PSAT deficiency

MeSH: C567032ORPHA: 284417

Overview

serine deficiency that has material basis in deficiency of phosphoserine aminotransferase and is characterized by low concentrations of serine and flycine in plasma and cerebrospinal fluid

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 PSAT deficiency, sourced from HPO and Orphanet clinical annotations.

Failure to thrive in infancyHyperglycinemiaFeeding difficulties in infancyPrimary microcephalyHyposerinemiaProfound global developmental delayInappropriate cryingThickened nuchal skin foldSeizureHypertoniaHyperreflexiaIntrauterine growth retardationHypoplasia of the corpus callosumAxial hypotoniaCerebral white matter hypoplasiaHypertelorismSloping foreheadMicrognathiaShort neckSpastic tetraparesisCerebellar vermis hypoplasiaMyoclonusLissencephalyCraniosynostosisBilateral talipes equinovarusEEG with polyspike wave complexesLimb joint contractureDepressed nasal bridgeKnee flexion contractureAnkle flexion contractureLateral ventricle dilatationParoxysmal involuntary eye movementsIchthyosisSimplified gyral patternEpileptic spasmEEG with focal sharp wavesGastrostomy tube feeding in infancyDelayed myelinationNasogastric tube feedingCyanotic episode

Classification & Codes

MeSH Code

C567032

Orphanet Code

ORPHA:284417
PSAT deficiency
MeSHC567032
OrphanetORPHA:284417
Treatments0 drug(s)
Symptoms on record40 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO