U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Brachydactyly syndrome type B

MedGen UID:
722046
Concept ID:
C1300267
Disease or Syndrome
Synonym: Brachydactyly type B
SNOMED CT: Brachydactyly syndrome type B (389168002)
 
Related genes: NOG, ROR2
 
HPO: HP:0005831
Monarch Initiative: MONDO:0019676

Definition

Type B1 brachydactyly (BDB1) is the most severe type of human brachydactyly, and shows high penetrance and variable expressivity. Hypoplastic or absent distal phalanges and nails of digits 2 through 5 in the hands and feet are cardinal phenotypic features of BDB1. The middle phalanges of digits 2 through 5 are usually short and may form a bony fusion with the corresponding hypoplastic distal phalanges. The deformed thumbs are often flat, broad, or bifid. A rarer feature of BDB1 is cutaneous syndactyly affecting both fingers and toes (summary by Lv et al., 2009). [from OMIM]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
Follow this link to review classifications for Brachydactyly syndrome type B in Orphanet.

Conditions with this feature

Coloboma of macula-brachydactyly type B syndrome
MedGen UID:
343882
Concept ID:
C1852752
Disease or Syndrome
A malformation syndrome with the combination of bilateral coloboma of macula, horizontal pendular nystagmus, severe visual loss and brachydactyly type B. The hand and feet defects comprise shortening of the middle and terminal phalanges of the second to fifth digits, hypoplastic or absent nails, broad or bifid thumbs and halluces, syndactyly and flexion deformities of the joints of some digits. Inherited in a dominant manner.
Brachydactyly type B1
MedGen UID:
349432
Concept ID:
C1862112
Congenital Abnormality
A rare subtype of brachydactyly type B characterized by hypoplasia or aplasia of the distal phalanges of digits 2-5 with or without nail dysplasia, in association with fusion of the middle and distal phalanges, a broad or bifid thumb, and occasionally distal and proximal symphalangism or syndactyly. The feet are less severely affected than the hands.

Recent clinical studies

Etiology

Gliem M, Müller PL, Mangold E, Bolz HJ, Stöhr H, Weber BH, Holz FG, Charbel Issa P
Ophthalmology 2015 Aug;122(8):1555-62. Epub 2015 Jun 12 doi: 10.1016/j.ophtha.2015.04.035. PMID: 26077580
Kapoor KG, Bakri SJ
J Ocul Pharmacol Ther 2013 May;29(4):444-7. Epub 2013 Apr 12 doi: 10.1089/jop.2011.0206. PMID: 23581613Free PMC Article
Poerink JG, Kon M, van Minnen LP
J Plast Reconstr Aesthet Surg 2011 Jun;64(6):822-6. Epub 2010 Oct 6 doi: 10.1016/j.bjps.2010.08.043. PMID: 20926359
Sammar M, Stricker S, Schwabe GC, Sieber C, Hartung A, Hanke M, Oishi I, Pohl J, Minami Y, Sebald W, Mundlos S, Knaus P
Genes Cells 2004 Dec;9(12):1227-38. doi: 10.1111/j.1365-2443.2004.00799.x. PMID: 15569154

Diagnosis

Shao J, Liu Y, Zhao S, Sun W, Zhan J, Cao L
BMC Pediatr 2022 Sep 5;22(1):528. doi: 10.1186/s12887-022-03564-z. PMID: 36064339Free PMC Article
Iznardo H, Bredrup C, Bernal S, Gladkauskas T, Mascaró JM Jr, Roé E, Baselga E
Am J Med Genet A 2022 Apr;188(4):1233-1238. Epub 2021 Dec 11 doi: 10.1002/ajmg.a.62603. PMID: 34894066
Simsek-Kiper PO, Taskiran EZ, Kosukcu C, Urel-Demir G, Akgun-Dogan O, Yilmaz G, Utine GE, Nishimura G, Boduroglu K, Alikasifoglu M
Am J Med Genet A 2018 Sep;176(9):2009-2016. Epub 2018 Jul 31 doi: 10.1002/ajmg.a.40427. PMID: 30063090
Gliem M, Müller PL, Mangold E, Bolz HJ, Stöhr H, Weber BH, Holz FG, Charbel Issa P
Ophthalmology 2015 Aug;122(8):1555-62. Epub 2015 Jun 12 doi: 10.1016/j.ophtha.2015.04.035. PMID: 26077580
Hellani A, Abu-Amero K, Azouri J, Al-Sharif H, Barblet H, El-Akoum S
Reprod Biomed Online 2009 Jan;18(1):127-31. doi: 10.1016/s1472-6483(10)60434-8. PMID: 19146779

