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4q21 microdeletion syndrome

MedGen UID:
930199
Concept ID:
C4304530
Disease or Syndrome
Synonym: Monosomy 4q21
SNOMED CT: 4q21 microdeletion syndrome (719660008); Monosomy 4q21 (719660008)

Definition

A newly described syndrome associated with facial dysmorphism, progressive growth restriction, severe intellectual deficit and absent or severely delayed speech. It has been reported in nine unrelated patients. The most common facial feature includes high or broad forehead, hypertelorism and short philtrum. Short hands and feet are frequently observed. The microdeletion critical region encompasses two candidate genes, PRKG2 and RASGEF1B, in which haploinsufficiency could participate to the phenotype. [from SNOMEDCT_US]

Recent clinical studies

Diagnosis

Giguet-Valard AG, Thevenin C, Dreux S, Decatrelle V, Juve ML, Yazza S, Adenet C, Lesueur M, Bouvagnet P, Gueneret M
Mol Genet Genomic Med 2024 Feb;12(2):e2397. doi: 10.1002/mgg3.2397. PMID: 38351708Free PMC Article
Hu X, Chen X, Wu B, Soler IM, Chen S, Shen Y
Am J Med Genet A 2017 Jan;173(1):120-125. Epub 2016 Sep 8 doi: 10.1002/ajmg.a.37965. PMID: 27604828
Lebedev IN, Nazarenko LP, Skryabin NA, Babushkina NP, Kashevarova AA
Am J Med Genet A 2016 Aug;170(8):2089-96. Epub 2016 Jun 10 doi: 10.1002/ajmg.a.37754. PMID: 27288323
Bhoj E, Halbach S, McDonald-McGinn D, Tan C, Lande R, Waggoner D, Zackai E
Am J Med Genet A 2013 Sep;161A(9):2327-33. Epub 2013 Aug 2 doi: 10.1002/ajmg.a.36061. PMID: 23913759

Prognosis

Tsang E, Rupps R, McGillivray B, Eydoux P, Marra M, Arbour L, Langlois S, Friedman JM, Zahir FR
Am J Med Genet A 2012 Oct;158A(10):2606-9. Epub 2012 Aug 17 doi: 10.1002/ajmg.a.35568. PMID: 22903878

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