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Multiple epiphyseal dysplasia, Lowry type

MedGen UID:
321890
Concept ID:
C1832112
Disease or Syndrome
Synonym: Multiple epiphyseal dysplasia with robin phenotype
SNOMED CT: Multiple epiphyseal dysplasia Lowry type (768935003); Multiple epiphyseal dysplasia with Robin phenotype (768935003)
 
Monarch Initiative: MONDO:0011109
OMIM®: 601560
Orphanet: ORPHA166016

Definition

A rare primary bone dysplasia with characteristics of small, flat epiphyses (especially the capital femoral epiphyses), rhizomelic shortening of limbs, cleft of secondary palate, micrognathia, mild joint contractures and facial dysmorphism (including mildly upward-slanting palpebral fissures, hypertelorism, broad nasal tip). Additionally reported features include scoliosis, genu valgum, mild pectus excavatum, platyspondyly, dislocated radial heads, brachydactyly, hypoplastic fibulae and talipes equinovarus. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMultiple epiphyseal dysplasia, Lowry type
Follow this link to review classifications for Multiple epiphyseal dysplasia, Lowry type in Orphanet.

Recent clinical studies

Diagnosis

Tabib A, Richmond CM, McGaughran J
Am J Med Genet A 2023 Apr;191(4):1094-1100. Epub 2023 Jan 9 doi: 10.1002/ajmg.a.63110. PMID: 36622817
Benoit-Pilven C, Besson A, Putoux A, Benetollo C, Saccaro C, Guguin J, Sala G, Cologne A, Delous M, Lesca G, Padgett RA, Leutenegger AL, Lacroix V, Edery P, Mazoyer S
PLoS One 2020;15(7):e0235655. Epub 2020 Jul 6 doi: 10.1371/journal.pone.0235655. PMID: 32628740Free PMC Article

Prognosis

Benoit-Pilven C, Besson A, Putoux A, Benetollo C, Saccaro C, Guguin J, Sala G, Cologne A, Delous M, Lesca G, Padgett RA, Leutenegger AL, Lacroix V, Edery P, Mazoyer S
PLoS One 2020;15(7):e0235655. Epub 2020 Jul 6 doi: 10.1371/journal.pone.0235655. PMID: 32628740Free PMC Article

Clinical prediction guides

Benoit-Pilven C, Besson A, Putoux A, Benetollo C, Saccaro C, Guguin J, Sala G, Cologne A, Delous M, Lesca G, Padgett RA, Leutenegger AL, Lacroix V, Edery P, Mazoyer S
PLoS One 2020;15(7):e0235655. Epub 2020 Jul 6 doi: 10.1371/journal.pone.0235655. PMID: 32628740Free PMC Article

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