Immune dysregulation-inflammatory bowel disease-arthritis-recurrent infections syndrome

Orpha code: 238569OMIM code: 613148

Definition

A rare immune dysregulation disease with immunodeficiency characterized by severe, progressive infantile onset inflammatory bowel disease with pancolitis, perianal disease (ulceration, fistulae), recurrent respiratory, genitourinary and cutaneous infections, arthritis and a high risk of B-cell lymphoma.

Disease data
Classification

Disease

Synonyms
IL10-related early-onset IBD
Nieswoiste zapalenie jelit o wczesnym początku zależne od IL10
IL10-related early-onset inflammatory bowel disease
ORPHA code
238569
OMIM code
613148
ICD10 code
K52.8
ICD11 code
-

No additional description.

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