Understanding Peutz-Jeghers Syndrome: A Familial Perspective

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Peutz-Jeghers Syndrome (PJS) is an autosomal-dominant inheritance condition that usually runs in families. Another name for this disorder is hereditary intestinal polyposis syndrome. PJS is a rare disorder and occurs in 1 in 160,000 to 1 in 280,000 persons. Peutz- Jeghers Syndrome is “caused by a change (mutation) in a gene that increases the risk for developing colon and other cancers.
 There are two common types of Peutz-Jeghers Syndrome. There is familial PJS and sporadic PJS. Familial PJS is the type that is due to a genetic mutation in a gene called STK11. The hereditary defect is passed down from one parent or both as a dominant trait. If a person has a parent that has this syndrome, the child has a 50% chance of inheriting the mutation …show more content…

For a patient with Peutz-Jeghers syndrome but does not have cancer, the dental hygienist should reiterate to the patient the important of flossing daily to mechanically remove the biofilm accumulating in their mouth. Another oral hygiene instruction would be to show the patient the proper way of brushing. Two other helpful methods to show the patient would be the modified bass to show the patient that the focus of the brushing is to point the brush filaments toward the gingival margin, also to discuss with the patient the importance of preventing dental caries by decreasing their intake of foods that will produce acid exposure to the teeth. For a patient with Peutz-Jeghers syndrome that does have patient, the dental hygienist should mostly focus on how to help the patient with preventative measures for xerostomia. Some beneficial aids for xerostomia would be biotin gels and mouthwashes, also nightly fluoride treatment’s to prevent caries because the patient is more susceptible due to the dryness of their oral cavity. Also with a patient with cancer, you want to stray away from any excess radiation that the patient doesn’t need because it could be potentially harmful to

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