MCID: MNR004
MIFTS: 23

Mounier-Kuhn Syndrome

Categories: Rare diseases, Respiratory diseases

Aliases & Classifications for Mounier-Kuhn Syndrome

MalaCards integrated aliases for Mounier-Kuhn Syndrome:

Name: Mounier-Kuhn Syndrome 52
Congenital Tracheobronchomegaly 52 71
Mounier Kuhn Syndrome 52
Tracheobronchomegaly 71

Classifications:



External Ids:

SNOMED-CT via HPO 68 11296007 233604007 32398004
UMLS 71 C0040587 C2713583

Summaries for Mounier-Kuhn Syndrome

NIH Rare Diseases : 52 Mounier-Kuhn syndrome is a lung disorder that causes the respiratory tract to dilate or enlarge. People with this condition develop frequent respiratory tract infections and recurrent cough. The condition can be diagnosed by lung function tests , bronchoscopy , and a chest CT scan . The cause of Mounier-Kuhn syndrome is unknown, although cigarette smoke and air pollutants may act as irritating factors. Some cases are associated with connective tissue diseases such as Ehlers-Danlos syndrome , Marfan syndrome , and cutis laxa and may be inherited . Treatment typically involves chest physical therapy and antibiotics to treat infections.

MalaCards based summary : Mounier-Kuhn Syndrome, also known as congenital tracheobronchomegaly, is related to tracheobronchomegaly and bronchiectasis. Affiliated tissues include lung, trachea and testes, and related phenotypes are tracheal stenosis and pneumonia

Wikipedia : 74 Tracheobronchomegaly is a very rare congenital disorder of the lung primarily characterized by an... more...

Related Diseases for Mounier-Kuhn Syndrome

Graphical network of the top 20 diseases related to Mounier-Kuhn Syndrome:



Diseases related to Mounier-Kuhn Syndrome

Symptoms & Phenotypes for Mounier-Kuhn Syndrome

Human phenotypes related to Mounier-Kuhn Syndrome:

31
# Description HPO Frequency HPO Source Accession
1 tracheal stenosis 31 hallmark (90%) HP:0002777
2 pneumonia 31 hallmark (90%) HP:0002090
3 bronchitis 31 hallmark (90%) HP:0012387
4 recurrent bronchopulmonary infections 31 hallmark (90%) HP:0006538
5 tracheobronchmegaly 31 hallmark (90%) HP:0010776

Drugs & Therapeutics for Mounier-Kuhn Syndrome

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Evaluation of the Presence of Airway Collapse in Patients With Mounier-Kuhn Syndrome and Titration With Continuous Positive Pressure Through Non-invasive Mechanical Ventilation to Reduce Collapse Unknown status NCT03101059

Search NIH Clinical Center for Mounier-Kuhn Syndrome

Genetic Tests for Mounier-Kuhn Syndrome

Anatomical Context for Mounier-Kuhn Syndrome

MalaCards organs/tissues related to Mounier-Kuhn Syndrome:

40
Lung, Trachea, Testes, Smooth Muscle

Publications for Mounier-Kuhn Syndrome

Articles related to Mounier-Kuhn Syndrome:

