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Fast Facts: Perio Edition - Periodontitis as a Manifestation of Systemic Disease - Cohen Syndrome

Fast Facts

Fast Facts: Perio Edition Episode 66
[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. I'm so excited, you guys. We are continuing the conversation around discussing different modalities affiliated with Periodontitis as a Manifestation of Systemic Disease. Last time we looked at Infantile Genetic Agranulocytosis, and we're kind of continuing to move through these processes by looking at different types of inherited disorders or conditions that will ultimately affect our patient's periodontium.
This week we're talking about Cohen Syndrome. It's important to know that Cohen Syndrome is a genetic condition. So oftentimes we will see that family members within this patient's circle will also have one, a few, several or maybe all of the characterizations of Cohen Syndrome.
So Cohen Syndrome affects many parts of the body and oftentimes is affiliated with developmental delays, so physical developmental delays and then intellectual disabilities as well. And in these patients, we will see microcephaly or a small head size and then hypotonia or a weak muscle tone. These individuals oftentimes experience challenges associated with eyesight because there's oftentimes a breakdown or a degradation of the tissue in the back of the eye. They also have unusually large joint movement or hyper mobility. So you may see these individuals similar to like Marfan syndrome, you'll see these individuals being extremely flexible in a lot of their joints. And they will have some very distinctive facial features, some things that you're going to look for on the face. So these are going to be facial features like really thick hair, very thick eyebrows, think like Eugene Levy eyebrows, long eyelashes. Their eyes are going to have an interesting shape. They're going to be a kind of wave shaped and down slanting. Their nose will have a kind of a bulbous tip onto it, and the area in between the nose and the upper lip that's that filtrum area will be a bit shortened. These individuals oftentimes will also have very prominent central incisors. Oftentimes when we see individuals who have that shortened filtrum and then prominent and central incisors, these individuals will also have a resting open mouth position. And so because of that, we can absolutely see some challenges associated with dry mouth, etcetera.
But one of the key cardinal signs of Cohen Syndrome that we really do get concerned about is that these individuals as a component of their disorder will have low white blood cell levels or neutropenia. This is where we absolutely see the development of periodontal disease. And, in fact, several research studies have actually identified that individuals who have Cohen Syndrome present with more frequent and more extensive alveolar bone loss than those individuals who have the same concentration of periodontal pathogens but do not present with Cohen Syndrome. So we've seen over time, research studies concluding that subjects that have Cohen Syndrome have an increased susceptibility to early periodontal breakdown, which we do believe is associated with their neutropenia.
We can also see obesity being a challenge. Typically this in Cohen Syndrome individuals occurs around the torso and so we do believe that the inflammatory condition of systemic obesity absolutely can elevate the risk associated with individuals experiencing periodontal destruction over time.
Thank you for joining us today. Tune in next time as we continue the conversation around 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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