title: Clinical outcome of nutrition-oriented intervention for primary intestinal lymphangiectasia.
abstract: BACKGROUND
  primary intestinal lymphangiectasia (PIL) is a rare digestive disease and few studies have focused on the therapeutic effect in PIL patients. This study was undertaken to evaluate nutrition-oriented intervention in children with PIL.
  
  
  METHODS
  four children with PIL were studied. Their medical records were reviewed. Anthropometric measurements and blood tests were performed during a 8-18 month follow-up.
  
  
  RESULTS
  during hospitalization, the 4 patients were subjected to diet intervention. Parenteral nutrition (PN) support was also given to 3 of them. Clinical symptoms and laboratory parameters of the patients were significantly improved at discharge. After discharge, the patients continued diet control, 2 of whom received intermittent PN support. The mean follow-up duration of the 4 patients was 13 months (range, 8-18 months) and they all kept in a stable condition without symptoms relapse. Weight, height and body mass index for age were normal during the follow-up, while total protein, albumin and immunoglobulin concentrations were still slightly below normal level.
  
  
  CONCLUSIONS
  nutrition therapy is effective as a valid and safe therapeutic management for PIL patients. No growth retardation was observed in the 4 children after the therapy, but they are still at risk of nutrient malabsorption. Therefore, they need long-term, regular monitoring and intensive nutritional care.
authors.affiliation: Clinical Nutrition Center, Shanghai Jiao Tong University, School of Medicine, Xin Hua Hospital, Shanghai, China.
authors.forename: Qing-Ya
authors.initials: QY
authors.lastname: Tang
authors.forename: Jie
authors.initials: J
authors.lastname: Wen
authors.forename: Jiang
authors.initials: J
authors.lastname: Wu
authors.forename: Ying
authors.initials: Y
authors.lastname: Wang
authors.forename: Wei
authors.initials: W
authors.lastname: Cai
mesh_terms: D000886:Anthropometry; D001827:Body Height; D015992:Body Mass Index; D001835:Body Weight; D002648:Child; D002675:Child, Preschool; D004750:Enteral Nutrition / Q000379:methods; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008201:Lymphangiectasis, Intestinal / Q000175:diagnosis / Q000178:diet therapy* / Q000628:therapy; D008297:Male; D016347:Medical Records Systems, Computerized; D010288:Parenteral Nutrition / Q000379:methods; D012016:Reference Values; D016896:Treatment Outcome