Primary intestinal lymphangiectasia (PIL), also known as Waldmann’s disease, is an exudative enteropathy resulting from morphologic abnormalities in the intestinal lymphatics. In this article, we describe a 12-year-old boy with PIL that led to protein-losing enteropathy characterized by diarrhea, hypoalbuminemia associated with edema (serum albumin level: 1.0 g/dL), and hypogammaglobulinemia (serum IgG level: 144 mg/dL). Severe hypoalbuminemia, electrolyte abnormalities, and tetany persisted despite a low-fat diet and propranolol. Everolimus (1.6 mg/m2/day) was added to his treatment as an antiangiogenic agent. With everolimus treatment, the patient’s diarrhea resolved and replacement therapy for hypoproteinemia was less frequent. Hematologic and scintigraphy findings also improved (serum albumin level: 2.5 g/dL). There were no adverse reactions during the 12-month follow-up. To the best of our knowledge, this is the first report of everolimus use in a patient with PIL.
Everolimus for Primary Intestinal Lymphangiectasia With Protein-Losing Enteropathy
POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of interest to disclose.
FINANCIAL DISCLOSURE: The authors have indicated they have no financial relationships relevant to this article to disclose.
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Michio Ozeki, Tomohiro Hori, Kaori Kanda, Norio Kawamoto, Takashi Ibuka, Tatsuhiko Miyazaki, Toshiyuki Fukao; Everolimus for Primary Intestinal Lymphangiectasia With Protein-Losing Enteropathy. Pediatrics March 2016; 137 (3): e20152562. 10.1542/peds.2015-2562
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