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Brachydactyly type E(BDE)

MedGen UID:
939359
Concept ID:
C4315392
Congenital Abnormality
Synonym: Brachydactyly, Type E
Modes of inheritance:
Autosomal dominant inheritance
MedGen UID:
141047
Concept ID:
C0443147
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in heterozygotes. In the context of medical genetics, an autosomal dominant disorder is caused when a single copy of the mutant allele is present. Males and females are affected equally, and can both transmit the disorder with a risk of 50% for each child of inheriting the mutant allele.
 
Related gene: HOXD13
 
HPO: HP:0005863
Monarch Initiative: MONDO:0019677
OMIM®: 113300; 142989
Orphanet: ORPHA93387

Definition

Brachydactyly type E comprises one or more shortened metacarpals and metatarsals (summary by Johnson et al., 2003). Another form of brachydactyly type E, BDE2 (613382), is caused by heterozygous mutation in the PTHLH gene (168470) on chromosome 12p11. Also see the hypertension and brachydactyly syndrome (112410). [from OMIM]

Conditions with this feature

Exostoses-anetodermia-brachydactyly type E syndrome
MedGen UID:
338695
Concept ID:
C1851428
Disease or Syndrome
An association reported in a single kindred characterized by the variable presence of the following features: anetodermia (macular atrophy of the skin), multiple exostoses, and brachydactyly type E. There have been no further descriptions in the literature since 1985.
Brachydactyly, type E, with atrial septal defect, type 2
MedGen UID:
354662
Concept ID:
C1862101
Disease or Syndrome
Brachydactyly type E1
MedGen UID:
396291
Concept ID:
C1862102
Finding
Any brachydactyly type E in which the cause of the disease is a mutation in the HOXD13 gene.
Brachydactyly-arterial hypertension syndrome
MedGen UID:
349445
Concept ID:
C1862170
Disease or Syndrome
The hypertension and brachydactyly syndrome (HTNB) is characterized by brachydactyly type E, severe salt-independent but age-dependent hypertension, an increased fibroblast growth rate, neurovascular contact at the rostral-ventrolateral medulla, altered baroreflex blood pressure regulation, and death from stroke before age 50 years when untreated (summary by Maass et al., 2015).
Chromosome 2q37 deletion syndrome
MedGen UID:
419169
Concept ID:
C2931817
Disease or Syndrome
Patients with chromosome 2q37 deletion syndrome show highly variable clinical manifestations likely resulting from different deletion sizes and deletions of different genes. Variable clinical features included brachydactyly type E (BDE), affecting the metacarpals and metatarsals (in about 50% of patients), short stature, mild to moderate intellectual disability, behavioral abnormalities, and dysmorphic facial features. However, many individuals with deletions do not show cognitive deficits (summary by Villavicencio-Lorini et al., 2013, Wheeler et al., 2014, Jean-Marcais et al., 2015).

Professional guidelines

PubMed

Gavril EC, Nucă I, Pânzaru MC, Ivanov AV, Mihai CT, Antoci LM, Ciobanu CG, Rusu C, Popescu R
Genes (Basel) 2023 Feb 11;14(2) doi: 10.3390/genes14020465. PMID: 36833393Free PMC Article
Pereda A, Garin I; Spanish Network for Imprinting Disorders, Perez de Nanclares G
BMC Med Genet 2018 Mar 2;19(1):32. doi: 10.1186/s12881-018-0530-z. PMID: 29499646Free PMC Article

Recent clinical studies

Etiology

Pereda A, Elli FM, Thiele S, de Sanctis L, Rothenbuhler A, Hanna P, Francou B, Ertl DA, Perez de Nanclares G, Linglart A, Mantovani G
Eur J Endocrinol 2021 Feb;184(2):311-320. doi: 10.1530/EJE-20-0625. PMID: 33270042
Maass PG, Aydin A, Luft FC, Schächterle C, Weise A, Stricker S, Lindschau C, Vaegler M, Qadri F, Toka HR, Schulz H, Krawitz PM, Parkhomchuk D, Hecht J, Hollfinger I, Wefeld-Neuenfeld Y, Bartels-Klein E, Mühl A, Kann M, Schuster H, Chitayat D, Bialer MG, Wienker TF, Ott J, Rittscher K, Liehr T, Jordan J, Plessis G, Tank J, Mai K, Naraghi R, Hodge R, Hopp M, Hattenbach LO, Busjahn A, Rauch A, Vandeput F, Gong M, Rüschendorf F, Hübner N, Haller H, Mundlos S, Bilginturan N, Movsesian MA, Klussmann E, Toka O, Bähring S
Nat Genet 2015 Jun;47(6):647-53. Epub 2015 May 11 doi: 10.1038/ng.3302. PMID: 25961942
Wang J, Wang Z, An Y, Wu C, Xu Y, Fu Q, Shen Y, Zhang Q
Clin Chim Acta 2015 Jun 15;446:9-14. Epub 2015 Mar 20 doi: 10.1016/j.cca.2015.03.019. PMID: 25801215
Williams KD, Blangero J, Subedi J, Jha B, Dyer T, Vandeberg JL, Towne B, Williams-Blangero S
Am J Hum Biol 2013 Nov-Dec;25(6):743-50. Epub 2013 Sep 10 doi: 10.1002/ajhb.22441. PMID: 24022874Free PMC Article
Stoll C, Alembik Y
Genet Couns 2004;15(4):421-8. PMID: 15658617

