Primary intestinal lymphangiectasia: four case reports and a review of the literature.
BACKGROUND
Primary intestinal lymphangiectasia (PIL) is a rare digestive disease and most articles on this condition are isolated case reports.


OBJECTIVE
Our purpose is to investigate the clinical characteristics, therapeutic management, and outcome of PIL through case studies.


METHODS
We conducted a retrospective analysis and obtained detailed clinical information for four PIL patients treated at our institution. A MEDLINE database search was also performed using the search term "intestinal lymphangiectasia" and all pertinent literature was carefully reviewed.


RESULTS
Four children treated in our department showed elevated IgE and a good response to diet intervention. After reviewing the literature, we conducted statistical analysis on the basis of all the cases, with a total of 84 cases. Thirty-eight cases have been reported with diet treatment, 24 (63%) of whom showed apparent improvement in clinical symptoms and laboratory parameters. Four cases (5%) had a malignant transformation of lymphoma, and the average time from PIL onset to lymphoma diagnosis was 31 years (range, 19-45 years). No difference was observed regarding the presence of major clinical manifestations among children and adults. Diet intervention in children was more effective than that in adults.


CONCLUSIONS
Diet intervention is the cornerstone of PIL medical management, which was found to be more effective in children than in adults. Early diagnosis and treatment of IL is of great importance for effective diet therapy. An elevated IgE level should be monitored periodically since it could be an indication of malignant transformation-lymphoma.
Wen
Jie
J
Clinical Nutrition Center, School of Medicine, Xin Hua Hospital, Shanghai Jiao Tong University, Shanghai, China.
Tang
Qingya
Q
Wu
Jiang
J
Wang
Ying
Y
Cai
Wei
W
D053704:Capsule Endoscopy; D002648:Child; D002675:Child, Preschool; D004108:Dilatation, Pathologic; D005260:Female; D006801:Humans; D007073:Immunoglobulin E / Q000097:blood; D008201:Lymphangiectasis, Intestinal / Q000097:blood / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy*; D042601:Lymphatic Vessels / Q000473:pathology; D008297:Male; D012189:Retrospective Studies
D007073:Immunoglobulin E