U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Ring chromosome 17

MedGen UID:
419477
Concept ID:
C2931714
Cell or Molecular Dysfunction
Synonym: Chromosome 17 ring
Modes of inheritance:
Unknown inheritance
MedGen UID:
989040
Concept ID:
CN307042
Finding
Source: Orphanet
Hereditary clinical entity whose mode of inheritance is unknown.
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0015433
Orphanet: ORPHA1441

Definition

Ring chromosome 17 syndrome is a rare chromosomal anomaly syndrome, resulting from partial deletion of chromosome 17, characterized by highly variable manifestations, ranging from a severe phenotype which presents with lissencephaly and severe intellectual disability to a milder phenotype that includes short stature, microcephaly, intellectual disability, seizures (that may be pharmacoresistant), café-au-lait spots, retinal flecks and minor facial dysmorphism, depending on the presence or absence of the Miller-Dieker critical region. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVRing chromosome 17
Follow this link to review classifications for Ring chromosome 17 in Orphanet.

Recent clinical studies

Etiology

Chapiro E, Lesty C, Gabillaud C, Durot E, Bouzy S, Armand M, Le Garff-Tavernier M, Bougacha N, Struski S, Bidet A, Laharanne E, Barin C, Veronese L, Prié N, Eclache V, Gaillard B, Michaux L, Lefebvre C, Gaillard JB, Terré C, Penther D, Bastard C, Nadal N, Fert-Ferrer S, Auger N, Godon C, Sutton L, Tournilhac O, Susin SA, Nguyen-Khac F; GroupeFrancophone de Cytogénétique Hématologique(GFCH) and the French Innovative Leukemia Organization (FILO) group
Am J Hematol 2018 Mar;93(3):375-382. Epub 2017 Dec 18 doi: 10.1002/ajh.24990. PMID: 29194741
Kotzot D
J Med Genet 2008 Sep;45(9):545-56. Epub 2008 Jun 4 doi: 10.1136/jmg.2008.058016. PMID: 18524837
Havlovicova M, Novotna D, Kocarek E, Novotna K, Bendova S, Petrak B, Hrdlicka M, Sedlacek Z
Am J Med Genet A 2007 Jan 1;143A(1):76-81. doi: 10.1002/ajmg.a.31569. PMID: 17163520
Shashi V, White JR, Pettenati MJ, Root SK, Bell WL
Clin Genet 2003 Oct;64(4):361-5. doi: 10.1034/j.1399-0004.2003.00146.x. PMID: 12974742

Diagnosis

Kotzot D
J Med Genet 2008 Sep;45(9):545-56. Epub 2008 Jun 4 doi: 10.1136/jmg.2008.058016. PMID: 18524837
Havlovicova M, Novotna D, Kocarek E, Novotna K, Bendova S, Petrak B, Hrdlicka M, Sedlacek Z
Am J Med Genet A 2007 Jan 1;143A(1):76-81. doi: 10.1002/ajmg.a.31569. PMID: 17163520
Dupont C, Pipiras E, Chantot-Bastaraud S, Verloes A, Baumann C, Wolf JP, Benzacken B
Eur J Hum Genet 2003 Jun;11(6):452-6. doi: 10.1038/sj.ejhg.5200984. PMID: 12774038
Stratton RF, Dobyns WB, Airhart SD, Ledbetter DH
Hum Genet 1984;67(2):193-200. doi: 10.1007/BF00273000. PMID: 6745939

Prognosis

Chapiro E, Lesty C, Gabillaud C, Durot E, Bouzy S, Armand M, Le Garff-Tavernier M, Bougacha N, Struski S, Bidet A, Laharanne E, Barin C, Veronese L, Prié N, Eclache V, Gaillard B, Michaux L, Lefebvre C, Gaillard JB, Terré C, Penther D, Bastard C, Nadal N, Fert-Ferrer S, Auger N, Godon C, Sutton L, Tournilhac O, Susin SA, Nguyen-Khac F; GroupeFrancophone de Cytogénétique Hématologique(GFCH) and the French Innovative Leukemia Organization (FILO) group
Am J Hematol 2018 Mar;93(3):375-382. Epub 2017 Dec 18 doi: 10.1002/ajh.24990. PMID: 29194741

Clinical prediction guides

Chapiro E, Lesty C, Gabillaud C, Durot E, Bouzy S, Armand M, Le Garff-Tavernier M, Bougacha N, Struski S, Bidet A, Laharanne E, Barin C, Veronese L, Prié N, Eclache V, Gaillard B, Michaux L, Lefebvre C, Gaillard JB, Terré C, Penther D, Bastard C, Nadal N, Fert-Ferrer S, Auger N, Godon C, Sutton L, Tournilhac O, Susin SA, Nguyen-Khac F; GroupeFrancophone de Cytogénétique Hématologique(GFCH) and the French Innovative Leukemia Organization (FILO) group
Am J Hematol 2018 Mar;93(3):375-382. Epub 2017 Dec 18 doi: 10.1002/ajh.24990. PMID: 29194741
Vazna A, Havlovicova M, Sedlacek Z
Gene 2008 Jan 15;407(1-2):186-92. Epub 2007 Oct 13 doi: 10.1016/j.gene.2007.10.009. PMID: 17996402
Ricard-Mousnier B, N'Guyen S, Dubas F, Pouplard F, Guichet A
Epileptic Disord 2007 Sep;9(3):327-31. Epub 2007 Sep 20 doi: 10.1684/epd.2007.0121. PMID: 17884758
Havlovicova M, Novotna D, Kocarek E, Novotna K, Bendova S, Petrak B, Hrdlicka M, Sedlacek Z
Am J Med Genet A 2007 Jan 1;143A(1):76-81. doi: 10.1002/ajmg.a.31569. PMID: 17163520
Teyssier M, Charrin C, Corgiolu Theuil G, David L
Ann Genet 1992;35(2):75-8. PMID: 1381884

Supplemental Content

Table of contents

    Genetic Testing Registry

    Clinical resources

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...