Diagnosis

Gavril EC, Nucă I, Pânzaru MC, Ivanov AV, Mihai CT, Antoci LM, Ciobanu CG, Rusu C, Popescu R
Genes (Basel) 2023 Feb 11;14(2) doi: 10.3390/genes14020465. PMID: 36833393Free PMC Article
Elli FM, Mattinzoli D, Lucca C, Piu M, Maffini MA, Costanza J, Fontana L, Santaniello C, Forino C, Milani D, Bonati MT, Secco A, Gastaldi R, Alfieri C, Messa P, Miozzo M, Arosio M, Mantovani G
J Bone Miner Res 2022 Mar;37(3):465-474. Epub 2022 Jan 17 doi: 10.1002/jbmr.4490. PMID: 34897794Free PMC Article
Libianto R, Wu KH, Devery S, Eisman JA, Center JR
Bone 2019 Jun;123:18-22. Epub 2019 Mar 12 doi: 10.1016/j.bone.2019.03.012. PMID: 30877071
Thomas-Teinturier C, Pereda A, Garin I, Diez-Lopez I, Linglart A, Silve C, de Nanclares GP
Am J Med Genet A 2016 Mar;170(3):734-42. Epub 2015 Dec 6 doi: 10.1002/ajmg.a.37490. PMID: 26640227
Pereda A, Garin I, Garcia-Barcina M, Gener B, Beristain E, Ibañez AM, Perez de Nanclares G
Orphanet J Rare Dis 2013 Sep 12;8:141. doi: 10.1186/1750-1172-8-141. PMID: 24028571Free PMC Article

Therapy

Du H, Gao S, Dong W, Huang Q, Qu H, Zhang C, Guo L, Liu Z, Liu M
J Clin Hypertens (Greenwich) 2024 Mar;26(3):295-298. Epub 2024 Feb 6 doi: 10.1111/jch.14769. PMID: 38319596Free PMC Article
Maass PG, Rump A, Schulz H, Stricker S, Schulze L, Platzer K, Aydin A, Tinschert S, Goldring MB, Luft FC, Bähring S
J Clin Invest 2012 Nov;122(11):3990-4002. Epub 2012 Oct 24 doi: 10.1172/JCI65508. PMID: 23093776Free PMC Article
Pfeiffer RA, Santelmann R
Birth Defects Orig Artic Ser 1977;13(1):319-37. PMID: 322750

Prognosis

Pereda A, Elli FM, Thiele S, de Sanctis L, Rothenbuhler A, Hanna P, Francou B, Ertl DA, Perez de Nanclares G, Linglart A, Mantovani G
Eur J Endocrinol 2021 Feb;184(2):311-320. doi: 10.1530/EJE-20-0625. PMID: 33270042
Li X, Li Z, Chen P, Wang Y, Wang DW, Wang DW
BMC Med Genet 2020 Jul 6;21(1):144. doi: 10.1186/s12881-020-01077-z. PMID: 32631253Free PMC Article
Thomas-Teinturier C, Pereda A, Garin I, Diez-Lopez I, Linglart A, Silve C, de Nanclares GP
Am J Med Genet A 2016 Mar;170(3):734-42. Epub 2015 Dec 6 doi: 10.1002/ajmg.a.37490. PMID: 26640227
Wang J, Wang Z, An Y, Wu C, Xu Y, Fu Q, Shen Y, Zhang Q
Clin Chim Acta 2015 Jun 15;446:9-14. Epub 2015 Mar 20 doi: 10.1016/j.cca.2015.03.019. PMID: 25801215
Stoll C, Alembik Y
Genet Couns 2004;15(4):421-8. PMID: 15658617

Clinical prediction guides

Gavril EC, Nucă I, Pânzaru MC, Ivanov AV, Mihai CT, Antoci LM, Ciobanu CG, Rusu C, Popescu R
Genes (Basel) 2023 Feb 11;14(2) doi: 10.3390/genes14020465. PMID: 36833393Free PMC Article
Pereda A, Garin I; Spanish Network for Imprinting Disorders, Perez de Nanclares G
BMC Med Genet 2018 Mar 2;19(1):32. doi: 10.1186/s12881-018-0530-z. PMID: 29499646Free PMC Article
Thomas-Teinturier C, Pereda A, Garin I, Diez-Lopez I, Linglart A, Silve C, de Nanclares GP
Am J Med Genet A 2016 Mar;170(3):734-42. Epub 2015 Dec 6 doi: 10.1002/ajmg.a.37490. PMID: 26640227
Tülay Koca T, Çiledağ Özdemir F
Eur Rev Med Pharmacol Sci 2015 Dec;19(23):4549-52. PMID: 26698251
Pfeiffer RA, Santelmann R
Birth Defects Orig Artic Ser 1977;13(1):319-37. PMID: 322750

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