Mounier-Kuhn Syndrome disease: Malacards - Research Articles, Drugs, Genes, Clinical Trials

MCID: MNR004 MIFTS: 23	Mounier-Kuhn Syndrome Categories: Rare diseases, Respiratory diseases
Tissues Related diseases Publications Symptoms & Phenotypes Expand all tables

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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:

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

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...

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Related Diseases for Mounier-Kuhn Syndrome

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

(show all 46)

#	Related Disease	Score
1	tracheobronchomegaly	12.1
2	bronchiectasis	11.1
3	respiratory failure	10.8
4	idiopathic interstitial pneumonia	10.8
5	pulmonary fibrosis	10.8
6	tracheomalacia	10.7
7	bronchitis	10.7
8	pneumothorax	10.6
9	tracheobronchomalacia	10.6
10	pneumothorax, primary spontaneous	10.5
11	pulmonary disease, chronic obstructive	10.5
12	sleep apnea	10.5
13	tracheal stenosis	10.5
14	pleomorphic carcinoma	10.5
15	connective tissue disease	10.5
16	end stage renal failure	10.5
17	syncope	10.5
18	gastroesophageal reflux	10.3
19	fibrosis of extraocular muscles, congenital, 1	10.3
20	immune deficiency disease	10.3
21	lymphangioleiomyomatosis	10.3
22	spermatogenic failure 3	10.3
23	ptosis	10.3
24	squamous cell carcinoma	10.3
25	malignant fibroxanthoma	10.3
26	relapsing polychondritis	10.3
27	larynx cancer	10.3
28	pulmonary tuberculosis	10.3
29	cutis laxa	10.3
30	aspiration pneumonitis	10.3
31	spindle cell carcinoma	10.3
32	systemic scleroderma	10.3
33	histiocytoma	10.3
34	fibrous histiocytoma	10.3
35	pneumonia	10.3
36	spinal stenosis	10.3
37	juvenile rheumatoid arthritis	10.3
38	arthritis	10.3
39	vasculitis	10.3
40	amyloidosis	10.3
41	amyloidosis aa	10.3
42	laryngocele	10.3
43	undifferentiated pleomorphic sarcoma	10.3
44	head injury	10.3
45	kenny-caffey syndrome	10.3
46	rare surgical neurologic disease	10.3

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

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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

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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

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Genetic Tests for Mounier-Kuhn Syndrome

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Anatomical Context for Mounier-Kuhn Syndrome

MalaCards organs/tissues related to Mounier-Kuhn Syndrome:

