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Mosaic trisomy 2

MedGen UID:
1631294
Concept ID:
C4707010
Disease or Syndrome
Synonym: Trisomy 2 mosaicism
SNOMED CT: Trisomy 2 mosaicism (764623009); Mosaic trisomy chromosome 2 (764623009); Mosaic trisomy 2 syndrome (764623009)
 
Monarch Initiative: MONDO:0015763
Orphanet: ORPHA1723

Definition

A rare chromosomal anomaly syndrome with a highly variable phenotype. Principle characteristics are intrauterine growth restriction, growth and motor delay, craniofacial dysmorphism (microcephaly, hypertelorism, micro/anophthalmia, midface hypoplasia, cleft lip/palate), congenital heart and neural tube defects, as well as various skeletal (scoliosis, radioulnar hypoplasia, preaxial polydactyly) and gastrointestinal (intestinal malrotation, Hirschsprung disease) anomalies. Central nervous system malformations (including ventriculomegaly, thin corpus callosum, spina bifida) have also been reported. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMosaic trisomy 2

Professional guidelines

PubMed

Smrcek JM, Germer U, Krokowski M, Berg C, Krapp M, Geipel A, Gembruch U
Ultrasound Obstet Gynecol 2003 Apr;21(4):322-8. doi: 10.1002/uog.84. PMID: 12704737

Recent clinical studies

Etiology

Tuğ E, Karcaaltincaba D, Yirmibeş Karaoğuz M, Saat H, Özek A
J Matern Fetal Neonatal Med 2017 Jul;30(13):1579-1583. Epub 2016 Oct 26 doi: 10.1080/14767058.2016.1214700. PMID: 27431726
Edlow AG, Reiss R, Benson CB, Gerrol P, Wilkins-Haug L
Prenat Diagn 2011 Mar;31(3):299-306. Epub 2011 Jan 13 doi: 10.1002/pd.2691. PMID: 21234973
Smrcek JM, Germer U, Krokowski M, Berg C, Krapp M, Geipel A, Gembruch U
Ultrasound Obstet Gynecol 2003 Apr;21(4):322-8. doi: 10.1002/uog.84. PMID: 12704737
Hahnemann JM, Vejerslev LO
Am J Med Genet 1997 May 16;70(2):179-87. doi: 10.1002/(sici)1096-8628(19970516)70:2<179::aid-ajmg15>3.0.co;2-g. PMID: 9128940
Fryburg JS, Dimaio MS, Mahoney MJ
Prenat Diagn 1992 Mar;12(3):157-62. doi: 10.1002/pd.1970120303. PMID: 1375377

Diagnosis

Chen CP, Wu FT, Chern SR, Wu PS, Pan YT, Lee CC, Pan CW, Wang W
Taiwan J Obstet Gynecol 2023 Jul;62(4):571-576. doi: 10.1016/j.tjog.2023.05.002. PMID: 37407197
Pontoriero F, Silverman AM, Pascasio JM, Bajaj R
Pediatr Dev Pathol 2020 Nov-Dec;23(6):448-452. Epub 2020 Aug 5 doi: 10.1177/1093526620945861. PMID: 32755442
Tuğ E, Karcaaltincaba D, Yirmibeş Karaoğuz M, Saat H, Özek A
J Matern Fetal Neonatal Med 2017 Jul;30(13):1579-1583. Epub 2016 Oct 26 doi: 10.1080/14767058.2016.1214700. PMID: 27431726
Chen CP, Chen YY, Chern SR, Wu PS, Su JW, Chen YT, Lee CC, Chen LF, Wang W
Taiwan J Obstet Gynecol 2013 Sep;52(3):395-400. doi: 10.1016/j.tjog.2013.06.004. PMID: 24075380
Chen CP, Su YN, Chern SR, Chen YT, Wu PS, Su JW, Pan CW, Wang W
Taiwan J Obstet Gynecol 2012 Dec;51(4):603-11. doi: 10.1016/j.tjog.2012.09.016. PMID: 23276565

Therapy

Webb AL, Sturgiss S, Warwicker P, Robson SC, Goodship JA, Wolstenholme J
Prenat Diagn 1996 Oct;16(10):958-62. doi: 10.1002/(SICI)1097-0223(199610)16:10<958::AID-PD971>3.0.CO;2-U. PMID: 8938070

Prognosis

Hahnemann JM, Vejerslev LO
Am J Med Genet 1997 May 16;70(2):179-87. doi: 10.1002/(sici)1096-8628(19970516)70:2<179::aid-ajmg15>3.0.co;2-g. PMID: 9128940
Fryburg JS, Dimaio MS, Mahoney MJ
Prenat Diagn 1992 Mar;12(3):157-62. doi: 10.1002/pd.1970120303. PMID: 1375377

Clinical prediction guides

Pontoriero F, Silverman AM, Pascasio JM, Bajaj R
Pediatr Dev Pathol 2020 Nov-Dec;23(6):448-452. Epub 2020 Aug 5 doi: 10.1177/1093526620945861. PMID: 32755442
Hahnemann JM, Vejerslev LO
Am J Med Genet 1997 May 16;70(2):179-87. doi: 10.1002/(sici)1096-8628(19970516)70:2<179::aid-ajmg15>3.0.co;2-g. PMID: 9128940

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