Dental care and oral health in Aagenaes syndrome/lymphedema cholestasis syndrome 1.

Abstract

UNLABELLED:

Aagenaes syndrome/lymphedema cholestasis syndrome 1 (LCS1) is a rare genetic disorder characterized by neonatal cholestasis and lymphedema. The aim was to assess dental care and oral health in adults with LCS1. Fifteen (9M, 6F) individuals diagnosed with LCS1, aged 19-59 years participated. The study evaluated salivary secretion rate, dental radiographs, intraoral photos and included a questionnaire. Eight (53%) had regular dental checkups. Three had received subsidized dental care. Seven (47%) had two or more subjective symptoms of xerostomia. Three (20%) had a decreased stimulated salivary secretion rate below 0.7 mL/minute. Seven (47%) had dentin caries. Marginal periodontitis was found in all six patients above 35 years of age, but not before that age. Thirteen (87%) had tooth discoloration, which was extensive in three (20%).

CONCLUSION:

Several patients with LCS1 have problems with periodontitis and tooth discoloration. Frequent dental checkups are therefore recommended.

• Authors: Mats Bågesund, Zahra Shafiee, Monica Drivdal, Jan Berdén, Kari Storhaug
• Journal: Special care in dentistry : official publication of the American Association of Hospital Dentists, the Academy of Dentistry for the Handicapped, and the American Society for Geriatric Dentistry (Spec Care Dentist) 2015 Mar-Apr Vol. 35 Issue 2 Pg. 83-9 ISSN: 1754-4505 [Electronic] United States
• PMID: 25039919 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: © 2014 Special Care Dentistry Association and Wiley Periodicals, Inc.

Topics

• Adult
• Cholestasis (complications)
• Dental Care for Chronically Ill
• Female
• Humans
• Lymphedema (complications)
• Male
• Middle Aged
• Mouth Diseases (diagnosis, therapy)
• Norway
• Surveys and Questionnaires
• Treatment Outcome