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Fast Facts: Perio Edition – Periodontitis as a Manifestation of Systemic Disease – Chediak-Higashi Syndrome

Fast Facts: Perio Edition Episode 62

[Andrew Johnston, RDH]
Welcome back everyone! You are listening to another episode of Fast Facts- Perio Edition brought to you by A Tale of Two Hygienists in partnership with DentistRX. And now, please welcome your host, Katrina Sanders.
[Katrina Sanders, RDH]
Hello, and welcome to Fast Facts: Perio Edition. We’re looking at the manifestation of systemic diseases that relate specifically to periodontitis. Last week we talked about Papillon-Lefèvre syndrome, and we are continuing the dialogue this week, looking at Chediak-Higashi syndrome. So remember, these are all syndromes in which if the patient has this syndrome, there’s some type of a chromosomal or chemotactic challenge. Something is going on in which the patient will likely experience periodontal disease. Chediak-Higashi syndrome is another one of those relatively obscure or rare diseases. However, it is inherited and it is considered an immune disorder.
Interestingly enough, here’s how this immune disorder works. So there are traffic patterns. If we think about the body and we think about where our immune systems move or in the case of Chediak-Higashi, when we look at how pigmentation moves to the correct cells in the body, I want you to think about all of this like a super highway system where things are constantly moving around. The challenge is in an individual who has this relatively obscure congenital or inherited defect, these individuals have some type of an issue in which their abnormal gene affects those traffic patterns or the ways in which proteins will move throughout the body and into cells.
So of course, one of those proteins is pigmentation, and this is where we can see that melanin pigmentation can be a challenge. Individuals who have Chediak-Higashi can experience light blonde, light Brown, or even a silvery tint to their hair, and even from a pigmentation of the skin, they can oftentimes be relatively sensitive or have a photo sensitivity to light. They can even have reduced pigmentation in the eyes, so they may be more sensitive and need to wear sunglasses. These individuals can have an accelerated sensitivity associated with the ways in which these proteins move.
The same is true, however, of specifically how our immune components move throughout the body. So an individual who has Chediak-Higashi also experiences challenges with regards to immune components. These individuals will experience issues in which they may bruise easily or experience excessive bleeding. Their platelet numbers are typically normal, but the platelets themselves are not functioning properly. Again, one of the issues associated with movement of proteins throughout the body. They can also develop because of their declined immune system, they can begin to develop things like fever, swollen lymph nodes, and enlargement of the liver, the spleen, anemias, and they can even in some cases, present with low white blood cell counts.
So these individuals who present with Chediak-Higashi will oftentimes experience the ramifications associated with having a declined immune system, which for these individuals is oftentimes experienced in advanced signs, stages, or symptoms of periodontitis. These patients are oftentimes susceptible to infection, and so even the most minimal of periodontal pathogen threat could render these individuals experiencing advanced signs, stages or symptoms of periodontitis. If you see a patient in your chair who has this relatively rare and obscure disease process, make sure that you have a strong understanding of this patient’s unique susceptibility to infections and the ways in which we need to protect these patients during their dental procedures.
Stay tuned next week as we continue the conversation about periodontitis as a manifestation of systemic disease.
This has been another episode of Fast Facts – Perio Edition with Katrina Sanders, RDH. Please feel free to reach me on Instagram @thedentalwinegenist or on my website Cheers.
[Andrew Johnston, RDH]
Thank you for listening to another episode of Fast Facts – Perio Edition, brought to you in part by DentistRX makers of the InteliSonic line of power brushes. Find out more by visiting their website at We’ll see you next week for another Fast Fact!

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