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Ring chromosome 2

MedGen UID:
1642621
Concept ID:
C4707448
Disease or Syndrome
Synonyms: chromosome 2 ring; R2; Ring 2; ring chromosome 2; Ring chromosome 2 syndrome; Ring chromosome type 2; rose cluster 2
SNOMED CT: Ring chromosome 2 syndrome (765485000); Ring chromosome 2 (765485000)
 
Monarch Initiative: MONDO:0019903
Orphanet: ORPHA96171

Definition

A rare chromosomal anomaly syndrome with highly variable phenotype. Principal characteristics are intrauterine growth retardation, failure to thrive, developmental delay, hypotonia, mild dysmorphic features (including microcephaly, short forehead, upslanting palpebral fissures, hypertelorism, epicanthal folds, wide nasal bridge, broad nasal tip, long philtrum, thin upper lip, micrognathia, short neck), skeletal anomalies (for example kyphosis, brachydactyly, clinodactyly, talipes equinovarus) and dermatological features (including cafe-au-lait spots). Patients may also present ventriculoseptal defects and genital abnormalities (for example genital hypoplasia, phimosis, cryptorchidism). [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVRing chromosome 2

Professional guidelines

PubMed

Chen CP, Lin CJ, Chang TY, Chern SR, Wu PS, Chen YT, Su JW, Lee CC, Chen LF, Wang W
Gene 2013 Apr 25;519(1):164-8. Epub 2013 Feb 9 doi: 10.1016/j.gene.2013.01.055. PMID: 23403238
Giardino D, Finelli P, Russo S, Gottardi G, Rodeschini O, Atza MG, Natacci F, Larizza L
Am J Med Genet 2002 Aug 15;111(3):319-23. doi: 10.1002/ajmg.10537. PMID: 12210331
Arthur DC, Bloomfield CD
Cancer Genet Cytogenet 1984 Jul;12(3):189-99. doi: 10.1016/0165-4608(84)90030-x. PMID: 6722761

Recent clinical studies

Etiology

Sarri C, Douzgou S, Kontos H, Anagnostopoulou K, Tümer Z, Grigoriadou M, Petersen MB, Kokotas H, Merou K, Pandelia E, Giouroukou E, Papanikolaou K, Côté GB, Gyftodimou Y
Cytogenet Genome Res 2015;145(1):6-13. Epub 2015 May 14 doi: 10.1159/000382046. PMID: 25997743

Diagnosis

Chen CP, Lin CJ, Chang TY, Chern SR, Wu PS, Chen YT, Su JW, Lee CC, Chen LF, Wang W
Gene 2013 Apr 25;519(1):164-8. Epub 2013 Feb 9 doi: 10.1016/j.gene.2013.01.055. PMID: 23403238
Chen CP, Chen M, Chern SR, Wu PS, Chang SP, Lee DJ, Chen YT, Chen LF, Su JW, Hwa-Ruey Hsieh A, Hwa-Jiun Hsieh A, Wang W
Taiwan J Obstet Gynecol 2012 Sep;51(3):411-7. doi: 10.1016/j.tjog.2012.07.017. PMID: 23040927
Giardino D, Finelli P, Russo S, Gottardi G, Rodeschini O, Atza MG, Natacci F, Larizza L
Am J Med Genet 2002 Aug 15;111(3):319-23. doi: 10.1002/ajmg.10537. PMID: 12210331

Prognosis

Sarri C, Douzgou S, Kontos H, Anagnostopoulou K, Tümer Z, Grigoriadou M, Petersen MB, Kokotas H, Merou K, Pandelia E, Giouroukou E, Papanikolaou K, Côté GB, Gyftodimou Y
Cytogenet Genome Res 2015;145(1):6-13. Epub 2015 May 14 doi: 10.1159/000382046. PMID: 25997743

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