Disease Directory Achondroplasia
Connective Tissue

Achondroplasia

Also known as: short-limbed dwarfism, FGFR3 gain-of-function, classic achondroplasia

Prevalence

1 in 15,000–40,000

Onset

Congenital

Type

Genetic — autosomal dominant (80% de novo)

Gene

FGFR3

About Achondroplasia

Achondroplasia is the most common form of disproportionate short stature, caused by a recurrent gain-of-function variant (p.Gly380Arg) in FGFR3, which encodes fibroblast growth factor receptor 3, resulting in constitutive inhibition of endochondral bone ossification. Clinical features include rhizomelic shortening of limbs, macrocephaly with midface hypoplasia, trident hand configuration, and lumbar hyperlordosis, with intelligence and life expectancy generally normal in the absence of complications. Neurological complications from foramen magnum stenosis and spinal canal narrowing represent the most serious potential morbidities.

Common Clinical Features

Disproportionate short stature with rhizomelic (proximal) limb shortening Macrocephaly and frontal bossing Midface hypoplasia and depressed nasal bridge Trident hand configuration Lumbar hyperlordosis and thoracolumbar kyphosis in infancy Foramen magnum stenosis with risk of cervicomedullary compression Spinal stenosis causing neurogenic claudication in adults

Clinical Trial Eligibility Tips

What to know before applying to Achondroplasia trials.

Height, growth velocity, and limb-length measurements are primary endpoints in most trials — ensure current anthropometric data (within 3 months) is available using condition-specific growth charts.

Most approved and investigational therapies (vosoritide, CNP analogues, FGFR3 inhibitors) have specific age and weight cutoffs; confirm current age, weight, and growth plate status before applying.

Prior or current growth hormone therapy, limb-lengthening surgery, or foramen magnum decompression must be disclosed as these significantly affect trial eligibility and stratification.

Patient Resources

Patient Organization

Little People of America

Visit website ↗

Natural History Registry

CLARITY (Achondroplasia International Registry)

Join registry ↗

Orphanet

European reference resource for rare diseases (ORPHA:15)

View on Orphanet ↗

NORD

National Organization for Rare Disorders

Search NORD ↗

Find recruiting Achondroplasia trials

Search 500,000+ studies from ClinicalTrials.gov, filtered for Achondroplasia. Updated daily.

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