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Teebi hypertelorism syndrome(TBHS; TBHS1; BBBG2, FORMERLY; OGS2, F...)

MedGen UID:
208673
Concept ID:
C0796179
Disease or Syndrome
Synonym: Brachycephalofrontonasal dysplasia
SNOMED CT: Hypertelorism Teebi type (724284005); Brachycephalofrontonasal dysplasia (724284005); Craniofrontonasal dysplasia Teebi type (724284005); Teebi hypertelorism syndrome (724284005); Teebi syndrome (724284005)
 
Related genes: SPECC1L, CDH11
 
Monarch Initiative: MONDO:0030639
OMIM®: 145420; 614140
OMIM® Phenotypic series: PS145420

Definition

A rare genetic disease with characteristics of hypertelorism with facial features that can closely resemble craniofrontonasal dysplasia, such as prominent forehead, widow''s peak, heavy and broad eyebrows, long palpebral fissures, ptosis, high and broad nasal bridge, short nose, low-set ears, natal teeth, thin upper lip and a grooved chin. Limb features include fifth-finger clinodactyly, pes adductus, mild interdigital webbing. Urogenital features include bilateral cryptorchidism and shawl scrotum in males. Other manifestations include umbilical hernia/omphalocele and cardiac defects. Psychomotor development is normal. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  

Recent clinical studies

Diagnosis

Kuroda Y, Saito Y, Enomoto Y, Naruto T, Kurosawa K
Am J Med Genet A 2024 Jan;194(1):94-99. Epub 2023 Aug 30 doi: 10.1002/ajmg.a.63386. PMID: 37646430
Han XD, Cox V, Slavotinek A
Am J Med Genet A 2006 Sep 15;140(18):1960-4. doi: 10.1002/ajmg.a.31439. PMID: 16906548

Prognosis

Li D, March ME, Fortugno P, Cox LL, Matsuoka LS, Monetta R, Seiler C, Pyle LC, Bedoukian EC, Sánchez-Soler MJ, Caluseriu O, Grand K, Tam A, Aycinena ARP, Camerota L, Guo Y, Sleiman P, Callewaert B, Kumps C, Dheedene A, Buckley M, Kirk EP, Turner A, Kamien B, Patel C, Wilson M, Roscioli T, Christodoulou J, Cox TC, Zackai EH, Brancati F, Hakonarson H, Bhoj EJ
Hum Genet 2021 Jul;140(7):1061-1076. Epub 2021 Apr 3 doi: 10.1007/s00439-021-02274-3. PMID: 33811546Free PMC Article

Clinical prediction guides

Kuroda Y, Saito Y, Enomoto Y, Naruto T, Kurosawa K
Am J Med Genet A 2024 Jan;194(1):94-99. Epub 2023 Aug 30 doi: 10.1002/ajmg.a.63386. PMID: 37646430
Li D, March ME, Fortugno P, Cox LL, Matsuoka LS, Monetta R, Seiler C, Pyle LC, Bedoukian EC, Sánchez-Soler MJ, Caluseriu O, Grand K, Tam A, Aycinena ARP, Camerota L, Guo Y, Sleiman P, Callewaert B, Kumps C, Dheedene A, Buckley M, Kirk EP, Turner A, Kamien B, Patel C, Wilson M, Roscioli T, Christodoulou J, Cox TC, Zackai EH, Brancati F, Hakonarson H, Bhoj EJ
Hum Genet 2021 Jul;140(7):1061-1076. Epub 2021 Apr 3 doi: 10.1007/s00439-021-02274-3. PMID: 33811546Free PMC Article
Bhoj EJ, Haye D, Toutain A, Bonneau D, Nielsen IK, Lund IB, Bogaard P, Leenskjold S, Karaer K, Wild KT, Grand KL, Astiazaran MC, Gonzalez-Nieto LA, Carvalho A, Lehalle D, Amudhavalli SM, Repnikova E, Saunders C, Thiffault I, Saadi I, Li D, Hakonarson H, Vial Y, Zackai E, Callier P, Drunat S, Verloes A
Eur J Med Genet 2019 Dec;62(12):103588. Epub 2018 Nov 22 doi: 10.1016/j.ejmg.2018.11.022. PMID: 30472488Free PMC Article
Machado-Paula LA, Guion-Almeida ML
Am J Med Genet A 2003 Mar 1;117A(2):181-3. doi: 10.1002/ajmg.a.10919. PMID: 12567419
Tsukahara M, Uchida M, Shinohara T
Am J Med Genet 1995 Oct 23;59(1):59-61. doi: 10.1002/ajmg.1320590113. PMID: 8849013

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