TEF
MCID: TRC040
MIFTS: 45

Tracheoesophageal Fistula (TEF) malady

Categories: Rare diseases

Aliases & Classifications for Tracheoesophageal Fistula

Aliases & Descriptions for Tracheoesophageal Fistula:

Name: Tracheoesophageal Fistula 50 29 52
Tracheoesophageal Fistula with or Without Esophageal Atresia 50
Te Fistula 50
Tef 50

Classifications:



Summaries for Tracheoesophageal Fistula

NIH Rare Diseases : 50 tracheoesophageal fistula (tef) is a life-threatening condition in which there is an abnormal connection between the esophagus and trachea (windpipe). the esophagus and trachea run next to each other through the chest cavity. the esophagus carries food and saliva to the stomach, while the trachea carries air to the lungs. tef can lead to severe and fatal lung complications. saliva and gastric secretions can be aspirated into the lungs, and normal swallowing and digestion of food cannot occur. most affected people are diagnosed immediately after birth or during infancy. symptoms may include frothy bubbles of mucus in the mouth and nose; episodes of coughing and choking; and worsening symptoms during feeding. tef may be isolated, or it may occur with other physical or developmental abnormalities (most commonly, esophageal atresia). in many cases the cause is unknown but it has been associated with some chromosome disorders. in some cases it may be acquired later in life after a cancer, infection, ruptured diverticula, or trauma. treatment includes immediate surgical repair with survival rates of almost 100%. last updated: 7/8/2015

MalaCards based summary : Tracheoesophageal Fistula, also known as tracheoesophageal fistula with or without esophageal atresia, is related to esophageal atresia/tracheoesophageal fistula and pancreatic hypoplasia, intestinal atresia, and gallbladder aplasia or hypoplasia, with or without tracheoesophageal fistula. An important gene associated with Tracheoesophageal Fistula is SHH (Sonic Hedgehog), and among its related pathways/superpathways are Fanconi anemia pathway and Differentiation Pathway. The drug threonine has been mentioned in the context of this disorder. Affiliated tissues include trachea, lung and thyroid, and related phenotypes are cellular and embryo

Wikipedia : 71 A tracheoesophageal fistula (TEF, or TOF; see spelling differences) is an abnormal connection (fistula)... more...

Related Diseases for Tracheoesophageal Fistula

Diseases related to Tracheoesophageal Fistula via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 108)
id Related Disease Score Top Affiliating Genes
1 esophageal atresia/tracheoesophageal fistula 12.5
2 pancreatic hypoplasia, intestinal atresia, and gallbladder aplasia or hypoplasia, with or without tracheoesophageal fistula 12.0
3 tracheoesophageal fistula with or without esophageal atresia 12.0
4 tracheoesophageal fistula symphalangism 12.0
5 feingold syndrome 11.3
6 vacterl association, x-linked 11.2
7 vater/vacterl association 11.2
8 vater association with macrocephaly and ventriculomegaly 11.2
9 fanconi anemia, complementation group b 11.0
10 mitchell-riley syndrome 11.0
11 feingold syndrome 2 11.0
12 esophagitis 10.9
13 esophageal atresia 10.8
14 laryngitis 10.1
15 duodenitis 10.1
16 pulmonary atresia with intact ventricular septum 10.1 CHD7 SHH
17 anosmia, isolated congenital 10.1 GLI3 SHH
18 tracheal stenosis 10.1
19 lymphoma 10.1
20 duodenal atresia 10.1
21 tracheomalacia 10.1
22 polydactyly, postaxial, types a1 and b 10.1 GLI3 SHH
23 extragonadal germ cell cancer 10.1 GLI3 SHH
24 esophageal cancer 10.1
25 breasts and/or nipples, aplasia or hypoplasia of, 2 10.0 GLI3 SHH
26 congenital diaphragmatic hernia 10.0
27 tracheal agenesis 10.0
28 cervicitis 10.0
29 aica-ribosiduria due to atic deficiency 10.0 GLI3 NOG
30 hypoparathyroidism 10.0 GLI3 SHH
31 horner's syndrome 10.0 GLI3 SHH
32 t cell immunodeficiency primary 10.0 GLI3 NOG
33 lung cancer 10.0
34 hypertrophic pyloric stenosis 10.0
35 pyloric stenosis 10.0
36 mediastinitis 10.0
37 right aortic arch 10.0
38 bone structure disease 9.9 GLI3 NOG
39 glanders 9.9 FANCI FANCL
40 imperforate anus 9.9
41 respiratory failure 9.9
42 laryngeal cleft 9.9
43 thyroiditis 9.9
44 gastroesophageal reflux 9.9
45 fibrous dysplasia 9.9 GLI3 SHH
46 atrophy of testis 9.9 GLI3 NOG
47 microcephaly 9.8
48 heart disease 9.8
49 aspiration pneumonia 9.8
50 congenital tracheal stenosis 9.8

Graphical network of the top 20 diseases related to Tracheoesophageal Fistula:



Diseases related to Tracheoesophageal Fistula

Symptoms & Phenotypes for Tracheoesophageal Fistula

MGI Mouse Phenotypes related to Tracheoesophageal Fistula:

44 (show all 13)
id Description MGI Source Accession Score Top Affiliating Genes
1 cellular MP:0005384 9.92 CHD7 NOG FANCL GLI3 SHH
2 embryo MP:0005380 9.88 FANCL SHH GLI3 CHD7 NOG
3 endocrine/exocrine gland MP:0005379 9.85 FANCL SHH GLI3 CHD7 NOG
4 mortality/aging MP:0010768 9.85 NOG FANCI FANCL SHH GLI3 CHD7
5 craniofacial MP:0005382 9.81 NOG SHH GLI3 CHD7
6 digestive/alimentary MP:0005381 9.8 CHD7 NOG SHH GLI3
7 hearing/vestibular/ear MP:0005377 9.73 SHH GLI3 CHD7 NOG
8 reproductive system MP:0005389 9.72 FANCL SHH GLI3 CHD7 NOG
9 limbs/digits/tail MP:0005371 9.71 NOG SHH GLI3 CHD7
10 respiratory system MP:0005388 9.56 NOG SHH GLI3 CHD7
11 skeleton MP:0005390 9.55 FANCL SHH GLI3 CHD7 NOG
12 taste/olfaction MP:0005394 9.13 SHH GLI3 NOG
13 vision/eye MP:0005391 8.92 CHD7 NOG GLI3 SHH

Drugs & Therapeutics for Tracheoesophageal Fistula

Drugs for Tracheoesophageal Fistula (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):


id Name Status Phase Clinical Trials Cas Number PubChem Id
1 threonine Nutraceutical

Interventional clinical trials:


id Name Status NCT ID Phase
1 Endoscopic Closure of Tracheoesophageal Fistulas With Occluder Device Terminated NCT01153061 Phase 4
2 Open or Keyhole Surgery Through the Chest for Newborn Babies: Effect on Blood Gases Unknown status NCT01467245
3 Trichloroacetic Acid for Endoscopic Tracho-esophageal Fistula Repair Unknown status NCT01287312
4 Chewing in Children With Repaired Esophageal Atresia-tracheoesophageal Fistula Completed NCT03026491
5 National Register of Oesophageal Atresia Recruiting NCT02883725
6 Prospective Data Collection of Patients < 6 Months of Age Undergoing Thoracoscopic Surgery Enrolling by invitation NCT02033772
7 A Physiological Study to Determine the Enteral Threonine Requirements in Infants Aged 1 to 6 Months Enrolling by invitation NCT02364843

Search NIH Clinical Center for Tracheoesophageal Fistula

Genetic Tests for Tracheoesophageal Fistula

Genetic tests related to Tracheoesophageal Fistula:

id Genetic test Affiliating Genes
1 Tracheoesophageal Fistula 29

Anatomical Context for Tracheoesophageal Fistula

MalaCards organs/tissues related to Tracheoesophageal Fistula:

39
Trachea, Lung, Thyroid, Colon, Bone, Heart, Testes

Publications for Tracheoesophageal Fistula

Articles related to Tracheoesophageal Fistula:

(show top 50) (show all 621)
id Title Authors Year
1
Oesophageal atresia with no distal tracheoesophageal fistula: Management and outcomes from a population-based cohort. ( 27894760 )
2017
2
Esophageal Atresia with or without Tracheoesophageal Fistula (EA/TEF): Association of Different EA/TEF Subtypes with Specific Co-occurring Congenital Anomalies and Implications for Diagnostic Workup. ( 28061520 )
2017
3
Ineffective Ventilation in A Neonate with A Large Pre-Carinal Tracheoesophageal Fistula and Bilateral Pneumonitis-Microcuff Endotracheal Tube to Our Rescue! ( 28083500 )
2017
4
Preliminary Study of Video-Based Pediatric Endoscopic Surgical Skill Assessment Using a Neonatal Esophageal Atresia/Tracheoesophageal Fistula Model. ( 27858521 )
2017
5
Congenital bilateral syngnathia and tracheoesophageal fistula: A rare presentation. ( 27994431 )
2016
6
Dual Use of Fiberoptic Bronchoscope in TracheoEsophageal Fistula. ( 28043317 )
2016
7
Spontaneous Resolution of a Tracheoesophageal Fistula Caused by Button Battery Ingestion. ( 27195748 )
2016
8
Esophageal atresia with tracheoesophageal fistula in a patient with 7q35-36.3 deletion including SHH gene. ( 27614115 )
2016
9
Perioperative management and outcomes of esophageal atresia and tracheoesophageal fistula. ( 27993359 )
2016
10
Evaluation of the intraoperative risk factors for esophageal anastomotic complications after primary repair of esophageal atresia with tracheoesophageal fistula. ( 27461430 )
2016
11
Tracheoesophageal fistula repair in a neonate with tetralogy of Fallot: An anesthetic challenge. ( 27625509 )
2016
12
Tracheoesophageal fistula induced by invasive pulmonary aspergillosis. ( 27761449 )
2016
13
Thoracoscopic repair of esophageal atresia with tracheoesophageal fistula: Basics of technique and its nuances. ( 27365905 )
2016
14
Thoracoscopic Esophageal Atresia with Tracheoesophageal Fistula Repair: The First Iranian Group Report, Passing the Learning Curve. ( 27471677 )
2016
15
Accuracy of prenatal detection of tracheoesophageal fistula and oesophageal atresia. ( 26932255 )
2016
16
Presentation of H-Type Tracheoesophageal Fistula in Two Adolescents: Delayed Diagnosis. ( 27324122 )
2016
17
Tracheoesophageal fistula. ( 27301604 )
2016
18
The use of a sternothyroid muscle flap to prevent the re-recurrence of a recurrent tracheoesophageal fistula found 10A years after the primary repair. ( 27589983 )
2016
19
Weekday vs. weekend repair of esophageal atresia and tracheoesophageal fistula. ( 26932247 )
2016
20
Late Complication of Esophageal Atresia/Tracheoesophageal Fistula Repair. ( 27139297 )
2016
21
Late presenting iatrogenic tracheoesophageal fistula. ( 26936836 )
2016
22
Left thoracoscopic two-stage repair of tracheoesophageal fistula with a right aortic arch and a vascular ring. ( 27143697 )
2016
23
Validation of an inanimate low cost model for training minimal invasive surgery (MIS) of esophageal atresia with tracheoesophageal fistula (AE/TEF) repair. ( 27530889 )
2016
24
Swallowing Dysfunction and Quality of Life in Adults With Surgically Corrected Esophageal Atresia/Tracheoesophageal Fistula as Infants: Forty Years of Follow-up. ( 27607100 )
2016
25
Acquired tracheoesophageal fistula with undiagnosed tracheal stenosis: A simple innovation to avoid tracheostomy! ( 27942069 )
2016
26
Assessment of Clinical Symptoms and Multichannel Intraluminal Impedance and pH Monitoring in Children After Thoracoscopic Repair of Esophageal Atresia and Distal Tracheoesophageal Fistula. ( 28028956 )
2016
27
Cervical repair of congenital tracheoesophageal fistula: Complications lurking! ( 27372236 )
2016
28
Functional residual capacity and lung clearance index in infants treated for esophageal atresia and tracheoesophageal fistula. ( 26607967 )
2016
29
Effect of cauterizing esophageal mucosa in "double-patch" treatment for acquired benign tracheoesophageal fistula/bronchogastric stump fistula. ( 28032545 )
2016
30
Outcomes of thoracoscopy versus thoracotomy for esophageal atresia with tracheoesophageal fistula repair: A PRISMA-compliant systematic review and meta-analysis. ( 27472740 )
2016
31
Monitoring pre- and postductal saturation - A must for neonates undergoing tracheoesophageal fistula repair. ( 26725993 )
2016
32
Middle Cerebral Artery Stroke in a Neonate With a Congenital Hypercoagulable Condition Following Repair of an H-type Tracheoesophageal Fistula. ( 27164518 )
2016
33
Pedicled posterior pericardial repair of tracheoesophageal fistula after chemoradiotherapy for esophageal cancer. ( 26948167 )
2016
34
Successful Treatment of a Recurrent WideA Tracheoesophageal Fistula With aA Bioabsorbable Patch. ( 27106472 )
2016
35
Esophageal Microperforation due to Calcified Mediastinal Lymph Node Leading to Tracheoesophageal Fistula. ( 27366334 )
2016
36
Anesthetic Management of a Tracheoesophageal Fistula in a Patient With a Large Uncorrected Aortopulmonary Window. ( 27941485 )
2016
37
Congenital H-type tracheoesophageal fistula: a national multicenter study. ( 26852298 )
2016
38
Tracheoesophageal fistula length decreases over time. ( 26951219 )
2016
39
Copy number variations in 375 patients with oesophageal atresia and/or tracheoesophageal fistula. ( 27436264 )
2016
40
Intraoperative ventilatory management of adult tracheoesophageal fistula. ( 27654741 )
2016
41
Esophageal Atresia with Tracheoesophageal Fistula. ( 27387366 )
2016
42
Categorization and repair of recurrent and acquired tracheoesophageal fistulae occurring after esophageal atresia repair. ( 27616617 )
2016
43
Double Tracheoesophageal Fistula and Azygos Lobe. ( 27251121 )
2016
44
Management of Acquired Tracheoesophageal Fistula Complicating Endoscopic Management for Corrosive Esophageal Stricture. ( 26802853 )
2016
45
Vertebral defect, anal atresia, cardiac defect, tracheoesophageal fistula/esophageal atresia, renal defect, and limb defect association with Mayer-Rokitansky-KA1ster-Hauser syndrome in co-occurrence: two case reports and a review of the literature. ( 28003020 )
2016
46
Combined tracheoinnominate artery fistula and tracheoesophageal fistula: A very rare complication of indwelling tracheostomy tube. ( 27051118 )
2016
47
Endoscopic treatment of recurrent tracheoesophageal fistula with a combination of N-butyl-2-cyanocrylate (Histoacryl(Ar)) and lipiodol via esophagoscopy in a pediatric patient. ( 26817390 )
2016
48
Tracheoesophageal fistula: open versus endoscopic repair. ( 27653493 )
2016
49
ESPGHAN-NASPGHAN Guidelines for the Evaluation and Treatment of Gastrointestinal and Nutritional Complications in Children with Esophageal Atresia- Tracheoesophageal Fistula. ( 27579697 )
2016
50
Determinants of gap length in esophageal atresia with tracheoesophageal fistula and the impact of gap length on outcome. ( 27365907 )
2016

Variations for Tracheoesophageal Fistula

Expression for Tracheoesophageal Fistula

Search GEO for disease gene expression data for Tracheoesophageal Fistula.

Pathways for Tracheoesophageal Fistula

Pathways related to Tracheoesophageal Fistula according to GeneCards Suite gene sharing:

id Super pathways Score Top Affiliating Genes
1 11.13 FANCI FANCL
2 10.64 NOG SHH
3 9.58 GLI3 SHH

GO Terms for Tracheoesophageal Fistula

Biological processes related to Tracheoesophageal Fistula according to GeneCards Suite gene sharing:

(show all 50)
id Name GO ID Score Top Affiliating Genes
1 positive regulation of transcription from RNA polymerase II promoter GO:0045944 9.98 CHD7 GLI3 NOG SHH
2 regulation of cell proliferation GO:0042127 9.81 FANCL GLI3 SHH
3 in utero embryonic development GO:0001701 9.8 CHD7 GLI3 NOG
4 axon guidance GO:0007411 9.78 GLI3 NOG SHH
5 negative regulation of canonical Wnt signaling pathway GO:0090090 9.73 GLI3 NOG SHH
6 kidney development GO:0001822 9.71 GLI3 SHH
7 negative regulation of cell migration GO:0030336 9.71 NOG SHH
8 lung development GO:0030324 9.7 GLI3 SHH
9 anterior/posterior pattern specification GO:0009952 9.7 GLI3 SHH
10 wound healing GO:0042060 9.7 GLI3 NOG
11 forebrain development GO:0030900 9.7 GLI3 SHH
12 smoothened signaling pathway GO:0007224 9.69 GLI3 SHH
13 odontogenesis of dentin-containing tooth GO:0042475 9.69 GLI3 SHH
14 embryonic limb morphogenesis GO:0030326 9.69 GLI3 SHH
15 palate development GO:0060021 9.69 CHD7 GLI3 SHH
16 inner ear development GO:0048839 9.68 GLI3 SHH
17 interstrand cross-link repair GO:0036297 9.68 FANCI FANCL
18 neural tube development GO:0021915 9.68 GLI3 NOG
19 branching involved in ureteric bud morphogenesis GO:0001658 9.67 GLI3 SHH
20 embryonic skeletal system development GO:0048706 9.67 NOG SHH
21 embryonic organ development GO:0048568 9.67 GLI3 SHH
22 oligodendrocyte differentiation GO:0048709 9.66 GLI3 SHH
23 metanephros development GO:0001656 9.66 GLI3 SHH
24 neuron fate commitment GO:0048663 9.65 GLI3 SHH
25 T cell differentiation in thymus GO:0033077 9.65 GLI3 SHH
26 developmental growth GO:0048589 9.65 GLI3 SHH
27 branching morphogenesis of an epithelial tube GO:0048754 9.64 GLI3 SHH
28 embryonic hindlimb morphogenesis GO:0035116 9.64 CHD7 SHH
29 camera-type eye development GO:0043010 9.63 CHD7 GLI3 SHH
30 spinal cord motor neuron differentiation GO:0021522 9.62 GLI3 SHH
31 positive regulation of neuroblast proliferation GO:0002052 9.62 GLI3 SHH
32 lung morphogenesis GO:0060425 9.61 NOG SHH
33 negative regulation of cell differentiation GO:0045596 9.61 GLI3 NOG SHH
34 embryonic digestive tract morphogenesis GO:0048557 9.6 GLI3 SHH
35 positive regulation of protein import into nucleus GO:0042307 9.59 GLI3 SHH
36 somite development GO:0061053 9.58 NOG SHH
37 anatomical structure development GO:0048856 9.58 GLI3 SHH
38 embryonic digit morphogenesis GO:0042733 9.58 GLI3 NOG SHH
39 embryonic morphogenesis GO:0048598 9.57 GLI3 SHH
40 negative thymic T cell selection GO:0045060 9.55 GLI3 SHH
41 positive regulation of alpha-beta T cell differentiation GO:0046638 9.54 GLI3 SHH
42 artery development GO:0060840 9.52 GLI3 SHH
43 pattern specification process GO:0007389 9.5 GLI3 NOG SHH
44 spinal cord dorsal/ventral patterning GO:0021513 9.49 GLI3 SHH
45 hindgut morphogenesis GO:0007442 9.48 GLI3 SHH
46 central nervous system development GO:0007417 9.46 CHD7 GLI3 NOG SHH
47 dorsal/ventral pattern formation GO:0009953 9.43 GLI3 NOG SHH
48 negative regulation of alpha-beta T cell differentiation GO:0046639 9.37 GLI3 SHH
49 anatomical structure formation involved in morphogenesis GO:0048646 9.13 GLI3 NOG SHH
50 limb development GO:0060173 8.92 CHD7 GLI3 NOG SHH

Sources for Tracheoesophageal Fistula

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 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 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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