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Primary immunodeficiency with natural-killer cell deficiency and adrenal insufficiency

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Disease 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.

ORPHA:75391

Classification level: Disorder

Synonym(s):
  • Primary immunodeficiency due to MCM4 deficiency

Prevalence: <1 / 1 000 000

Inheritance: Autosomal recessive

Age of onset: Childhood

ICD-10: D84.8

OMIM: 609981

UMLS: C4518328

A summary on this disease is available in Français, Español, Deutsch, Italiano, Nederlands
Detailed information

Logo ERN: produced/endorsed by ERN(s) Logo FSMR: produced/endorsed by FSMR(s)

Guidelines
Emergency guidelines
Français (2019.pdf) - Orphanet Urgences
Italiano (2015.pdf) - Orphanet Urgences
Clinical practice guidelines
The documents contained in this website are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.