気管気管支肥大
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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/03/17 16:22:15」(JST)
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| Mounier-Kuhn syndrome |
| Classification and external resources |
| ICD-9 |
748.3 |
| OMIM |
275300 |
| DiseasesDB |
31501 |
| MeSH |
D014137 |
Mounier-Kuhn syndrome (also called tracheobronchomegaly) is a very rare congenital disorder of the lung primarily characterized by an abnormal widening of the upper airways.[1] The abnormally widened trachea and mainstem bronchi are associated with recurrent lower respiratory tract infection and copious purulent sputum production, eventually leading to bronchiectasis and other respiratory complications.[2]
§Diagnosis
Woodring et al. (1991) suggested the following diagnostic criteria for tracheomegaly in adults based on chest radiography:[3]
- Adult Males: Tracheal transverse diameter > 25 mm and sagittal diameter > 27 mm.
- Adult Females: Trachel transverse diameter > 21 mm and sagittal diameter > 23 mm.
§History
The term "Mounier-Kuhn syndrome" derives from the characterization of the condition by Mounier-Kuhn in 1932, while the name "tracheobronchomegaly" was introduced by Katz et al. in 1962.[4][5][6]
§References
- ^ Schwartz, W; Rossoff L (Nov 1994). "Tracheobronchomegaly" (PDF). Chest (American College of Chest Physicians) 106 (5): 1589–1590. doi:10.1378/chest.106.5.1589. PMID 7956426. Retrieved 2008-02-27.
- ^ Menon B, Aggarwal B, Iqbal A (2008). "Mounier-Kuhn syndrome: report of 8 cases of tracheobronchomegaly with associated complications". South Med J. 101 (1): 83–87. doi:10.1097/SMJ.0b013e31815d4259. PMID 18176298.
- ^ Woodring J et al (1999). "Congenital tracheobronchomegaly (Mounier-Kuhn syndrome)". J Thorac Imaging 6 (1).
- ^ Smith DL, Withers N, Holloway B, Collins JV (August 1994). "Tracheobronchomegaly: an unusual presentation of a rare condition". Thorax 49 (8): 840–1. doi:10.1136/thx.49.8.840. PMC 475137. PMID 8091335.
- ^ KATZ I, LEVINE M, HERMAN P (December 1962). "Tracheobronchiomegaly. The Mounier-Kuhn syndrome". Am J Roentgenol Radium Ther Nucl Med 88: 1084–94. PMID 13958486.
- ^ Mounier-Kuhn P. Dilatation de la trachée: constatations radiographiques etbronchoscopiques. Lyon Med. 1932;150:106-9.
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Congenital malformations and deformations of respiratory system (Q30–Q34, 748)
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| Upper RT |
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Nose
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Larynx
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- Laryngeal cyst
- Laryngocele
- Laryngomalacia
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| Lower RT |
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Trachea and bronchus
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- Tracheomalacia
- Tracheal stenosis
- Bronchomalacia
- Tracheobronchomegaly
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Lung
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- Bronchiectasis
- Pulmonary hypoplasia
- Pulmonary sequestration
- Congenital cystic adenomatoid malformation
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Index of the respiratory system
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| Description |
- Anatomy
- Physiology
- Development
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| Disease |
- Congenital
- Neoplasms and cancer
- Chest trauma
- Infection
- Other
- Symptoms and signs
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| Treatment |
- Procedures
- Drugs
- nasal
- throat
- obstructive airway diseases
- cough and cold
- histaminergics
- pulmonary arterial hypertension
- other
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UpToDate Contents
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English Journal
- A Clinical Classification Scheme for Tracheobronchomegaly (Mounier-Kuhn Syndrome).
- Payandeh J1,2, McGillivray B1,2, McCauley G3, Wilcox P3, Swiston JR3, Lehman A4,5.
- Lung.Lung.2015 Oct;193(5):815-22. doi: 10.1007/s00408-015-9757-z. Epub 2015 Jul 19.
- INTRODUCTION: Tracheobronchomegaly (Mounier-Kuhn Syndrome) is a rare disease characterized by tracheal enlargement and associated loss of elastic fibers in the trachea and main bronchi.MATERIALS: MEDLINE, Index Medicus, and other databases were searched with pre-defined criteria to identify cases of
- PMID 26189148
- Coincidence of Tracheobronchomegaly (Mounier-Kuhn Syndrome) and Juvenile Idiopathic Arthritis.
- Sailer S1, Osona B1, Peña-Zarza JA1, Gil-Sanchez JA1, Lacruz-Perez L1, Mulet JF1.
- Klinische Padiatrie.Klin Padiatr.2015 Sep;227(5):290-292. Epub 2015 Jun 3.
- Mounier-Kuhn syndrome (MKS) or tracheobronchomegaly includes clinical and radiographic findings of tracheobronchial dilatation and recurrent respiratory infections. MKS is a very rare pathology, especially in the paediatric age group which makes it a diagnostic challenge. A 4-year-old girl suffered
- PMID 26038962
- [Tracheobronchial prosthesis in Mounier-Kuhn syndrome: New perspectives].
- Sauvage M1, Tiffet O2, Vergnon JM3.
- Revue des maladies respiratoires.Rev Mal Respir.2015 May;32(5):519-23. doi: 10.1016/j.rmr.2015.02.003. Epub 2015 Feb 27.
- INTRODUCTION: Mounier-Kuhn syndrome or tracheobronchomegaly is a rare congenital condition, the management of which is complex. We report the case of a patient who was treated with interventional endoscopy.OBSERVATION: We describe the case of a 74-year-old man with a diagnosis of tracheobronchomegal
- PMID 25737189
Japanese Journal
- 49.Tracheobronchomegaly(Mounier-Kuhn症候群)の1例(第10回 日本気管支学会中国四国支部会)
- 大下 慎一郎,土井 美帆子,山崎 正弘,近藤 圭一,磯部 威,檜山 桂子,粟屋 幸一,河野 修興,坂井 晃,叶 康司,栗本 典昭
- 気管支学 : 日本気管支研究会雑誌 24(4), 342, 2002-05-25
- NAID 110002813524
- 気管気管支巨大症(Mounier-Kuhn症候群)の1例
- 吉富 淳,桑田 博史,鈴木 隆,桝田 幹郎,成島 道昭,妹川 史朗,塚本 克紀,須田 隆文,千田 金吾,中村 浩淑
- 日本呼吸器学会雑誌 = The journal of the Japanese Respiratory Society 38(7), 571-574, 2000-07-10
- NAID 10005610554
- 左肺全摘後にみられた Tracheobronchomegaly の 1 例(第 7 回 日本気管支学会中国四国支部会)
Related Links
- Tracheobronchomegaly symptoms, causes, diagnosis, and treatment information for Tracheobronchomegaly (Mounier-Kuhn syndrome) with alternative diagnoses, full-text book chapters, misdiagnosis, research treatments, prevention ...
- Tracheobronchomegaly is a rare cause of recurrent chest infections often with persistent, unproductive cough. A case is described which presented as a severe life ... Full text is available as a scanned copy of the original print ...
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