title: Immune-mediated protein-losing enteropathy with Down syndrome.
abstract: A 28-year-old woman previously diagnosed to have Down syndrome presented with a one-month history of severe hypoalbuminemia, lower extremity edema, and diarrhea. Her urine was negative for protein. She was diagnosed with immune-mediated protein-losing enteropathy (PLE) based on clinical findings, protein loss evident on (99m)Technetium-labeled human serum albumin scintigraphy, and IgM and complement C3 deposition in the duodenum. She did not exhibit any manifestations of collagen diseases. A dramatic remission was achieved and maintained with corticosteroid administration. This is the first report of immune-mediated PLE in a patient with Down syndrome.
authors.affiliation: Department of Gastroenterology and Hematology, Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama, Japan.
authors.forename: Sohachi
authors.initials: S
authors.lastname: Nanjo
authors.forename: Jun
authors.initials: J
authors.lastname: Nishikawa
authors.forename: Shigeharu
authors.initials: S
authors.lastname: Miwa
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Mihara
authors.forename: Haruka
authors.initials: H
authors.lastname: Fujinami
authors.forename: Hiroki
authors.initials: H
authors.lastname: Yoshita
authors.forename: Akira
authors.initials: A
authors.lastname: Ueda
authors.forename: Shinya
authors.initials: S
authors.lastname: Kajiura
authors.forename: Yushi
authors.initials: Y
authors.lastname: Hasumoto
authors.forename: Takayuki
authors.initials: T
authors.lastname: Ando
authors.forename: Ayumu
authors.initials: A
authors.lastname: Hosokawa
authors.forename: Toshiro
authors.initials: T
authors.lastname: Sugiyama
mesh_terms: D000328:Adult; D003967:Diarrhea / Q000209:etiology; D004314:Down Syndrome / Q000150:complications*; D004487:Edema / Q000209:etiology; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D034141:Hypoalbuminemia / Q000209:etiology; D011239:Prednisolone / Q000627:therapeutic use; D011504:Protein-Losing Enteropathies / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology*
chemical_list: D005938:Glucocorticoids; D011239:Prednisolone