis. An incomplete complement cascade

results in the formation of immune complexes that are deposited in tissues causing chronic

inflammation. Vaccination against encapsulated organisms is important in patients with complement deficiencies.

In some cases, antibiotic prophylaxis is used.

Let's talk about hereditary angiodema.

Hereditary angiodema, which is also called C1-estorasehibitor Deficiency, involves deficiency or abnormal

functioning of C1 esterase inhibitor. Deficiency leads to excessive bradychinin.

Bradikinin is released during the inflammatory response. It's responsible for promoting blood vessel dilatation

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