MCID: BFD001
MIFTS: 23

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 53 59
Median Fissure of Nose 53
Nose, Median Cleft of 53

Characteristics:

Orphanet epidemiological data:

59
bifid nose
Inheritance: Autosomal dominant,Autosomal recessive; Age of onset: Antenatal,Neonatal;

Classifications:



External Ids:

Orphanet 59 ORPHA2695
MESH via Orphanet 45 C535441
UMLS via Orphanet 74 C0221363
ICD10 via Orphanet 34 Q30.2
SNOMED-CT via HPO 69 194021007 22006008 204521002

Summaries for Bifid Nose

NIH Rare Diseases : 53 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 skin, and related phenotypes are hypertelorism and bifid nose

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:

59 32
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 hypertelorism 59 32 occasional (7.5%) Occasional (29-5%) HP:0000316
2 bifid nose 59 32 hallmark (90%) Very frequent (99-80%) HP:0011803

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 Epinephryl borate Not Applicable
4 Anesthetics 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:

41
Bone, Skin

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 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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