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Криптогенний мультифокальний виразковий стенозуючий ентерит

ORPHA:468635· ICD-10 K63.3· Cryptogenic multifocal ulcerous stenosing enteritis

Визначення(English summary)

A rare intestinal disease characterized by chronic or relapsing subileus or ileus resulting from multiple unexplained fibrous structures and multiple shallow (i. e. limited to the mucosa or submucosa) ulcerations of the small intestine (mainly the ileum), in the absence of signs of a systemic inflammatory reaction. Patients may present with chronic iron-deficiency anemia due to chronic intestinal blood loss, chronic recurrent abdominal pain, fatigue, edema, or growth retardation. Extraintestinal manifestations such as Sicca syndrome, polyarthralgia, or Raynaud's phenomenon may also be observed.

Поширеність
<1 / 1 000 000
Успадкування
Autosomal recessive
Вік початку
Adolescent, Adult, Childhood