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Milky Way: Management of Primary Intestinal Lymphangiectasia

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References

  1. Waldmann TA, Steinfeld JL, Dutcher TF, Davidson JD, Gordon RS. The role of the gastrointestinal system in “idiopathic hypoproteinemia.” Gastroenterology 1961;41:197–207.

    Article  CAS  PubMed  Google Scholar 

  2. Huber R, Semmler G, Mayr A, Offner F, Datz C. Primary intestinal lymphangiectasia in an adult patient: a case report and review of literature. World J Gastroenterol. 2020;26:7707–7718. https://doi.org/10.3748/wjg.v26.i48.7707.

    Article  PubMed  PubMed Central  Google Scholar 

  3. Vignes S, Bellanger J. Primary intestinal lymphangiectasia (Waldmann’s disease). Orphanet J Rare Dis. 2008;3:5. https://doi.org/10.1186/1750-1172-3-5.

    Article  PubMed  PubMed Central  Google Scholar 

  4. Freeman HJ, Nimmo M. Intestinal lymphangiectasia in adults. World J Gastrointest Oncol. 2011;3:19–23. https://doi.org/10.4251/wjgo.v3.i2.19.

    Article  PubMed  PubMed Central  Google Scholar 

  5. Kwon Y, Kim ES, Choe YH, Hyun D, Kim MJ. Therapeutic lymphatic embolization in pediatric primary intestinal lymphangiectasia. Yonsei Med J. 2021;62:470–473. https://doi.org/10.3349/ymj.2021.62.5.470.

    Article  PubMed  PubMed Central  Google Scholar 

  6. Chamouard P, Nehme-Schuster H, Simler J-M. Videocapsule endoscopy is useful for the diagnosis of intestinal lymphangiectasia. Digest Liver Dis. 2006;38:699–703.

    Article  CAS  Google Scholar 

  7. Cammarata SK, Brush RE, Hyzy RC. Chylothorax after childbirth. Chest. 1991;99:1539–1540. https://doi.org/10.1378/chest.99.6.1539.

    Article  CAS  PubMed  Google Scholar 

  8. Jeffries GH, Chapman A, Sleisenger MH. Low-Fat Diet in Intestinal Lymphangiectasia. N Engl J Med. 1964;270:761–766. https://doi.org/10.1056/NEJM196404092701503.

    Article  CAS  PubMed  Google Scholar 

  9. Nancy You YQ, Ling PR, Qu JZ, Bistrian BR. Effects of medium-chain triglycerides, long-chain triglycerides, or 2-monododecanoin on fatty acid composition in the portal vein, intestinal lymph, and systemic circulation in rats. JPEN J Parenter Enteral Nutr. 2008;32:169–175. https://doi.org/10.1177/0148607108314758.

    Article  PubMed Central  Google Scholar 

  10. Shah ND, Limketkai BN. The use of medium-chain triglycerides in gastrointestinal disorders. Pract Gastroenterol. 2017;160:20–8.

    Google Scholar 

  11. Kwon Y, Kim MJ. The update of treatment for primary intestinal lymphangiectasia. Pediatr Gastroenterol Hepatol Nutr. 2021;24:413–422. https://doi.org/10.5223/pghn.2021.24.5.413.

    Article  PubMed  PubMed Central  Google Scholar 

  12. Makrilakis K, Pavlatos S, Giannikopoulos G, Toubanakis C, Katsilambros N. Successful octreotide treatment of chylous pleural effusion and lymphedema in the yellow nail syndrome. Ann Intern Med. 2004;141:246–247. https://doi.org/10.7326/0003-4819-141-3-200408030-00028.

    Article  PubMed  Google Scholar 

  13. Adair D, Rabah R, Ladino-Torres M, Saba TG. Case Report: A relatively mild presentation of unilateral congenital pulmonary lymphangiectasia. Front Pediatr. 2021. https://doi.org/10.3389/fped.2021.657473.

    Article  PubMed  PubMed Central  Google Scholar 

  14. Bellini C, Boccardo F, Campisi C, Bonioli E. Congenital pulmonary lymphangiectasia. Orphanet J Rare Dis. 2006;1:43. https://doi.org/10.1186/1750-1172-1-43.

    Article  PubMed  PubMed Central  Google Scholar 

  15. Adams DM, Trenor CC, Hammill AM et al. Efficacy and safety of sirolimus in the treatment of complicated vascular anomalies. Pediatrics. 2016;137:e20153257. https://doi.org/10.1542/peds.2015-3257.

    Article  PubMed  PubMed Central  Google Scholar 

  16. Kwon Y, Kim ES, Choe YH, Kim MJ. Individual approach for treatment of primary intestinal lymphangiectasia in children: single-center experience and review of the literature. BMC Pediatr. 2021;21:21. https://doi.org/10.1186/s12887-020-02447-5.

    Article  PubMed  PubMed Central  Google Scholar 

  17. Lee SY, Yang SR, Lee KR. Congenital pulmonary lymphangiectasia with chylothorax. Asian Cardiovasc Thorac Ann. 2002;10:76–77. https://doi.org/10.1177/021849230201000122.

    Article  PubMed  Google Scholar 

  18. Ruggeri G, Destro F, Maffi M, Gregori G, Lima M. A rare case of chylothorax due to pulmonary lymphangiectasia in a 7-year-old boy. Eur J Pediatr Surg Rep. 2013;1:18–20. https://doi.org/10.1055/s-0033-1337111.

    Article  Google Scholar 

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Authors and Affiliations

  1. Department of Medicine, Stanford University, Stanford, CA, 94304, USA

    Joshua S. Norman, Stefano Testa & Thomas Savage

  2. Department of Pathology, Stanford University, Stanford, CA, 94304, USA

    Cindy Xinnan Wang

  3. Stanford, USA

    Joshua S. Norman

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  1. Joshua S. Norman
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  2. Stefano Testa
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Correspondence to Joshua S. Norman.

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Norman, J.S., Testa, S., Wang, C.X. et al. Milky Way: Management of Primary Intestinal Lymphangiectasia. Dig Dis Sci 68, 3872–3877 (2023). https://doi.org/10.1007/s10620-023-08077-y

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