⁴⁰

Lung, Trachea, Testes, Smooth Muscle

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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	Aguiar F...Silveira P	30777312	2019
2	Tracheobronchoplasty after a trial with a silicone Y-stent in a patient with Mounier-Kuhn syndrome. ⁶¹	Munoz Fos A...Galan G	31747002	2019
3	Airway management of a patient incidentally diagnosed with Mounier-Kuhn syndrome during general anesthesia. ⁶¹	No HJ...Choi S	31723671	2019
4	Orphan Lung Diseases. ⁶¹	Ali MS...Musani AI	30955517	2019
5	[Mounier-Kuhn syndrome]. ⁶¹	Castro HM...Las Heras MJ	31076098	2019
6	Mounier-Kuhn syndrome: an unusual cause of bronchiectasis. ⁶¹	Santos RFT...Nunes TF	31019346	2019
7	Mounier-Kuhn syndrome. ⁶¹	Alami B...Maaroufi M	31565119	2019
8	An Unusual Cause of Spontaneous Pneumomediastinum: The Mounier-Kuhn Syndrome. ⁶¹	Naciri S...Bourkadi JE	31360573	2019
9	Mounier Kuhn syndrome presenting with recurrent atelectasis. ⁶¹	Quentin C...Hanssens L	28891754	2018
10	An unusual cause of bronchial dilation: Mounier-Kuhn syndrome. ⁶¹	Wu T...Deng Z	29894236	2018
11	Flexible bronchoscopy and mechanical ventilation in managing Mounier-Kuhn syndrome: a case report. ⁶¹	Kaya AG...Karnak D	28443953	2018
12	Mounier-Kuhn syndrome (tracheobronchomegaly): An analysis of eleven cases. ⁶¹	Akgedik R...Cakir L	28026118	2018
13	Mounier-Kuhn Syndrome Mimicking Lymphangioleiomyomatosis. ⁶¹	Pacheco GG...Moss J	29406234	2018
14	Tracheal enlargement or Mounier-Kuhn syndrome in giant cell arteritis: a possible causal association with therapeutic implications. ⁶¹	Damian LO...Poenaru M	30173269	2018
15	Mounier-Kuhn syndrome. ⁶¹	Simkins A...Estrada-Y-Martin RM	28596443	2017
16	Dural ectasia associated with Mounier-Kuhn syndrome: a novel association in the context of spinal stenosis. ⁶¹	Sheridan GA...Devitt A	28855219	2017
17	A case of tracheobronchomegaly. ⁶¹	Chenbhanich J...Konter J	28109701	2017
18	First Case Report of Metastatic Squamous Cell Carcinoma of Lung Associated with Mounier-Kuhn Syndrome and Review of Literature. ⁶¹	Ayub F...Saif MW	28845373	2017
19	Congenital Tracheobronchomegaly (Mounier-Kuhn Syndrome) in a Woman with Human Immunodeficiency Virus: A Case Report. ⁶¹	Fletcher A...Jamoulis S	28480154	2017
20	Mounier-Kuhn syndrome: a case of tracheal smooth muscle remodeling. ⁶¹	Cook DP...Stoltz DA	28174630	2017
21	Anesthesia for a patient with unexpected giant tracheobronchomegaly. ⁶¹	Lee CC...Chuang CC	28757767	2017
22	An Atypical Case of Mounier-Kuhn Syndrome: Time to Change the Diagnostic Approach? ⁶¹	Kuwal A...Saini LK	27367849	2017
23	The Association of Mounier-Kuhn Syndrome and Pulmonary Aspergillomas: A Case Report. ⁶¹	Akgedik R...Tas N	27761295	2016
24	Tracheobronchomegaly associated with laryngo-tracheal amyloidosis: First case report. ⁶¹	Adachi K...Komune S	26791590	2016
25	An unusual cause of recurrent spontaneous pneumothorax: the Mounier-Kuhn syndrome. ⁶¹	Unlu EN...Guclu D	26127019	2016
26	Respiratory Conditions Associated with Tracheobronchomegaly (Mounier-Kuhn Syndrome): A Study of Seventeen Cases. ⁶¹	Schmitt P...Groupe d''Endoscopie de Langue Francaise (GELF)	27022925	2016
27	Mounier-Kuhn Syndrome in an Elderly Female with Pulmonary Fibrosis. ⁶¹	Boglou P...Steiropoulos P	27597867	2016
28	Tracheobronchomegaly as a Cause of Bronchiectasis in an Adult. ⁶¹	Govindaraj V...Narayanasami S	27022496	2016
29	Mounier-Kuhn syndrome: a systematic analysis of 128 cases published within last 25 years. ⁶¹	Krustins E	25130790	2016
30	Huge Tracheal Diverticulum in a Patient with Mounier-Kuhn Syndrome. ⁶¹	Mondoni M...Centanni S	30755880	2016
31	A Clinical Classification Scheme for Tracheobronchomegaly (Mounier-Kuhn Syndrome). ⁶¹	Payandeh J...Lehman A	26189148	2015
32	Coincidence of Tracheobronchomegaly (Mounier-Kuhn Syndrome) and Juvenile Idiopathic Arthritis. ⁶¹	Sailer S...Mulet JF	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	Mitterbauer A...Ankersmit HJ	24725636	2015
34	Mounier-Kuhn Syndrome. ⁶¹	Schiettecatte E...Verstraete K	30394453	2015
35	[Tracheobronchial prosthesis in Mounier-Kuhn syndrome: New perspectives]. ⁶¹	Sauvage M...Vergnon JM	25737189	2015
36	A 54-Year-Old Man with Tracheomegaly, Tracheal Diverticulas and Bronchiectasis--Mounier-Kuhn Syndrome. ⁶¹	Singh M...Kumar R	26591972	2015
37	Anesthetic considerations for bilateral lung transplantation in Mounier-Kuhn syndrome. ⁶¹	Subramani S...Rajagopal S	24529664	2015
38	Mounier-Kuhn syndrome (MKS) - Pathognomonic Findings. ⁶¹	Kumar S...Mittal AK	25654012	2014
39	The man with the large trachea: Mounier-Kuhn syndrome. ⁶¹	Lerner C...Julka K	25004454	2014
40	Mounier-Kuhn syndrome: more than just a cough. ⁶¹	Azzopardi C...Grech R	25213787	2014
41	Mounier-Kuhn syndrome. Imaging and bronchoscopic findings. ⁶¹	El-Kersh K...Perez RL	24983230	2014
42	A delayed diagnosis of Mounier-Kuhn syndrome. ⁶¹	Gouder C...Montefort S	24891495	2014
43	Mounier-Kuhn syndrome presenting as cough. ⁶¹	Andrews SM...Lee DW	24787068	2014
44	[Mounier-Kuhn syndrome - a rare cause of recurrent bronchitis]. ⁶¹	Bayer M...Kern L	24668436	2014
45	Mounier-Kuhn syndrome. ⁶¹	Simon M...Andrei F	24739689	2014
46	A grossly abnormal trachea- severe tracheal diverticulosis and Mounier-Kuhn syndrome. ⁶¹	Lyons OD...Gilmartin JJ	24757893	2014
47	Mounier-Kuhn syndrome masquerading pulmonary thromboembolism in an elderly male. ⁶¹	Gupta P...Khandelwal N	24669091	2014
48	Recurrent pneumonia as a result of Mounier-Kuhn syndrome. ⁶¹	Desiderio M...Nagarajan V	24401975	2014
49	Tracheomalacia treatment using a large-diameter, custom-made airway stent in a case with mounier-kuhn syndrome. ⁶¹	Ozdemir C...Dalar L	25276462	2014
50	Mounier-Kuhn syndrome: a rare and often overlooked cause of bronchial dilation and recurrent respiratory tract infections. ⁶¹	Kapoor S	24626279	2014

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Genes for Mounier-Kuhn Syndrome

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Variations for Mounier-Kuhn Syndrome

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Expression for Mounier-Kuhn Syndrome

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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