(show top 50) (show all 162)
# Title Authors PMID Year
1
Mounier-Kuhn Syndrome. 61
30777312 2019
2
Tracheobronchoplasty after a trial with a silicone Y-stent in a patient with Mounier-Kuhn syndrome. 61
31747002 2019
3
Airway management of a patient incidentally diagnosed with Mounier-Kuhn syndrome during general anesthesia. 61
31723671 2019
4
Orphan Lung Diseases. 61
30955517 2019
5
[Mounier-Kuhn syndrome]. 61
31076098 2019
6
Mounier-Kuhn syndrome: an unusual cause of bronchiectasis. 61
31019346 2019
7
Mounier-Kuhn syndrome. 61
31565119 2019
8
An Unusual Cause of Spontaneous Pneumomediastinum: The Mounier-Kuhn Syndrome. 61
31360573 2019
9
Mounier Kuhn syndrome presenting with recurrent atelectasis. 61
28891754 2018
10
An unusual cause of bronchial dilation: Mounier-Kuhn syndrome. 61
29894236 2018
11
Flexible bronchoscopy and mechanical ventilation in managing Mounier-Kuhn syndrome: a case report. 61
28443953 2018
12
Mounier-Kuhn syndrome (tracheobronchomegaly): An analysis of eleven cases. 61
28026118 2018
13
Mounier-Kuhn Syndrome Mimicking Lymphangioleiomyomatosis. 61
29406234 2018
14
Tracheal enlargement or Mounier-Kuhn syndrome in giant cell arteritis: a possible causal association with therapeutic implications. 61
30173269 2018
15
Mounier-Kuhn syndrome. 61
28596443 2017
16
Dural ectasia associated with Mounier-Kuhn syndrome: a novel association in the context of spinal stenosis. 61
28855219 2017
17
A case of tracheobronchomegaly. 61
28109701 2017
18
First Case Report of Metastatic Squamous Cell Carcinoma of Lung Associated with Mounier-Kuhn Syndrome and Review of Literature. 61
28845373 2017
19
Congenital Tracheobronchomegaly (Mounier-Kuhn Syndrome) in a Woman with Human Immunodeficiency Virus: A Case Report. 61
28480154 2017
20
Mounier-Kuhn syndrome: a case of tracheal smooth muscle remodeling. 61
28174630 2017
21
Anesthesia for a patient with unexpected giant tracheobronchomegaly. 61
28757767 2017
22
An Atypical Case of Mounier-Kuhn Syndrome: Time to Change the Diagnostic Approach? 61
27367849 2017
23
The Association of Mounier-Kuhn Syndrome and Pulmonary Aspergillomas: A Case Report. 61
27761295 2016
24
Tracheobronchomegaly associated with laryngo-tracheal amyloidosis: First case report. 61
26791590 2016
25
An unusual cause of recurrent spontaneous pneumothorax: the Mounier-Kuhn syndrome. 61
26127019 2016
26
Respiratory Conditions Associated with Tracheobronchomegaly (Mounier-Kuhn Syndrome): A Study of Seventeen Cases. 61
27022925 2016
27
Mounier-Kuhn Syndrome in an Elderly Female with Pulmonary Fibrosis. 61
27597867 2016
28
Tracheobronchomegaly as a Cause of Bronchiectasis in an Adult. 61
27022496 2016
29
Mounier-Kuhn syndrome: a systematic analysis of 128 cases published within last 25 years. 61
25130790 2016
30
Huge Tracheal Diverticulum in a Patient with Mounier-Kuhn Syndrome. 61
30755880 2016
31
A Clinical Classification Scheme for Tracheobronchomegaly (Mounier-Kuhn Syndrome). 61
26189148 2015
32
Coincidence of Tracheobronchomegaly (Mounier-Kuhn Syndrome) and Juvenile Idiopathic Arthritis. 61
26038962 2015
33
Clinical-radiological, histological and genetic analyses in a lung transplant recipient with Mounier-Kuhn syndrome and end-stage chronic obstructive pulmonary disease. 61
24725636 2015
34
Mounier-Kuhn Syndrome. 61
30394453 2015
35
[Tracheobronchial prosthesis in Mounier-Kuhn syndrome: New perspectives]. 61
25737189 2015
36
A 54-Year-Old Man with Tracheomegaly, Tracheal Diverticulas and Bronchiectasis--Mounier-Kuhn Syndrome. 61
26591972 2015
37
Anesthetic considerations for bilateral lung transplantation in Mounier-Kuhn syndrome. 61
24529664 2015
38
Mounier-Kuhn syndrome (MKS) - Pathognomonic Findings. 61
25654012 2014
39
The man with the large trachea: Mounier-Kuhn syndrome. 61
25004454 2014
40
Mounier-Kuhn syndrome: more than just a cough. 61
25213787 2014
41
Mounier-Kuhn syndrome. Imaging and bronchoscopic findings. 61
24983230 2014
42
A delayed diagnosis of Mounier-Kuhn syndrome. 61
24891495 2014
43
Mounier-Kuhn syndrome presenting as cough. 61
24787068 2014
44
[Mounier-Kuhn syndrome - a rare cause of recurrent bronchitis]. 61
24668436 2014
45
Mounier-Kuhn syndrome. 61
24739689 2014
46
A grossly abnormal trachea- severe tracheal diverticulosis and Mounier-Kuhn syndrome. 61
24757893 2014
47
Mounier-Kuhn syndrome masquerading pulmonary thromboembolism in an elderly male. 61
24669091 2014
48
Recurrent pneumonia as a result of Mounier-Kuhn syndrome. 61
24401975 2014
49
Tracheomalacia treatment using a large-diameter, custom-made airway stent in a case with mounier-kuhn syndrome. 61
25276462 2014
50
Mounier-Kuhn syndrome: a rare and often overlooked cause of bronchial dilation and recurrent respiratory tract infections. 61
24626279 2014

Variations for Mounier-Kuhn Syndrome

Expression for Mounier-Kuhn Syndrome

Search GEO for disease gene expression data for Mounier-Kuhn Syndrome.

Pathways for Mounier-Kuhn Syndrome

GO Terms for Mounier-Kuhn Syndrome

Sources for Mounier-Kuhn Syndrome

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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