hypermethioninemia with deficiency of S-adenosylhomocysteine hydrolase

ORPHA: 88618

Overview

human disease

Available Treatments (0)

No treatments linked yet

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Clinical Presentation

Signs and symptoms associated with hypermethioninemia with deficiency of S-adenosylhomocysteine hydrolase, sourced from HPO and Orphanet clinical annotations.

HyperhomocystinemiaElevated circulating hepatic transaminase concentrationHypoalbuminemiaAbnormal circulating methionine concentrationAbnormal circulating homocysteine concentrationStrabismusEsotropiaAtypical behaviorShort attention spanGlobal developmental delayCerebellar hypoplasiaAbnormality of the liverFailure to thriveGrowth delayHydrops fetalisAbnormality of coagulationReduced antithrombin III activityAbnormal facial shapeHypoplasia of the corpus callosumDevelopmental regressionPoor head controlElevated circulating creatine kinase concentrationCNS hypomyelinationMuscular dystrophyProlonged prothrombin timeReduced factor VII activityFloppy infantHypofibrinogenemiaElevated coagulation factor V activityHypoplasia of the ponsDelayed myelinationHyperintensity of cerebral white matter on MRIAbnormality of the dentitionMicrocephalyMuscle weaknessHepatocellular carcinomaCardiomyopathyPes planusVentriculomegalyRespiratory failureHypermethioninemiaSensorimotor neuropathyAbnormality of hair textureWidened subarachnoid space

Classification & Codes

Orphanet Code

ORPHA:88618
hypermethioninemia with deficiency of S-adenosylhomocysteine hydrolase
OrphanetORPHA:88618
Treatments0 drug(s)
Symptoms on record44 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO