MCID: BFD001
MIFTS: 25

Bifid Nose

Categories: Bone diseases, Fetal diseases, Rare diseases, Smell/Taste diseases

Aliases & Classifications for Bifid Nose

MalaCards integrated aliases for Bifid Nose:

Name: Bifid Nose 54 60
Median Fissure of Nose 54
Nose, Median Cleft of 54

Characteristics:

Orphanet epidemiological data:

60
bifid nose
Inheritance: Autosomal dominant,Autosomal recessive; Age of onset: Antenatal,Neonatal;

Classifications:



External Ids:

MESH via Orphanet 46 C535441
ICD10 via Orphanet 35 Q30.2
UMLS via Orphanet 75 C0221363
Orphanet 60 ORPHA2695
SNOMED-CT via HPO 70 194021007 204521002 22006008

Summaries for Bifid Nose

NIH Rare Diseases : 54 A bifid nose is a relatively uncommon malformation that is characterized by the nose being divided into two parts. There is a large degree of variability in the severity of the condition, ranging from a minimally noticeable groove down the center of the nasal tip to a complete clefting of the underlying bones and cartilage, resulting in 2 complete half noses. It is often associated with hypertelorbitism and midline clefts of the lip. The airway usually is adequate despite the cosmetic appearance associated with the condition. Both autosomal recessive and autosomal dominant inheritance of a bifid nose has been observed. It may also occur with frontonasal dysplasia (a condition in with several possible findings limited to the head and neck), for which several inheritance patterns have been reported. Treatment typically consists of surgical reconstruction to repair the malformation.

MalaCards based summary : Bifid Nose, also known as median fissure of nose, is related to bifid nose with or without anorectal and renal anomalies and trigonobrachycephaly, bulbous bifid nose, micrognathia, and abnormalities of the hands and feet. An important gene associated with Bifid Nose is FREM1 (FRAS1 Related Extracellular Matrix 1). The drugs Racepinephrine and Epinephrine have been mentioned in the context of this disorder. Affiliated tissues include bone, and related phenotypes are bifid nose and hypertelorism

Related Diseases for Bifid Nose

Graphical network of the top 20 diseases related to Bifid Nose:



Diseases related to Bifid Nose

Symptoms & Phenotypes for Bifid Nose

Human phenotypes related to Bifid Nose:

60 33
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 bifid nose 60 33 hallmark (90%) Very frequent (99-80%) HP:0011803
2 hypertelorism 60 33 occasional (7.5%) Occasional (29-5%) HP:0000316

Drugs & Therapeutics for Bifid Nose

Drugs for Bifid Nose (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):


# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Racepinephrine Approved Not Applicable 329-65-7 838
2
Epinephrine Approved, Vet_approved Not Applicable 51-43-4 5816
3 Anesthetics Not Applicable
4 Epinephryl borate Not Applicable

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Objective Assessment for Cleft Lip Nasal Deformity Correction Not yet recruiting NCT03683199 Not Applicable

Search NIH Clinical Center for Bifid Nose

Genetic Tests for Bifid Nose

Anatomical Context for Bifid Nose

MalaCards organs/tissues related to Bifid Nose:

42
Bone

Publications for Bifid Nose

Articles related to Bifid Nose:

(show all 21)
# Title Authors Year
1
Open Rhinoplasty Using Concealing Incisions for Mild Bifid Nose With Unilateral Mini-Microform Cleft Lip. ( 29543682 )
2018
2
Surgical correction of bifid nose. ( 27260584 )
2016
3
Midline Cleft Lip and Bifid Nose Deformity: Description, Classification, and Treatment. ( 26594965 )
2015
4
Novel FREM1 mutations expand the phenotypic spectrum associated with Manitoba-oculo-tricho-anal (MOTA) syndrome and bifid nose renal agenesis anorectal malformations (BNAR) syndrome. ( 23401257 )
2013
5
Bifid nose - a mild degree of frontonasal dysplasia. A case report. ( 23810549 )
2013
6
Repair of a bifid nose combined with a cleft of the primary palate in a 1-year-old dog. ( 22380669 )
2011
7
FREM1 mutations cause bifid nose, renal agenesis, and anorectal malformations syndrome. ( 19732862 )
2009
8
Open approach via the nasal dorsum with dermal graft for bifid nose. ( 19816255 )
2009
9
Pai syndrome: a further report of a case with bifid nose, lipoma, and agenesis of the corpus callosum. ( 18369643 )
2008
10
Bifid nose with cleft hand deformity: syndromic association or undescribed anomaly? ( 19098558 )
2008
11
Combined intraoral and nasal approach to Tessier No:0 cleft with bifid nose. ( 15655475 )
2005
12
Pai syndrome: an adult patient with bifid nose and frontal hairline marker. ( 12733964 )
2003
13
Midline cleft. Treatment of the bifid nose. ( 10937104 )
1999
14
Trigonobrachycephaly, bulbous bifid nose, macrostomia, micrognathia, acral anomalies, and hypotonia in sibs. ( 2063891 )
1991
15
Dominant inheritance of bifid nose. ( 6711607 )
1984
16
Epignathus and bifid nose: a report of a rare congenital anomaly. ( 4726107 )
1973
17
Hypertelorism and the bifid nose. ( 5935148 )
1966
18
The bifid nose. With a report of 3 cases in siblings. ( 5845694 )
1965
19
Treatment of median cleft lip associated with bifid nose and hypertelorism. ( 14405033 )
1959
20
The surgical treatment of the bifid nose. ( 15430171 )
1950
21
Bifid nose associated with midline cleft of the upper lip. ( 21002094 )
1946

Variations for Bifid Nose

Expression for Bifid Nose

Search GEO for disease gene expression data for Bifid Nose.

Pathways for Bifid Nose

GO Terms for Bifid Nose

Sources for Bifid Nose

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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