The oral manifestations of intestinal lymphangiectasia: case report.
Intestinal lymphangiectasia is a rare autosomal dominant disorder or acquired condition that leads to lymph obstruction, poor chyle transport, and concomitant problems of hypoproteinemia, lymphocytopenia, hypogammaglobulinemia, and peripheral edema. Patients develop diarrhea, steatorrhea, and hypocalcemia secondary to fat-soluble vitamin malabsorption. Treatment is a restrictive diet of low fat, medium chain triglycerides. Oral manifestations are gingivitis due to poor PMN function and enamel defects due to poor calcium absorption. A case of a 14-year-old boy with both gingival and enamel problems secondary to intestinal lymphangiectasia is reported.
Ralph
P M
PM
Bronx Lebanon Hospital, New York, USA.
Troutman
K C
KC
D000293:Adolescent; D000361:Agammaglobulinemia / Q000473:pathology; D001798:Blood Proteins / Q000378:metabolism; D002446:Celiac Disease / Q000473:pathology; D002913:Chyle / Q000378:metabolism; D003744:Dental Enamel Hypoplasia / Q000473:pathology; D003967:Diarrhea / Q000473:pathology; D018752:Diet, Fat-Restricted; D004041:Dietary Fats / Q000008:administration & dosage; D004487:Edema / Q000473:pathology; D005891:Gingivitis / Q000473:pathology*; D006801:Humans; D006996:Hypocalcemia / Q000473:pathology; D008196:Lymph / Q000378:metabolism; D008201:Lymphangiectasis, Intestinal / Q000178:diet therapy / Q000473:pathology* / Q000503:physiopathology; D008231:Lymphopenia / Q000473:pathology; D008297:Male; D014075:Tooth Discoloration / Q000473:pathology; D014076:Tooth Diseases / Q000473:pathology*; D014280:Triglycerides / Q000008:administration & dosage
D001798:Blood Proteins; D004041:Dietary Fats; D014280:Triglycerides