Chronic Ileum Obstruction, Granulation, and Ulceration With IgG4-Positive Plasma Cell Infiltration in a Six-Year-Old Girl With 21trisomy

Patients with 21trisomy often develop congenital or acquired gastrointestinal diseases, such as duodenal or anal atresia, celiac disease, intussusception, and constipation. In these patients, it is often challenging to diagnose gastrointestinal diseases because most patients have difficulty explaini...

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Bibliographic Details
Published in:Curēus (Palo Alto, CA) CA), 2024-05, Vol.16 (5), p.e60177
Main Authors: Yamanaka, Hiroshi, Ueki, Masahiro, Kikuchi, Keisuke, Yakuwa, Satoshi
Format: Article
Language:English
Subjects:
Abdomen
Anemia
Case reports
Congenital diseases
Constipation
Feces
Gastroenterology
Gastrointestinal diseases
Hemoglobin
Hospitals
Immunology
Inflammation
Inflammatory bowel disease
Intestinal obstruction
Ischemia
Neutrophils
Patients
Pediatric Surgery
Pediatrics
Plasma
Small intestine
Surgery
Tomography
Ulcers
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Summary:Patients with 21trisomy often develop congenital or acquired gastrointestinal diseases, such as duodenal or anal atresia, celiac disease, intussusception, and constipation. In these patients, it is often challenging to diagnose gastrointestinal diseases because most patients have difficulty explaining their complaints in detail. Furthermore, these patients also possess immunological disorders, such as increased type I interferon activation, innate immune hypersensitivity, and polarization to autoimmune. Here, we present a girl with 21trisomy and constipation who developed severe anemia, occult blood and elevated levels of calprotectin in stool, and chronic ileum obstruction confirmed by computed tomography. The patient underwent surgical resection of the ileum and recovered without complications. Pathological examination demonstrated intussusception, ischemia, ulceration, inflammatory granulation, and massive IgG4-positive plasma cell infiltration. After the surgery, her fecal calprotectin levels were normalized. We assumed that the ileum inflammation caused by ileum dilation generated ulcers and granulation, which could be associated with immunological, gastrointestinal, and intellectual disorders in patients with 21trisomy.
ISSN:2168-8184
2168-8184
DOI:10.7759/cureus.60177