Case Study Brittany Brueggeman: Date: 11/9/2010

Patient: Age: 18 Gender: Male Height: 6’4” Weight: 200 lbs Vital Signs: HR: 62bpm Respiratory rate: 17 rpm Blood Pressure: 116/72 mm/hg Temperature: 98.6 degrees F Chief complaint Rapid tooth decay Medical Alert Vasodepressor syncope is common. Keep stress level low during visit Name of Syndrome: Klinefelter Syndrome/XXY Syndrome Cause of Syndrome - Medical History (if applicable) Occurs when an ovum carrying two X chromosomes are fertilized by a spermatozoon with a Y chromosome. Majority of cases result from nondisjunction of the X chromosome of older women. Also commonly known as the XXY condition.

Orofacial Clinical Features -Maxilla becomes increasingly hypoplasic -Patient is extremely susceptible to caries -Protruding lips and cone-shaped teeth -Hydontia Age/race/sex predilections and Systemic Clinical Features: -Occurs at birth -No race predilections -Mainly in males -1 of every 500 males has this condition but may not show symptoms Symptoms -slow learning development -trouble with speech -wide hips -development of female-like breasts -female pubic hair distribution -tall & thin -weak bones -low energy -quiet and shy personality -low testosterone levels -small and firm testes -usually infertile Radiographic Features of this Syndrome Hypoplasic: May cause lack of cementum, lack of alveolar bone, early shedding of primary and permanent teeth, large pulp chambers, cracked dentin, periapical and gingival abscesses, hypodontia, cone-shaped teeth, and taurodontism.

Special considerations in Treatment of this patient? How is it Diagnosed? How common is it? Is it a horse or zebra? Special considerations: Patient is timid, sensitive, and nervous. Vasodepressor syncope is common, so keep patient calm and use nitrous oxide if needed. Diagnosis: Testicular biopsies, hormonal evaluations, 47,XXY is present 90% of time, and buccal smears show one Barr body for each extra X chromosome. Approximately 1 in 500 males are born with an extra chromosome; however, it usually isn’t enough to cause symptoms. The more X chromosomes acquired, the more severe of the syndrome. Most prevalent in those with mental retardation. Zebra: Not a variant of normal when symptoms are present. It can cause many oral conditions that become pathological.

Klinefelter’s Syndrome

Intraoral Pictures

List Sources and References (This should be more than your text book!) Isben, Olga and Joan Phelan. Oral Pathology for the Dental Hygienist. 5 th edition. St. Louis: Saunders, Chen, Harold. “Klinefelter Syndrome.” Emedicine. Nov. 2, “Klinefelter Syndrome.” Eunice Kennedy Shriver National Institute of Child Health and Human Development. Nov. 6, Images: Aurorahealthcare.org Mdconsult.com Imaging.consult.com