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Journal Information
Vol. 13. Issue 5.
Pages 721-727 (September - October 2007)
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Vol. 13. Issue 5.
Pages 721-727 (September - October 2007)
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Síndroma de Mounier-Kuhn: Uma causa rara de infecções respiratórias de repetição
Mounier-Kuhn syndrome: A rare aetiology of recurrent respiratory infections
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Ana Marques1, Miguel Félix2, Fernando Barata3, Jorge Pires4,6, Maria Helena Estêvão5,8, S. Martinho do Bispo Coimbra7
1 Interna Complementar de Pneumologia, Serviço de Pneumologia
2 Assistente Hospitalar de Pediatria, Serviço de Medicina
3 Assistente Hospitalar de Pneumologia, Serviço de Pneumologia
4 Chefe de Serviço de Pneumologia, Director do Serviço de Pneumologia
5 Chefe de Serviço de Pediatria, Directora do Serviço de Medicina
6 Hospital Pediátrico – Centro Hospitalar de Coimbra, Director do Serviço de Pneumologia do Centro Hospitalar de Coimbra
7 Serviço de Pneumologia – Hospital Geral – Centro Hospitalar de Coimbra Quinta dos Vales – 3041-801.
8 Directora do Serviço de Medicina do Hospital Pediátrico de Coimbra, Estêvão Serviço de Medicina – Hospital Pediátrico de Coimbra Avenida Bissaya Barreto – 3000-076 Coimbra
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Resumo

A síndroma de Mounier-Kuhn é uma doença rara, de etiologia desconhecida e que se presume subdiagnosticada. Cursa frequentemente com infecções respiratórias de repetição e bronquiectasias. A clínica inespecífica, semelhante a outras patologias supurativas brônquicas crónicas, dificulta o diagnóstico, muitas vezes efectuado vários anos após o início dos sintomas. Os autores apresentam uma revisão da literatura a propósito de dois casos clínicos que ilustram a heterogeneidade e as dificuldades diagnósticas desta entidade nosológica. Um dos doentes encontrava-se em idade pediátrica na altura do diagnóstico e outro na quarta década de vida.

Palavras-chave:
Síndroma de Mounier-Kuhn
traqueobroncomegalia
infecções respiratórias de repetição
Abstract

The incidence of the syndrome may be greater than is suspected. Patients present with recurrent respiratory infections and bronchiectasis. The symptoms are non-specific and indistinguishable from those of other chronic respiratory diseases. The diagnosis is often made several years after the first clinical complaints. The authors perform a brief review of the literature and report two cases of Mounier--Kuhn syndrome. One of the patients was diagnosed in childhood and the other in the fourth decade of life.

Key-words:
Mounier-Kuhn syndrome
tracheobronchomegaly
recurrent respiratory infections
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Treatment of tracheobronchomegaly with an Ultraflex prosthesis. A case report.
Rev Pneumol Clin, 58 (2002), pp. 19-22
Copyright © 2007. Sociedade Portuguesa de Pneumologia
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