Therapy

Iznardo H, Bredrup C, Bernal S, Gladkauskas T, Mascaró JM Jr, Roé E, Baselga E
Am J Med Genet A 2022 Apr;188(4):1233-1238. Epub 2021 Dec 11 doi: 10.1002/ajmg.a.62603. PMID: 34894066
Kapoor KG, Bakri SJ
J Ocul Pharmacol Ther 2013 May;29(4):444-7. Epub 2013 Apr 12 doi: 10.1089/jop.2011.0206. PMID: 23581613Free PMC Article
Battaglia Parodi M, Iacono P, Bandello F
Dev Ophthalmol 2010;46:107-110. Epub 2010 Aug 10 doi: 10.1159/000320013. PMID: 20703036

Prognosis

Shao J, Liu Y, Zhao S, Sun W, Zhan J, Cao L
BMC Pediatr 2022 Sep 5;22(1):528. doi: 10.1186/s12887-022-03564-z. PMID: 36064339Free PMC Article
Kapoor KG, Bakri SJ
J Ocul Pharmacol Ther 2013 May;29(4):444-7. Epub 2013 Apr 12 doi: 10.1089/jop.2011.0206. PMID: 23581613Free PMC Article
Battaglia Parodi M, Iacono P, Bandello F
Dev Ophthalmol 2010;46:107-110. Epub 2010 Aug 10 doi: 10.1159/000320013. PMID: 20703036
Schwabe GC, Tinschert S, Buschow C, Meinecke P, Wolff G, Gillessen-Kaesbach G, Oldridge M, Wilkie AO, Kömec R, Mundlos S
Am J Hum Genet 2000 Oct;67(4):822-31. Epub 2000 Sep 12 doi: 10.1086/303084. PMID: 10986040Free PMC Article
Oldridge M, Fortuna AM, Maringa M, Propping P, Mansour S, Pollitt C, DeChiara TM, Kimble RB, Valenzuela DM, Yancopoulos GD, Wilkie AO
Nat Genet 2000 Mar;24(3):275-8. doi: 10.1038/73495. PMID: 10700182

Clinical prediction guides

Shao J, Liu Y, Zhao S, Sun W, Zhan J, Cao L
BMC Pediatr 2022 Sep 5;22(1):528. doi: 10.1186/s12887-022-03564-z. PMID: 36064339Free PMC Article
Iznardo H, Bredrup C, Bernal S, Gladkauskas T, Mascaró JM Jr, Roé E, Baselga E
Am J Med Genet A 2022 Apr;188(4):1233-1238. Epub 2021 Dec 11 doi: 10.1002/ajmg.a.62603. PMID: 34894066
Gliem M, Müller PL, Mangold E, Bolz HJ, Stöhr H, Weber BH, Holz FG, Charbel Issa P
Ophthalmology 2015 Aug;122(8):1555-62. Epub 2015 Jun 12 doi: 10.1016/j.ophtha.2015.04.035. PMID: 26077580
Yoda A, Oishi I, Minami Y
J Recept Signal Transduct Res 2003 Feb;23(1):1-15. doi: 10.1081/rrs-120018757. PMID: 12680586
Oldridge M, Temple IK, Santos HG, Gibbons RJ, Mustafa Z, Chapman KE, Loughlin J, Wilkie AO
Am J Hum Genet 1999 Feb;64(2):578-85. doi: 10.1086/302255. PMID: 9973296Free PMC Article

Supplemental Content

Table of contents

    Clinical resources

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...