silver-Russell syndrome due to maternal uniparental disomy of chromosome 7

ORPHA: 96182

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 silver-Russell syndrome due to maternal uniparental disomy of chromosome 7, sourced from HPO and Orphanet clinical annotations.

Thin vermilion borderTriangular faceAbnormality of the outer earDelayed speech and language developmentBrachydactylySyndactylyGlobal developmental delayMotor delayFailure to thriveSmall for gestational ageAbnormal facial shapeGastroesophageal refluxDownturned corners of mouthFasting hypoglycemiaRelative macrocephalyFeeding difficulties in infancyProminent foreheadClinodactylyAsymmetric growthCryptorchidismHypospadiasAbnormality of the genitourinary systemNarrow mouthPierre-Robin sequenceShort chinDental crowdingMicrodontiaDecreased response to growth hormone stimulation testPrecocious pubertyInsulin resistanceHyperhidrosisSpecific learning disabilityDelayed closure of the anterior fontanelleAbnormally high-pitched voiceAbnormal heart morphologyFrontal bossingVomitingConstipationScoliosisHigh, narrow palateAspirationObstructive sleep apneaDecreased muscle massNarrow joint spaces of the elbowClinodactyly of the 5th fingerPoor appetiteSevere intrauterine growth retardationShoulder dimpleIncreased overbitePremature adrenarcheOral aversionLower limb asymmetryEsophagitisHorseshoe kidneyRenal dysplasiaMicrognathiaIntrauterine growth retardationDelayed skeletal maturationPostnatal growth retardation

Classification & Codes

Orphanet Code

ORPHA:96182
silver-Russell syndrome due to maternal uniparental disomy of chromosome 7
OrphanetORPHA:96182
Treatments0 drug(s)
Symptoms on record59 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO