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Everolimus for Primary Intestinal Lymphangiectasia With Protein-Losing Enteropathy

Michio Ozeki1, Tomohiro Hori2, Kaori Kanda2

  • 1Departments of Pediatrics, michioo@gifu-u.ac.jp.

Pediatrics
|February 25, 2016
PubMed

Insights

Primary intestinal lymphangiectasia (PIL) is a rare condition causing protein loss. Everolimus effectively treated a young patient with PIL, resolving diarrhea and improving protein levels without adverse effects.

Area of Science:

  • Gastroenterology
  • Pediatric Gastroenterology
  • Vascular Biology

Background:

  • Primary intestinal lymphangiectasia (PIL), or Waldmann's disease, is a rare exudative enteropathy caused by intestinal lymphatic abnormalities.
  • It leads to protein-losing enteropathy, presenting with diarrhea, hypoalbuminemia, edema, and hypogammaglobulinemia.

Observation:

  • A 12-year-old boy with severe PIL experienced persistent symptoms including diarrhea, hypoalbuminemia (1.0 g/dL), and hypogammaglobulinemia (144 mg/dL) despite conventional treatments.
  • Symptoms like severe hypoalbuminemia, electrolyte imbalances, and tetany did not improve with a low-fat diet and propranolol.

Findings:

  • Everolimus, an antiangiogenic agent, was administered at 1.6 mg/m(2)/day.
  • Treatment with everolimus led to resolution of diarrhea and reduced need for protein replacement therapy.
  • Significant improvements were observed in hematologic and scintigraphy findings, with serum albumin levels rising to 2.5 g/dL.

Implications:

  • This case suggests everolimus is a potential therapeutic option for primary intestinal lymphangiectasia.
  • Further research is warranted to explore the efficacy and safety of everolimus in managing PIL.
  • This represents the first reported use of everolimus in a patient diagnosed with primary intestinal lymphangiectasia.

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