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Severe tracheal and bronchial collapse in adults with type II mucopolysaccharidosis.
Rutten, M; Ciet, P; van den Biggelaar, R; Oussoren, E; Langendonk, J G; van der Ploeg, A T; Langeveld, M.
Afiliación
  • Rutten M; Department of Pulmonology, Erasmus University Medical Center, Rotterdam, The Netherlands.
  • Ciet P; Department of Radiology, Erasmus University Medical Center, Rotterdam, The Netherlands.
  • van den Biggelaar R; Department of Paediatric Pulmonology, Sophia Children's Hospital, Erasmus University Medical Center, Rotterdam, The Netherlands.
  • Oussoren E; Department of Pulmonology, Erasmus University Medical Center, Rotterdam, The Netherlands.
  • Langendonk JG; Center for Lysosomal and Metabolic Diseases, Erasmus University Medical Center, Rotterdam, The Netherlands.
  • van der Ploeg AT; Center for Lysosomal and Metabolic Diseases, Erasmus University Medical Center, Rotterdam, The Netherlands.
  • Langeveld M; Division of Pharmacology, Vascular and Metabolic Diseases, Department of Internal Medicine, Erasmus University Medical Center, Rotterdam, The Netherlands.
Orphanet J Rare Dis ; 11: 50, 2016 Apr 26.
Article en En | MEDLINE | ID: mdl-27112191
BACKGROUND: Mucopolysaccharidosis type II (MPSII) patients frequently suffer from dyspnoea caused by restrictive airway disease due to skeletal abnormalities as well as glycosaminoglycans (GAG) accumulation at different levels of the airway, including the trachea. In this study we describe the extent of the tracheal and bronchial narrowing, the changes in airway diameter during respiration and the effects of these obstructions on respiratory function in adult MPSII patients. METHODS: Five adult MPSII patients (mean age 40 years) were included. Pulmonary function tests and in- and expiratory chest CT scans were obtained. Cross-sectional areas of trachea and main bronchi were measured at end-inspiration and -expiration and percentage collapse was calculated. RESULTS: There was diffuse narrowing of the entire intra-thoracic trachea and main bronchi and severe expiratory collapse of the trachea in all patients. At 1 cm above the aortic arch the median % collapse of the trachea was 68 (range 60 to 77%), at the level of the aortic arch 64 (range 21-93%), for the main bronchi this was 58 (range 26-66%) on the left and 44 (range 9-76%) on the right side. The pulmonary function tests showed that this airway collapse results in obstructive airway disease in all patients, which was severe (forced expiratory volume <50% of predicted) in four out of five patients. CONCLUSION: In adult MPS II patients, central airways diameters are strikingly reduced and upon expiration there is extensive collapse of the trachea and main bronchi. This central airways obstruction explains the severe respiratory symptoms in MPSII patients.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Tráquea / Bronquios / Mucopolisacaridosis II Límite: Adult / Female / Humans / Male / Middle aged Idioma: En Revista: Orphanet J Rare Dis Asunto de la revista: MEDICINA Año: 2016 Tipo del documento: Article País de afiliación: Países Bajos Pais de publicación: Reino Unido
Texto completo
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Tráquea / Bronquios / Mucopolisacaridosis II Límite: Adult / Female / Humans / Male / Middle aged Idioma: En Revista: Orphanet J Rare Dis Asunto de la revista: MEDICINA Año: 2016 Tipo del documento: Article País de afiliación: Países Bajos Pais de publicación: Reino Unido