The n e w e ng l a n d j o u r na l
Images in Clinical Medicine
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Chisato Akita, M.D.
Yutaka Saikawa, M.D., Ph.D.
Kanazawa Medical University
Ishikawa, Japan
saikawa@kanazawa-med.ac.jp
774
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Copyright 2017 Massachusetts Medical Society. All rights reserved.
of m e dic i n e
Chana A. Sacks, M.D., Editor
Gastric Gyri Pediatric Mntriers Disease
C
A
previously healthy 5-year-old boy presented with a 4-day his-
tory of nausea, vomiting, and intermittent abdominal pain. Physical findings
were unremarkable except for mild periorbital edema. Laboratory studies
revealed hypoproteinemia with an albumin level of 2.0 g per deciliter (normal range
in Japanese children >1 year of age, 4.0 to 5.0), in the absence of proteinuria and
liver dysfunction. Computed tomography with contrast material (Panel A shows the
coronal view, and Panel B the sagittal view) and gastrointestinal endoscopy (Panel C)
revealed giant cerebriform enlargement of rugal folds in the fundus and body. Biopsy
findings indicated foveolar hyperplasia with glandular atrophy. Testing for Helico-
bacter pylori and viral inclusion bodies was negative. Scintigraphy with technetium-
99mlabeled human serum albumin revealed gastrointestinal protein loss. The
presence of large mucosal folds within the stomach and evidence of gastrointesti-
nal protein loss suggested a diagnosis of Mntriers disease. This rare form of ac-
quired protein-losing gastropathy is characterized by giant gastric rugal folds and
epithelial hyperplasia and is associated with hypoalbuminemia and edema. The pedi-
atric form of Mntriers disease symptomatically resembles the adult-onset type, but
it may have a benign, self-limited course with spontaneous resolution. The patient
was treated with supportive care, and his symptoms resolved 4 months after the
initial presentation.
DOI: 10.1056/NEJMicm1605473
Copyright 2017 Massachusetts Medical Society.
n engl j med 376;8 nejm.org February 23, 2017
The New England Journal of Medicine