ICD-11 MMS

04 Diseases of the immune system

Primary immunodeficiencies  (4A00-4A0Z)

4A01 Primary immunodeficiencies due to disorders of adaptive immunity

4A01.3 Other well-defined immunodeficiency syndromes due to defects in adaptive immunity

4A01.32 Immuno-osseous dysplasia

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01


This is an autosomal recessive disorder with the diagnostic features of spondyloepiphyseal dysplasia, renal dysfunction, and T-cell immunodeficiency.

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