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Short stature due to GHSR deficiency

ORPHA:314811· ICD-10 E34.3

Definition

Short stature due to GHSR deficiency is a rare, genetic, endocrine growth disease, resulting from growth hormone secretagogue receptor (GHSR) deficiency, characterized by postnatal growth delay that results in short stature (less than -2 SD). The pituitary gland is typically without morphological changes, although anterior pituitary gland hypoplasia has been reported.

Prevalence
<1 / 1 000 000
Inheritance
Autosomal dominant, Autosomal recessive
Age of onset
Infancy, Neonatal