Síndrome de Mounier-Kuhn: achados radiológicos e apresentação clínica

Bastos, Andréa de Lima; Brito, Isabela Lage Alves de

doi:10.1590/s0100-39842011000300016

Cited by 3 publications

(2 citation statements)

References 6 publications

(8 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Prognosis of MKS is unclear as the published reports mostly provide information about the patient only as seen at the point of diagnosis and lack follow‐up data. In 10.9% ( n = 14) of the cases, a lasting improvement with therapy was noted and in 5.5% ( n = 7) case the disease progressed over time, including three cases (2.3%) where the patient died [in two of these of progressing respiratory failure and in the other of acute respiratory failure due to pulmonary embolism and subsequent nosocomial pneumonia ]. But it must be noted that of 10 cases where length of follow up was noted, in just two of them the follow up was 8 and 16 years, with an average of 2.2 years in remaining eight, so long‐term data are not available.…”

Section: Resultsmentioning

confidence: 99%

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

Krustiņš

2014

Clinical Respiratory J

View full text Add to dashboard Cite

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.

show abstract

Section: Resultsmentioning

confidence: 99%

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

Krustiņš

2014

Clinical Respiratory J

View full text Add to dashboard Cite

show abstract

“…Although this condition is typically encountered in middle-aged men [3–5, 11–13] our patient was an elderly female, which emphasises that MKS needs to be considered in older subjects as well. Another important issue relating to the patient presented was the previous diagnosis of pulmonary fibrosis.…”

Section: Discussionmentioning

confidence: 85%

Mounier-Kuhn Syndrome in an Elderly Female with Pulmonary Fibrosis

Boglou

Παπάνας

Oikonomou

et al. 2016

Case Reports in Medicine

View full text Add to dashboard Cite

Mounier-Kuhn syndrome (MKS), or tracheobronchomegaly, is a rare clinical and radiologic condition characterized by pronounced tracheobronchial dilation and recurrent lower respiratory tract infections. Tracheobronchomegaly presents when the defect extends to the central bronchi. MKS can be diagnosed in adult women when the transverse and sagittal diameters of the trachea, right mainstem bronchus, and left mainstem bronchus exceed 21, 23, 19.8, and 17.4 mm, respectively. Its diagnosis is based on chest radiograph and chest computed tomography (CT). Patients, usually middle-aged men, may be asymptomatic or present with clinical manifestations ranging from minimal symptoms with preserved lung function to severe respiratory failure. Pulmonary function tests (PFTs) typically reveal a restrictive pattern. This report presents an elderly woman with previously diagnosed pulmonary fibrosis with symptoms of increased sputum production and haemoptysis. High-resolution chest CT showed tracheal and main stem bronchi dilatation along with bronchial diverticulosis. PFTs indicated a restrictive pattern characteristic of the underlying pulmonary fibrosis. The patient is the oldest, referred to the female gender, at presentation of MKS hitherto reported. This case highlights the need to include MKS in the differential diagnosis of recurrent lower respiratory tract infections, even in older subjects.

show abstract

Early manifestation of ARDS in COVID-19 infection in a 51- year-old man affected by Mounier-Kuhn syndrome

et al. 2020

View full text Add to dashboard Cite

A 51-year-old man known to be affected by Mounier-Kuhn syndrome (MKS). presented to Accident & Emergecy (A&E) with fever, dyspnea and deterioration of his chronic coughs. Increased diameters of his trachea (39 mm), right (30 mm) and left (26 mm) main bronchi were revealed by chest computerized tomography (CT) scan. CT scan showed also ground-glass opacities (GGO) and bronchiectasis in the mid and lower zones of both lungs. COIVD-19 infection was eventually confirmed by RT-PCR. A severe form of COVID-19 could occur even in the early stages of the disease in presence of underlying co-morbidities including MKS, which increases the susceptibility to more recurrent and severe respiratory infections.

show abstract

Síndrome de Mounier-Kuhn: achados radiológicos e apresentação clínica

Cited by 3 publications

References 6 publications

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

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

Mounier-Kuhn Syndrome in an Elderly Female with Pulmonary Fibrosis

Early manifestation of ARDS in COVID-19 infection in a 51- year-old man affected by Mounier-Kuhn syndrome

Contact Info

Product

Resources

About