Dolichol kinase deficiency

ORPHA: 91131

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 Dolichol kinase deficiency, sourced from HPO and Orphanet clinical annotations.

Dilated cardiomyopathyType I transferrin isoform profileAbnormal circulating enzyme concentration or activityCardiomyocyte hypertrophySeizureFailure to thriveCongestive heart failureElevated circulating hepatic transaminase concentrationIchthyosisMild global developmental delayInterstitial cardiac fibrosisProgressive microcephalyStrabismusVisual impairmentAutistic behaviorReduced eye contactIntellectual disabilityMotor delayAbsent speechBilateral tonic-clonic seizureHypsarrhythmiaProgressive muscle weaknessShort statureSevere muscular hypotoniaFocal-onset seizureFloppy infantMultifocal epileptiform dischargesEEG with generalized slow activityArrhythmiaInfantile spasmsDelayed ability to standDelayed ability to walk

Classification & Codes

Orphanet Code

ORPHA:91131
Dolichol kinase deficiency
OrphanetORPHA:91131
Treatments0 drug(s)
Symptoms on record32 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO