hyperlysinemia

MeSH: D020167ORPHA: 2203

Overview

amino acid metabolic disorder that involves an abnormal increase of lysine in the blood

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 hyperlysinemia, sourced from HPO and Orphanet clinical annotations.

Upgaze palsyTip-toe gaitNeck hypertoniaNasogastric tube feedingThin eyebrowAbnormality of movementMicrocephalyAtypical behaviorShort attention spanDelayed speech and language developmentIntellectual disabilitySeizureGlobal developmental delayHyperammonemiaHyperlysinemiaCystinuriaArgininuriaHyperlysinuriaShort statureFloppy infantEEG with spike-wave complexesAbnormal circulating enzyme concentration or activityDecreased urine alpha-ketoglutarate concentrationNeurodevelopmental delayHypoornithinemiaDecreased CSF arginine concentrationIncreased CSF lysine concentrationAbnormal CSF ornithine concentrationHigh palateSmooth philtrumDepressed nasal ridgeStrabismusHypotelorismHyperactivitySpastic diplegiaSpastic tetraparesisDysmetriaTremorBrisk reflexesCraniosynostosisFailure to thriveAbnormal facial shapeVomitingDysphagiaGastroesophageal refluxPoor suckOpisthotonusIncoordinationClumsinessPulmonary artery hypoplasiaRecurrent pneumoniaBorderline intellectual disabilityHypoplastic helicesHypoplasia of the antihelixSimple febrile seizureMild global developmental delayFeeding difficulties

Classification & Codes

MeSH Code

D020167

Orphanet Code

ORPHA:2203
hyperlysinemia
MeSHD020167
OrphanetORPHA:2203
Treatments0 drug(s)
Symptoms on record57 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO