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Spec Care Dentist. 2015 Mar-Apr;35(2):83-9. doi: 10.1111/scd.12080. Epub 2014 Jul 18.

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

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TAKO'Centre, National Resource Centre for Oral Health in Rare Medical Conditions, Lovisenberg Diakonale Hospital, Oslo, Norway; Department of Pediatrics and Department of Clinical and Experimental Medicine, Linköping University, Centre for Orthodontics and Pediatric dentistry, Norrköping, Sweden.


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%).


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


dental caries; government subsidy; lymphedema cholestasis syndrome; periodontitis; tooth discoloration

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