Non-acquired panhypopituitarism

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Non-acquired panhypopituitarism

ORPHA:90695Disease

Also called Genetic panhypopituitarism

What it is

A rare genetic pituitary disease characterized by variable deficiency of all hormones produced in the anterior lobe of the pituitary gland. Clinical manifestations include hypothyroidism, hypogonadism, growth retardation and short stature, and secondary adrenal insufficiency. Age of onset is variable. Signs and symptoms usually develop gradually, and loss of the different hormones is often sequential.

Key facts

Prevalence
<1 / 1 000 000
Age of onset
All ages
Inheritance
Autosomal recessive, X-linked recessive
Classified as
Disease

Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.

Signs and symptoms

These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.

Genes

PROP1Disease-causing germline mutation(s)
SOX3Disease-causing germline mutation(s) (loss of function)

ICD-10 codes

E23.0filed under a broader ICD-10 category — shared with 23 other rare diseases

ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.

Related health topics

Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.

Benefit programs to look at

Programs whose eligibility touches the same ICD-10 categories. Eligibility is decided by the administering agency, never by this page.

Cross-references

MEDDRA 10033662MONDO 0019591MONDO 19591OMIM 262600OMIM 312000UMLS C5680191

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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