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Clinical outcome of nutrition-oriented intervention for primary intestinal lymphangiectasia

World Journal of Pediatrics volume 7, pages 79–82 (2011)Cite this article

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.

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References

  1. Vignes S, Bellanger J. Primary intestinal lymphangiectasia (Waldmann’s disease). Orphanet J Rare Dis 2008;3:5.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    CAS  Article  PubMed  Google Scholar 

  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.

    CAS  PubMed  Google Scholar 

  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.

    CAS  Article  PubMed  Google Scholar 

  6. Takahashi H, Imai K. What are the objectives of treatment for intestinal lymphangiectasia. J Gastroenterol 2001;36:137–138.

    CAS  Article  PubMed  Google Scholar 

  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.

    PubMed  Google Scholar 

  8. Andersen UM, Lund HT. Primary intestinal lymphangiectasis. Ugeskr Laeger 1996;158:1840–1841.

    CAS  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    CAS  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    CAS  Article  PubMed  Google Scholar 

  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.

    CAS  Article  PubMed  Google Scholar 

  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.

    CAS  Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

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Affiliations

  1. Clinical Nutrition Center, Shanghai Jiao Tong University, School of Medicine, Xin Hua Hospital, Shanghai, China

    Qing-Ya Tang, Jie Wen, Jiang Wu & Ying Wang

  2. Department of Pediatric Surgery, Shanghai Jiao Tong University, School of Medicine, Xin Hua Hospital, Shanghai Institute of Pediatric Research, Shanghai, China

    Wei Cai

  3. Department of Pediatric Surgery, Xin Hua Hospital, School of Medicine, Shanghai Jiao Tong University, Kong-Jiang Road, Yang-Pu District, Shanghai, 200092, China

    Wei Cai

Authors
  1. Qing-Ya Tang
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  2. Jie Wen
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  3. Jiang Wu
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  4. Ying Wang
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  5. Wei Cai
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Correspondence to Wei Cai.

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Tang, QY., Wen, J., Wu, J. et al. Clinical outcome of nutrition-oriented intervention for primary intestinal lymphangiectasia. World J Pediatr 7, 79–82 (2011). https://doi.org/10.1007/s12519-011-0248-x

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  • DOI: https://doi.org/10.1007/s12519-011-0248-x

Key words

  • nutrition assessment
  • nutrition intervention
  • primary intestinal lymphangiectasia