Primary immunodeficiency with natural-killer cell deficiency and adrenal insufficiency

Orpha code: 75391OMIM code: 609981

Definition

A rare DNA repair defect other than combined T-cell and B-cell immunodeficiencies characterized by intrauterine and postnatal growth retardation resulting in short stature, microcephaly, glucocorticoid deficiency, natural killer cell deficiency, and recurrent viral infections. Patients may also have increased susceptibility to cancer.

Disease data
Classification

Disease

Synonyms
Primary immunodeficiency due to MCM4 deficiency
Pierwotny Niedobór odporności spowodowany deficytem MCM4
ORPHA code
75391
OMIM code
609981
ICD10 code
D84.8
ICD11 code
-

No additional description.

Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl