{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,27]],"date-time":"2026-05-27T20:09:18Z","timestamp":1779912558325,"version":"3.53.1"},"reference-count":12,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2014,5,21]],"date-time":"2014-05-21T00:00:00Z","timestamp":1400630400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Clinical Respiratory J"],"published-print":{"date-parts":[[2015,7]]},"abstract":"Abstract<\/jats:title>Background and Aims<\/jats:title>The M<\/jats:styled-content>ounier\u2013K<\/jats:styled-content>uhn syndrome (MKS<\/jats:styled-content>) is a rare disease characterized by a pathological dilation of the trachea and the bronchial system. The etiology of the disorder remains elusive, but genetic alterations and degradation of elastic fibers are thought to be involved in the pathogenesis. No causative treatment is available although transplantation is an option for end\u2010stage disease. Here, we describe a patient suffering from MKS<\/jats:styled-content> who received a double lung transplant at our department.<\/jats:p><\/jats:sec>Methods<\/jats:title>Since a familial clustering of MKS<\/jats:styled-content> is discussed in the literature, we performed a chromosomal analysis and an array\u2010comparative genomic hybridization (CGH<\/jats:styled-content>) to search for genetic abnormalities. At the time of transplantation, we collected samples from the bronchi and performed hematoxylin and eosin (HE<\/jats:styled-content>), Elastic von\u2010Gieson (EVG<\/jats:styled-content>) and immunohistochemical stains of the explanted MKS<\/jats:styled-content> bronchus, a control bronchus and of the inflammatory infiltrates. Specimens of main bronchi from the donor lung harvested for transplant served as control. Bronchial smears were taken from both main bronchi of the recipient for microbiological cultures.<\/jats:p><\/jats:sec>Results<\/jats:title>No genetic alterations could be found in chromosomal analysis and in array\u2010CGH<\/jats:styled-content>. Histological analysis revealed a strong reduction of elastic fibers in the submucosal connective tissue and a diffuse inflammatory infiltrate, mainly comprised of CD4<\/jats:styled-content>+ cells. In addition, immunohistochemistry showed increased matrix metalloproteinases (MMP<\/jats:styled-content>s) protein expression of MMP<\/jats:styled-content>\u20101, 2, 3 and 9.<\/jats:p><\/jats:sec>Conclusions<\/jats:title>Based on our findings, we hypothesize that MKS<\/jats:styled-content> is a chronic inflammatory disease characterized by an MMP<\/jats:styled-content>\u2010mediated degradation of submucosal elastic fibers.<\/jats:p><\/jats:sec>","DOI":"10.1111\/crj.12139","type":"journal-article","created":{"date-parts":[[2014,4,14]],"date-time":"2014-04-14T11:35:01Z","timestamp":1397475301000},"page":"375-379","source":"Crossref","is-referenced-by-count":7,"title":["Clinical\u2010radiological, histological and genetic analyses in a lung transplant recipient with M<\/scp>ounier\u2013K<\/scp>uhn syndrome and end\u2010stage chronic obstructive pulmonary disease"],"prefix":"10.1111","volume":"9","author":[{"given":"Andreas","family":"Mitterbauer","sequence":"first","affiliation":[{"name":"Department of Thoracic Surgery Medical University of Vienna Vienna Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Konrad","family":"Hoetzenecker","sequence":"additional","affiliation":[{"name":"Department of Thoracic Surgery Medical University of Vienna Vienna Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"Birner","sequence":"additional","affiliation":[{"name":"Clinical Institute of Pathology Medical University of Vienna Vienna Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Mildner","sequence":"additional","affiliation":[{"name":"Department of Dermatology Medical University of Vienna Vienna Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Helmut","family":"Prosch","sequence":"additional","affiliation":[{"name":"Department of Radiology Medical University of Vienna Vienna Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Berthold","family":"Streubel","sequence":"additional","affiliation":[{"name":"Clinical Institute of Pathology Medical University of Vienna Vienna Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shahrokh","family":"Taghavi","sequence":"additional","affiliation":[{"name":"Department of Thoracic Surgery Medical University of Vienna Vienna Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Walter","family":"Klepetko","sequence":"additional","affiliation":[{"name":"Department of Thoracic Surgery Medical University of Vienna Vienna Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hendrik Jan","family":"Ankersmit","sequence":"additional","affiliation":[{"name":"Department of Thoracic Surgery Medical University of Vienna Vienna Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2014,5,21]]},"reference":[{"key":"e_1_2_4_2_1","doi-asserted-by":"publisher","DOI":"10.1097\/00004728-198805010-00004"},{"key":"e_1_2_4_3_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.106.5.1589"},{"key":"e_1_2_4_4_1","doi-asserted-by":"publisher","DOI":"10.1164\/arrd.1965.91.1.35"},{"key":"e_1_2_4_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.rmed.2013.08.042"},{"key":"e_1_2_4_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jtcvs.2005.11.029"},{"key":"e_1_2_4_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.healun.2005.06.018"},{"issue":"10","key":"e_1_2_4_8_1","doi-asserted-by":"crossref","first-page":"1303","DOI":"10.1183\/09031936.93.04101303","article-title":"Tracheobronchomegaly \u2013 the Mounier\u2010Kuhn syndrome: report of two cases and review of the literature","volume":"4","author":"Van Schoor J","year":"1991","journal-title":"Eur Respir J"},{"key":"e_1_2_4_9_1","doi-asserted-by":"publisher","DOI":"10.2147\/COPD.S6133"},{"key":"e_1_2_4_10_1","doi-asserted-by":"publisher","DOI":"10.1136\/thx.53.8.685"},{"key":"e_1_2_4_11_1","doi-asserted-by":"publisher","DOI":"10.1136\/thorax.57.1.15"},{"key":"e_1_2_4_12_1","doi-asserted-by":"publisher","DOI":"10.1007\/s000110300020"},{"key":"e_1_2_4_13_1","doi-asserted-by":"publisher","DOI":"10.1038\/nm1583"}],"container-title":["The Clinical Respiratory 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