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Deficiency in anterior pituitary function-variable immunodeficiency syndrome

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Disease definition

A rare, genetic endocrine disease characterized by the association of common variable immunodeficiency, manifesting with hypogammaglobulinemia and recurrent or severe childhood-onset sinopulmonary infections, followed, possibly many years later, by symptomatic adrenocorticotropic hormone (ACTH) deficiency resulting from anterior pituitary hormone deficiency.

ORPHA:293978

Classification level: Disorder

Synonym(s):
  • DAVID syndrome

Prevalence: <1 / 1 000 000

Inheritance: Autosomal dominant

Age of onset: Childhood

ICD-10: E23.0

ICD-11: 4A01.0Y

OMIM: 615577

UMLS: C4751122

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

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

Guidelines
Emergency guidelines
Français (2020.pdf) - Orphanet Urgences
Clinical practice guidelines
English (2022) - Eur J Endocrinol Logo ERN
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