Double Lung Transplantation in a Patient with Tracheobronchomegaly (Mounier-Kuhn Syndrome)

Drain, Andrew J.; Perrin, Felicity; Tasker, A.D.; Stewart, Susan; Wells, Francis C.; Tsui, Steven; Sivasothy, Siva

doi:10.1016/j.healun.2005.06.018

Cited by 19 publications

(6 citation statements)

References 5 publications

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“…This is the third described case of lung transplantation in an MKS patient. In contrast to the previous reports the postoperative course of our patient was uneventful . Despite the reduced mucociliary clearance of the disease, he developed no infectious complications.…”

Section: Discussioncontrasting

confidence: 89%

Clinical‐radiological, histological and genetic analyses in a lung transplant recipient with Mounier–Kuhn syndrome and end‐stage chronic obstructive pulmonary disease

Mitterbauer

Höetzenecker

Birner

et al. 2014

Clinical Respiratory J

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Section: Discussioncontrasting

confidence: 89%

Clinical‐radiological, histological and genetic analyses in a lung transplant recipient with Mounier–Kuhn syndrome and end‐stage chronic obstructive pulmonary disease

Mitterbauer

Höetzenecker

Birner

et al. 2014

Clinical Respiratory J

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“…But again, the lack of data in the case reports did not allow for a grading of severity of patients' symptoms. Additionally, among the excluded cases with no tracheal measurements, there were two with (presumably) isolated bronchomegaly , the significance of which remains uncertain. The fact that increasing age does not correlate with tracheal or bronchial size could mean that once certain enlargement has taken place, the anatomical changes do not progress over time.…”

Section: Discussionmentioning

confidence: 99%

Mounier‐Kuhn syndrome: a systematic analysis of 128 cases published within last 25 years

Krustiņš

2014

Clinical Respiratory J

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Background and Aims: Mounier-Kuhn syndrome is a rare disease with abnormal enlargement of major airways, but epidemiological studies are lacking, and currently the most available data about it come from case reports, making it difficult to collate changes in a particular patient to those in previously published cases.The aim of this work is to systematically review cases published in the last 25 years and to use descriptive statistics to summarize the patient demographic and clinical information therein in order to acquire details about patient clinical characteristics. Methods: Cases published in world literature between 1987 and 2013 were sought and reviewed according to PRISMA guidelines. Cases were included only if patient's age was at least 18 years, and a computed tomography scan with tracheal measurements was available.Results: An 8:1 male predominance was found in 89 identified reports (128 cases). Mean age was 53.9 years, and average tracheal diameter was 36.1 mm. No correlation between increasing age and increasing tracheal diameter was found. Bronchiectasis, tracheal diverticulosis and tracheobronchial dyskinesia were common (49.2%, 33.6% and 28.9%, respectively). Cough, dyspnea and recurrent respiratory infections (71.1%, 51.6% and 50.8%, respectively) were the most common complaints. Conclusions:The data confirm that syndrome mostly manifests with nonspecific respiratory symptoms and is significantly more common in males. Importantly, there was no connection between age and airway diameter, a fact that could mean that the enlargement does not progress over time, and its severity depends on some other yet undetermined factors.

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“…Lung transplantation has been reported twice: a bilateral lung transplantation in a patient with 27 years of pulmonary symptoms and rheumatoid arthritis [14] and in a patient, whom the MKS was diagnosed in the explanted lungs [27]. Both patients died soon after the transplantation [28].…”

Section: Treatment Optionsmentioning

confidence: 99%

Mounier-Kuhn syndrome or congenital tracheobronchomegaly: A literature review

Krustiņš

Kravale

Buls

2013

Respiratory Medicine

124

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Mounier-Kuhn syndrome or congenital tracheobronchomegaly is a chronic airway condition which for currently unknown reasons mostly affects males. It is commonly overlooked on conventional chest X-rays, and is considered to be rare, but the prevalence might be higher as commonly assumed. The hallmark of it is a dilatation of the main airways which frequently, but not always, causes marked, mainly respiratory, symptoms, and patients usually present with varying degrees of recurrent infections, breathlessness, haemoptysis, dyspnoea. Although at least 200 case reports have been published, there have been only a few attempts to review them, and none in the last 20 years. Due to the lack of clinical trials and wide variability of case-report format, a systematic review was deemed not feasible, therefore PubMed and Medline databases were searched using terms "Mounier-Kuhn syndrome", "tracheobronchomegaly", "tracheomegaly", and "bronchomegaly", without any time restrictions, to summarize currently known facts about the syndrome. To the authors' best knowledge, the result is currently the most comprehensive review of previously published literature about the congenital tracheobronchomegaly, and summarizes what's known about symptoms, prevalence, disease associations, and treatment options for this syndrome.

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Double Lung Transplantation in a Patient with Tracheobronchomegaly (Mounier-Kuhn Syndrome)

Cited by 19 publications

References 5 publications

Clinical‐radiological, histological and genetic analyses in a lung transplant recipient with Mounier–Kuhn syndrome and end‐stage chronic obstructive pulmonary disease

Clinical‐radiological, histological and genetic analyses in a lung transplant recipient with Mounier–Kuhn syndrome and end‐stage chronic obstructive pulmonary disease

Mounier‐Kuhn syndrome: a systematic analysis of 128 cases published within last 25 years

Mounier-Kuhn syndrome or congenital tracheobronchomegaly: A literature review

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