title: Everolimus for Primary Intestinal Lymphangiectasia With Protein-Losing Enteropathy.
abstract: 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/m(2)/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.
authors.affiliation: Departments of Pediatrics, michioo@gifu-u.ac.jp.
authors.forename: Michio
authors.initials: M
authors.lastname: Ozeki
authors.affiliation: Departments of Pediatrics.
authors.forename: Tomohiro
authors.initials: T
authors.lastname: Hori
authors.affiliation: Departments of Pediatrics.
authors.forename: Kaori
authors.initials: K
authors.lastname: Kanda
authors.affiliation: Departments of Pediatrics.
authors.forename: Norio
authors.initials: N
authors.lastname: Kawamoto
authors.affiliation: Gastroenterology, and.
authors.forename: Takashi
authors.initials: T
authors.lastname: Ibuka
authors.affiliation: Pathology, Gifu University Graduate School of Medicine, Gifu University, Gifu, Japan.
authors.forename: Tatsuhiko
authors.initials: T
authors.lastname: Miyazaki
authors.affiliation: Departments of Pediatrics.
authors.forename: Toshiyuki
authors.initials: T
authors.lastname: Fukao
mesh_terms: D002648:Child; D018752:Diet, Fat-Restricted; D004305:Dose-Response Relationship, Drug; D000068338:Everolimus / Q000008:administration & dosage*; D005500:Follow-Up Studies; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D008201:Lymphangiectasis, Intestinal / Q000150:complications* / Q000178:diet therapy / Q000188:drug therapy; D008209:Lymphedema / Q000150:complications* / Q000178:diet therapy / Q000188:drug therapy; D008297:Male; D011504:Protein-Losing Enteropathies / Q000188:drug therapy* / Q000209:etiology
chemical_list: D007166:Immunosuppressive Agents; D000068338:Everolimus