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MCID: MNR004
MIFTS: 23

Mounier-Kuhn Syndrome

Categories: Rare diseases, Respiratory diseases
Tissues Related diseases Publications Symptoms & Phenotypes
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Aliases & Classifications for Mounier-Kuhn Syndrome

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MalaCards integrated aliases for Mounier-Kuhn Syndrome:

Name: Mounier-Kuhn Syndrome 20
Congenital Tracheobronchomegaly 20 71
Mounier Kuhn Syndrome 20
Tracheobronchomegaly 71

Classifications:

MalaCards categories:
Global: Rare diseases
Anatomical: Respiratory diseases
See all MalaCards categories (disease lists)


External Ids:

SNOMED-CT via HPO 68 11296007 233604007 32398004
UMLS 71 C0040587 C2713583
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Summaries for Mounier-Kuhn Syndrome

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GARD : 20 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 smooth muscle, and related phenotypes are pneumonia and tracheal stenosis

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

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Related Diseases for Mounier-Kuhn Syndrome

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Diseases related to Mounier-Kuhn Syndrome via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 42)
# Related Disease Score Top Affiliating Genes
1 tracheobronchomegaly 11.5
2 bronchiectasis 10.9
3 respiratory failure 10.6
4 pulmonary fibrosis 10.6
5 tracheomalacia 10.5
6 bronchitis 10.5
7 tracheobronchomalacia 10.5
8 pneumothorax 10.5
9 sleep apnea 10.4
10 pneumothorax, primary spontaneous 10.3
11 pulmonary disease, chronic obstructive 10.3
12 tracheal stenosis 10.3
13 pleomorphic carcinoma 10.3
14 connective tissue disease 10.3
15 syncope 10.3
16 gastroesophageal reflux 10.2
17 fibrosis of extraocular muscles, congenital, 1 10.2
18 immune deficiency disease 10.2
19 lymphangioleiomyomatosis 10.2
20 spermatogenic failure 3 10.2
21 ptosis 10.2
22 covid-19 10.2
23 kenny-caffey syndrome 10.2
24 squamous cell carcinoma 10.2
25 malignant fibrous histiocytoma 10.2
26 relapsing polychondritis 10.2
27 larynx cancer 10.2
28 pulmonary tuberculosis 10.2
29 cutis laxa 10.2
30 aspiration pneumonitis 10.2
31 sarcomatoid carcinoma 10.2
32 systemic scleroderma 10.2
33 fibrous histiocytoma 10.2
34 pneumonia 10.2
35 spinal stenosis 10.2
36 juvenile rheumatoid arthritis 10.2
37 end stage renal disease 10.2
38 vasculitis 10.2
39 amyloidosis 10.2
40 amyloidosis aa 10.2
41 laryngocele 10.2
42 head injury 10.2

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



Diseases related to Mounier-Kuhn Syndrome
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Symptoms & Phenotypes for Mounier-Kuhn Syndrome

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Human phenotypes related to Mounier-Kuhn Syndrome:

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

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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

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Genetic Tests for Mounier-Kuhn Syndrome

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Anatomical Context for Mounier-Kuhn Syndrome

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MalaCards organs/tissues related to Mounier-Kuhn Syndrome:

40
Lung, Trachea, Smooth Muscle
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Publications for Mounier-Kuhn Syndrome

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Articles related to Mounier-Kuhn Syndrome:

(show top 50) (show all 173)
# Title Authors PMID Year
1
What is the optimal large airway size reduction value to determine malacia: exploratory bronchoscopic analysis in patients in Mounier-Kuhn syndrome. 61
33569226 2021
2
Mounier-Kuhn syndrome. 61
33509891 2021
3
Early manifestation of ARDS in COVID-19 infection in a 51- year-old man affected by Mounier-Kuhn syndrome. 61
32425272 2020
4
[Evaluation with chest CT scan of CPAP as a treatment for tracheobronchomalacia in the frame of a Mounier-Kuhn syndrome]. 61
32675007 2020
5
Improving Airways Patency and Ventilation Through Optimal Positive Pressure Identified by Noninvasive Mechanical Ventilation Titration in Mounier-Kuhn Syndrome: Protocol for an Interventional, Open-Label, Single-Arm Clinical Trial. 61
32795996 2020
6
Tracheobronchoplasty after a trial with a silicone Y-stent in a patient with Mounier-Kuhn syndrome. 61
31747002 2020
7
Tracheobronchomegaly, cough and recurrent chest infection: Mounier-Kuhn syndrome. 61
32613017 2020
8
The effect of ankaferd blood stopper used for massive haemoptysis in a patient with mounier-kuhn syndrome: A case report. 61
32296224 2020
9
Recurrent Pneumonias in a Previously Healthy and Immunocompetent Young Adult: A Case Report Mounier-Kuhn Syndrome. 61
32036382 2020
10
[Mounier-Kuhn syndrome]. 61
31076098 2020
11
A curious case of cough: Mounier-Kuhn syndrome in a Namibian female patient. 61
32733627 2020
12
Thoracic Computed Tomography Scan and Bronchoscopy Appearance of Mounier-Kuhn Syndrome: A Case Report. 61
32779489 2020
13
Mounier-Kuhn syndrome: A variable course disease. 61
33088707 2020
14
Mounier-Kuhn Syndrome. 61
30777312 2019
15
Airway management of a patient incidentally diagnosed with Mounier-Kuhn syndrome during general anesthesia. 61
31723671 2019
16
Orphan Lung Diseases. 61
30955517 2019
17
Mounier-Kuhn syndrome: an unusual cause of bronchiectasis. 61
31019346 2019
18
Volume Measurement in the Diagnosis of Mounier Kuhn Syndrome and an Unknown Accompanying Pathology: Pulmonary Artery Enlargement. 61
32008547 2019
19
Mounier-Kuhn syndrome. 61
31565119 2019
20
An Unusual Cause of Spontaneous Pneumomediastinum: The Mounier-Kuhn Syndrome. 61
31360573 2019
21
Mounier Kuhn syndrome presenting with recurrent atelectasis. 61
28891754 2018
22
An unusual cause of bronchial dilation: Mounier-Kuhn syndrome. 61
29894236 2018
23
Flexible bronchoscopy and mechanical ventilation in managing Mounier-Kuhn syndrome: a case report. 61
28443953 2018
24
Mounier-Kuhn syndrome (tracheobronchomegaly): An analysis of eleven cases. 61
28026118 2018
25
Mounier-Kuhn Syndrome Mimicking Lymphangioleiomyomatosis. 61
29406234 2018
26
Tracheal enlargement or Mounier-Kuhn syndrome in giant cell arteritis: a possible causal association with therapeutic implications. 61
30173269 2018
27
Mounier-Kuhn syndrome. 61
28596443 2017
28
Dural ectasia associated with Mounier-Kuhn syndrome: a novel association in the context of spinal stenosis. 61
28855219 2017
29
A case of tracheobronchomegaly. 61
28109701 2017
30
First Case Report of Metastatic Squamous Cell Carcinoma of Lung Associated with Mounier-Kuhn Syndrome and Review of Literature. 61
28845373 2017
31
Congenital Tracheobronchomegaly (Mounier-Kuhn Syndrome) in a Woman with Human Immunodeficiency Virus: A Case Report. 61
28480154 2017
32
Mounier-Kuhn syndrome: a case of tracheal smooth muscle remodeling. 61
28174630 2017
33
An Atypical Case of Mounier-Kuhn Syndrome: Time to Change the Diagnostic Approach? 61
27367849 2017
34
Anesthesia for a patient with unexpected giant tracheobronchomegaly. 61
28757767 2017
35
The Association of Mounier-Kuhn Syndrome and Pulmonary Aspergillomas: A Case Report. 61
27761295 2016
36
Tracheobronchomegaly associated with laryngo-tracheal amyloidosis: First case report. 61
26791590 2016
37
Tracheobronchomegaly as a Cause of Bronchiectasis in an Adult. 61
27022496 2016
38
Huge Tracheal Diverticulum in a Patient with Mounier-Kuhn Syndrome. 61
30755880 2016
39
An unusual cause of recurrent spontaneous pneumothorax: the Mounier-Kuhn syndrome. 61
26127019 2016
40
Respiratory Conditions Associated with Tracheobronchomegaly (Mounier-Kuhn Syndrome): A Study of Seventeen Cases. 61
27022925 2016
41
Mounier-Kuhn Syndrome in an Elderly Female with Pulmonary Fibrosis. 61
27597867 2016
42
Mounier-Kuhn syndrome: a systematic analysis of 128 cases published within last 25 years. 61
25130790 2016
43
A Clinical Classification Scheme for Tracheobronchomegaly (Mounier-Kuhn Syndrome). 61
26189148 2015
44
Coincidence of Tracheobronchomegaly (Mounier-Kuhn Syndrome) and Juvenile Idiopathic Arthritis. 61
26038962 2015
45
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
46
Mounier-Kuhn Syndrome. 61
30394453 2015
47
[Tracheobronchial prosthesis in Mounier-Kuhn syndrome: New perspectives]. 61
25737189 2015
48
A 54-Year-Old Man with Tracheomegaly, Tracheal Diverticulas and Bronchiectasis--Mounier-Kuhn Syndrome. 61
26591972 2015
49
Anesthetic considerations for bilateral lung transplantation in Mounier-Kuhn syndrome. 61
24529664 2015
50
Mounier-Kuhn syndrome (MKS) - Pathognomonic Findings. 61
25654012 2014
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Variations for Mounier-Kuhn Syndrome

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Expression for Mounier-Kuhn Syndrome

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

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GO Terms for Mounier-Kuhn Syndrome

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Sources for Mounier-Kuhn Syndrome

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3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
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
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Mar-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
The MalaCards human disease database index: 1-9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
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