Kniest dysplasia

ICD-10: Q77.8MeSH: C537207ORPHA: 2746

Overview

Human disease

Available Treatments (0)

No treatments linked yet

Clinical trials or compassionate use may be available — consult a specialist.

Clinical Presentation

Signs and symptoms associated with Kniest dysplasia, sourced from HPO and Orphanet clinical annotations.

Large fontanellesMacrocephalyBlue scleraePectus excavatumNarrow chestAbnormal metaphysis morphologyBrachydactylyTapered fingerHypotoniaJoint stiffnessSplenomegalyFrontal bossingRespiratory insufficiencyRecurrent respiratory infectionsHepatomegalyDelayed skeletal maturationHypoplastic pubic boneHypoplastic ischiaSquared iliac bonesShort noseSevere short statureDepressed nasal bridgeFlat occiputAbnormal epiphysis morphologyHypoplastic vertebral bodiesBroad thumbAbnormally ossified vertebrae

Classification & Codes

ICD-10 Code

Q77.8

MeSH Code

C537207

Orphanet Code

ORPHA:2746
Kniest dysplasia
ICD-10Q77.8
MeSHC537207
OrphanetORPHA:2746
Treatments0 drug(s)
Symptoms on record27 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO