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Distal trisomy 10q

MedGen UID:
419480
Concept ID:
C2931728
Disease or Syndrome
Synonyms: Chromosome 10, distal trisomy 10q; Distal Duplication 10q; Distal Trisomy 10q Syndrome
SNOMED CT: Distal trisomy 10q (718689000); Distal duplication 10q (718689000)
 
Monarch Initiative: MONDO:0019884
Orphanet: ORPHA96102

Definition

Distal trisomy of the long arm of chromosome 10 results in characteristics of pre and postnatal growth retardation, a pattern of specific facial features, hypotonia, and developmental and psychomotor delay. To date, approximately 40 cases have been reported. Most cases are diagnosed in infancy or in childhood. The range and severity of symptoms and physical findings may vary from case to case, depending upon the exact length and location of the duplicated portion of chromosome 10q. The duplicated region almost always includes 10qter, with the most frequent proximal breakpoint at 10q24 (with variation from q22 to q25). Interstitial duplications of 10q have also been reported. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  

Recent clinical studies

Etiology

He JP, Qian Y, Liu WJ, Tang J, Qin MH, Luo SJ, Hou JH, Lv MX
Taiwan J Obstet Gynecol 2021 Mar;60(2):318-323. doi: 10.1016/j.tjog.2021.01.010. PMID: 33678334

Diagnosis

He JP, Qian Y, Liu WJ, Tang J, Qin MH, Luo SJ, Hou JH, Lv MX
Taiwan J Obstet Gynecol 2021 Mar;60(2):318-323. doi: 10.1016/j.tjog.2021.01.010. PMID: 33678334
Soekarman D, Fryns JP
Genet Couns 1992;3(2):111-3. PMID: 1642808

Clinical prediction guides

Bregant L, Gersak K, Veble A
Genet Couns 2005;16(1):59-63. PMID: 15844780
Garcia-Heras J, Martin JA, Witchel SF, Scacheri P
J Med Genet 1997 Mar;34(3):242-5. doi: 10.1136/jmg.34.3.242. PMID: 9132498Free PMC Article

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