Symptoma

Mounier-Kühn Syndrome

SCD

The clinical presentation is variable and it can occur in several different age groups. We present the case of a young man who was admitted to the hospital for an asthma exacerbation and was found to have tracheobronchomegaly on his CT scan. [ncbi.nlm.nih.gov]

A case is described which presented as a severe life threatening pneumonia in which the bronchoscopic, radiographic, and computed tomographic findings are given. [doi.org]

This case is being presented due to its rarity and unusual presentation as a single mass lesion arising from the posterior wall of the larynx causing near total obliteration of the tracheal lumen. Financial support and sponsorship Nil. [ijpmonline.org]

Tracheobronchomegaly presents when the defect extends to the central bronchi. [hindawi.com]

In mild cases, patients are asymptomatic or present only with chronic cough and have normal pulmonary function. [orpha.net]

  • Splenomegaly

    There was no lymphadenopathy or splenomegaly. Routine laboratory findings revealed low hemoglobin level with elevated serum transaminases and alkaline phosphatase levels. [academic.oup.com]

  • Recurrent Bronchitis

    bronchitis]. 61 Bayer M...Kern L 24668436 2014 47 Mounier-Kuhn syndrome. 61 Simon M...Andrei F 24739689 2014 48 A grossly abnormal trachea- severe tracheal diverticulosis and Mounier-Kuhn syndrome. 61 Lyons OD...Gilmartin JJ 24757893 2014 49 Mounier-Kuhn [malacards.org]

    Dyspnea, cough, difficulty in clearing secretions, recurrent bronchitis, pneumonia, and respiratory failure have been reported in patients with the Mounier-Kuhn syndrome.7 These are nonspecific symptoms and are similar to those in patients with chronic [journals.lww.com]

  • Constitutional Symptom

    There were generalized constitutional symptoms of fatigue, malaise and weight loss. The patient had no family history of chronic obstructive pulmonary diseases. He had always been a non- smoker and worked as manual labourer. [jemds.com]

  • Bronchial Breath Sounds

    Cardiorespiratory examination was significant for bilateral crepitations and occasional bronchial breath sounds. The heart sounds were normal. Hemogram revealed mild anemia (hemoglobin 11.5 g%). [lungindia.com]

    Tachycardia, bronchial breath sounds, diffuse expiratory wheezing, and rhonchi were noted on physical examination. Laboratory analysis was significant for leukocytosis (white blood cell count of 12.0 103/µL) with normal procalcitonin ( 0.05 ng/mL). [cureus.com]

  • Decreased Breath Sounds

    Physical examination revealed increased body temperature with decreased breath sound in both lungs with inspiratory crepitations, more pronounced in mid and lower lung field. Finger clubbing was present. [omicsonline.org]

  • Chronic Abdominal Pain

    In our case, despite presence of chronic cough, the diseases was incidentally discovered at imaging during exploration of a chronic abdominal pain. [academic.oup.com]

  • Abdominal Pain

    In our case, despite presence of chronic cough, the diseases was incidentally discovered at imaging during exploration of a chronic abdominal pain. [academic.oup.com]

  • Regurgitation

    Associated findings included decreased contractility of RV and moderate tricuspid regurgitation. A diagnosis of acute pulmonary thromboembolism (PTE) was suggested. [lungindia.com]

  • Hepatomegaly

    The physical examination was unremarkable, except hepatomegaly. There was no lymphadenopathy or splenomegaly. Routine laboratory findings revealed low hemoglobin level with elevated serum transaminases and alkaline phosphatase levels. [academic.oup.com]

The clinical presentation of TO is variable and ranges from incidental diagnosis in asymptomatic patients during workup or management for unrelated medical problems, to devastating disease with central airway obstruction. [utsouthwestern.influuent.utsystem.edu]

For further diagnostic workup bronchoscopy was performed afterwards. How to Cite: Schiettecatte E, Van Hedent E, Van Herreweghe R, Verstraete K. Mounier-Kuhn Syndrome. Journal of the Belgian Society of Radiology. 2015;98(3):139–40. [jbsr.be]

Our case is the oldest female patient described in literature with MKS, indicating that appropriate diagnostic workup may be required in elderly subjects as well. Competing Interests The authors declare that there are no competing interests. [hindawi.com]

Although her HIV has been well-managed, clinical management of future exacerbations could lead to complicated and extensive infectious disease workups. [cureus.com]

  • Air Bronchogram

    The study revealed an air space consolidation involving right upper lobe with air bronchograms appearing as a soft tissue density on soft tissue window [Figure 1] a and [Figure 1] b. [aihbonline.com]

  • Pseudomonas in the Sputum

    We describe a case of an older lady labelled for many years as COAD but who was a lifelong non-smoker and had Pseudomonas in her sputum. A CT of thorax for suspected bronchiectasis revealed tracheobroncomegaly (Mounier-Kuhn syndrome). [ageing.oxfordjournals.org]

Management and treatment Treatment is symptomatic. Chest physiotherapy can be proposed to improve mucociliary clearance and antibiotics are administered for treatment of pulmonary infections. [orpha.net]

The treatment of TO is usually symptomatic. with emphasis on the management and prevention of recurrent respiratory infections. [utsouthwestern.influuent.utsystem.edu]

Case reports discussing the choice and efficiency of antibiotic treatment are random. [tandfonline.com]

We report on a group of patients with TO and discuss their presentation and their treatment modalities. [link.springer.com]

Overall prognosis is generally good and is dependent upon airway stenosis caused by nodules. [radiopaedia.org]

Prognosis The impact of this condition on life expectancy has not been evaluated. [orpha.net]

Etiology The etiology of tracheobronchomegaly is unknown. Some authors have postulated a congenital etiology Only gold members can continue reading. [radiologykey.com]

The etiologic mechanism remains unknown. Irritants like cigarette smoke and air pollution could act as irritating factors. [orpha.net]

This is the first report which strongly supports granulomatous etiology such as TB as possible etiology for TPO. [lungindia.com]

HRCT not only allows the identification of bronchiectasis, determining the extent and type of the disease, but also allows in some cases the establishment of the etiology of bronchiectasis. 1. Habesoglu MA, Ugurlu AO, Eyuboglu FO. [thoracicmedicine.org]

Summary Epidemiology It is a rare underdiagnosed condition of unknown prevalence. About 300 cases have been reported to date. The condition is more frequent in men. [orpha.net]

Home Online First Current Issue Past Issues Journal Home FIND ISSUES Current Issue ARTICLE & MULTIMEDIA Most Accessed Articles Clinical Epidemiology Workshop Series Supplements Videos FOR AUTHORS Guide for Author Reviewer List BOPA Online Submission ABOUT [hkmj.org]

While no epidemiologic study has been published, MKS has been found to disproportionally affect men with an 8:1 male to female ratio [4-5]. [cureus.com]

Epidemiology Mounier-Kuhn syndrome is most frequently seen in middle age men before the age of 50 1,6. Clinical pr... [wcsjrawq.ga]

He was then treated according to the guidelines for P. aeruginosa management in cystic fibrosis patients considering the similarities in clinical presentations and pathophysiology of both diseases. [tandfonline.com]

The etiology and pathophysiology of TO are still needed to be found. An association between atrophic rhinitis and TO was reported ( 6 ), but the directly relationship still lacks enough evidence. [jtd.amegroups.com]

This is an incidental finding, since from the pathophysiological point of view there is no causal relationship between idiopathic pulmonary fibrosis and Mounier Kuhn's syndrome. [scielo.org.co]

View Article PubMed Google Scholar McCool FD: Global physiology and pathophysiology of cough: ACCP evidence-based clinical practice guidelines. Chest. 2006, 129: 48S-53S. 10.1378/chest.129.1_suppl.48S. [coughjournal.biomedcentral.com]

The treatment of TO is usually symptomatic. with emphasis on the management and prevention of recurrent respiratory infections. [utsouthwestern.influuent.utsystem.edu]

Postoperative continuous positive airway pressure to prevent airway collapse followed awake extubation. [dailyrounds.org]

It is important to identify MKS, as early recognition can lead to better management and prevention of complications. Very few cases of MKS with Bronchial Asthma have been reported so far. [ijmrhs.com]

Control and prevention of recurrent infections will prevent progression to irreversible pulmonary fibrosis. Several trials have also shown benefit of continuous positive airway pressure, airway stenting, and tracheobronchoplasty [9]. [cureus.com]

Management: There is no permanent and definitive treatment to prevent the formation of the respiratory nodules. In advanced cases, surgical resection or laser therapy of the nodules may be required. [xpertdox.com]