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.