Immune-mediated Protein-losing Enteropathy with Down Syndrome

Sohachi Nanjo; Jun Nishikawa; Shigeharu Miwa; Hiroshi Mihara; Haruka Fujinami; Hiroki Yoshita; Akira Ueda; Shinya Kajiura; Yushi Hasumoto; Takayuki Ando; Ayumu Hosokawa; Toshiro Sugiyama

doi:10.2169/internalmedicine.53.1980

CASE REPORTS

Immune-mediated Protein-losing Enteropathy with Down Syndrome

Sohachi Nanjo, Jun Nishikawa, Shigeharu Miwa, Hiroshi Mihara, Haruka Fujinami, Hiroki Yoshita, Akira Ueda, Shinya Kajiura, Yushi Hasumoto, Takayuki Ando, Ayumu Hosokawa, Toshiro Sugiyama

Author information

Sohachi Nanjo
Department of Gastroenterology and Hematology, Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama, Japan
Jun Nishikawa
Department of Gastroenterology and Hematology, Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama, Japan
Shigeharu Miwa
Department of Diagnostic Pathology, Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama, Japan
Hiroshi Mihara
Department of Gastroenterology and Hematology, Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama, Japan
Haruka Fujinami
Department of Gastroenterology and Hematology, Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama, Japan
Hiroki Yoshita
Department of Gastroenterology and Hematology, Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama, Japan
Akira Ueda
Department of Gastroenterology and Hematology, Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama, Japan
Shinya Kajiura
Department of Gastroenterology and Hematology, Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama, Japan
Yushi Hasumoto
Department of Gastroenterology and Hematology, Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama, Japan
Takayuki Ando
Department of Gastroenterology and Hematology, Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama, Japan
Ayumu Hosokawa
Department of Gastroenterology and Hematology, Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama, Japan
Toshiro Sugiyama
Department of Gastroenterology and Hematology, Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama, Japan

Keywords: protein-losing enteropathy, immune alteration, Down syndrome

JOURNAL OPEN ACCESS

2014 Volume 53 Issue 20 Pages 2301-2305

DOI https://doi.org/10.2169/internalmedicine.53.1980

Details

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 ^99mTechnetium-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.

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