World Journal of Pediatrics

, Volume 7, Issue 1, pp 79–82 | Cite as

Clinical outcome of nutrition-oriented intervention for primary intestinal lymphangiectasia

  • Qing-Ya Tang
  • Jie Wen
  • Jiang Wu
  • Ying Wang
  • Wei Cai
Brief Report



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.


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.


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.


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.

Key words

nutrition assessment nutrition intervention primary intestinal lymphangiectasia 


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  1. 1.
    Vignes S, Bellanger J. Primary intestinal lymphangiectasia (Waldmann’s disease). Orphanet J Rare Dis 2008;3:5.CrossRefPubMedGoogle Scholar
  2. 2.
    Lee J, Kong MS. Primary intestinal lymphangiectasia diagnosed by endoscopy following the intake of a high-fat meal. Eur J Pediatr 2008;167:237–239.CrossRefPubMedGoogle Scholar
  3. 3.
    Moller A, Kalhoff H, Reuter T, Friedrichs N, Wagner N. Congenital intestinal lymphangiectasia: a rare differential diagnosis in hypoproteinemia in infants. Klin Padiatr 2006;218:224–225.CrossRefPubMedGoogle Scholar
  4. 4.
    Diaz N, Khoury A, Taboada M, Isava I, Calderon A, Lopez C, et al. Primary intestinal lymphangiectasia in children: report of 3 cases. G.E.N 1995;49:307–309.PubMedGoogle Scholar
  5. 5.
    Kuroiwa G, Takayama T, Sato Y, Takahashi Y, Fujita T, Nobuoka A, et al. Primary intestinal lymphangiectasia successfully treated with octreotide. J Gastroenterol 2001;36:129–132.CrossRefPubMedGoogle Scholar
  6. 6.
    Takahashi H, Imai K. What are the objectives of treatment for intestinal lymphangiectasia. J Gastroenterol 2001;36:137–138.CrossRefPubMedGoogle Scholar
  7. 7.
    Martin CC, Garcia AF, Restrepo JM, Perez AS. Successful dietetic-therapy in primary intestinal lymphangiectasia and recurrent chylous ascites: a case report. Nutr Hosp 2007;22:723–725.PubMedGoogle Scholar
  8. 8.
    Andersen UM, Lund HT. Primary intestinal lymphangiectasis. Ugeskr Laeger 1996;158:1840–1841.PubMedGoogle Scholar
  9. 9.
    Koo NH, Lee HJ, Jung JW, Hwan Kim S, Lee KM, Hwang JS. Intestinal lymphangiectasia: a response to medium-chain triglyceride formula. Acta Paediatr 2005;94:982–983.CrossRefPubMedGoogle Scholar
  10. 10.
    Greco F, Piccolo G, Sorge A, Pavone P, Triglia T, Spina M, et al. Early-onset of primary intestinal lymphangiectasia: a case report and diet treatment. Minerva Pediatr 2003;55:615–619.PubMedGoogle Scholar
  11. 11.
    Aoyagi K, Iida M, Matsumoto T, Sakisaka S. Enteral nutrition as a primary therapy for intestinal lymphangiectasia: value of elemental diet and polymeric diet compared with total parenteral nutrition. Dig Dis Sci 2005;50:1467–1470.CrossRefPubMedGoogle Scholar
  12. 12.
    Desai AP, Guvenc BH, Carachi R. Evidence for medium chain triglycerides in the treatment of primary intestinal lymphangiectasia. Eur J Pediatr Surg 2009;19:241–245.CrossRefPubMedGoogle Scholar
  13. 13.
    Kuroiwa G, Takayama T, Sato Y, Takahashi Y, Fujita T, Nobuoka A, et al. Primary intestinal lymphangiectasia successfully treated with octreotide. J Gastroenterol 2001;36:129–132.CrossRefPubMedGoogle Scholar
  14. 14.
    Filik L, Oguz P, Koksal A, Koklu S, Sahin B. A case with intestinal lymphangiectasia successfully treated with slow-release octreotide. Dig Liver Dis 2004;36:687–690.CrossRefPubMedGoogle Scholar
  15. 15.
    Lynn J, Knight AK, Kamoun M, Levinson AI. A 55-year-old man with hypogammaglobulinemia, lymphopenia, and unrelenting cutaneous warts. J Allergy Clin Immunol 2004;114:409–414.CrossRefPubMedGoogle Scholar

Copyright information

© Children's Hospital, Zhejiang University School of Medicine and Springer-Verlag Berlin Heidelberg 2011

Authors and Affiliations

  • Qing-Ya Tang
    • 1
  • Jie Wen
    • 1
  • Jiang Wu
    • 1
  • Ying Wang
    • 1
  • Wei Cai
    • 2
    • 3
  1. 1.Clinical Nutrition CenterShanghai Jiao Tong University, School of Medicine, Xin Hua HospitalShanghaiChina
  2. 2.Department of Pediatric SurgeryShanghai Jiao Tong University, School of Medicine, Xin Hua Hospital, Shanghai Institute of Pediatric ResearchShanghaiChina
  3. 3.Department of Pediatric Surgery, Xin Hua Hospital, School of MedicineShanghai Jiao Tong UniversityShanghaiChina

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