Cystic fibrosis diagnosed in adult patients

Show simple item record McWilliams, T.J. en Wilsher, M. en Kolbe, J. en 2009-06-09T03:44:32Z en 2009-06-09T03:44:32Z en 2000 en
dc.identifier.citation New Zealand Medical Journal 113 (1102), 6-8. 2000 en
dc.identifier.issn 0028-8446 en
dc.identifier.other 10738492 en
dc.identifier.uri en
dc.description An open access copy of this article is available and complies with the copyright holder/publisher conditions. en
dc.description.abstract Aim. To review the presentation, diagnosis and long-term, clinical follow-up of cystic fibrosis in adult patients diagnosed in adulthood at Green Lane Hospital. Methods. A retrospective review of the case notes of patients with cystic fibrosis diagnosed in adulthood at Green Lane Hospital or referred there for management. Information was collected on diagnostic tests, including sweat tests and genotyping. Relevant family history was documented as were spirometry results and microbial colonisation. Results. Six patients conclusively fulfilled the diagnostic criteria for cystic fibrosis. There was a wide range of ages at diagnostic (18-68) and half of the patients had a positive family history. A single mutation was identified in all, but in only one of the cases was the second mutation identified. All patients had evidence of bronchopulmonary suppuration and all had retained pancreatic function. Colonisation with P aeruginosa was associated with marked impairment in lung function. Conclusion. The patients at Green Lane Hospital represent part of the broad-spectrum disease in adult patients diagnosed with cystic fibrosis and highlight the differences between this group and those patients diagnosed in childhood with the more classical phenotype. Patients generally have less severe lung disease and retain pancreatic function. Sweat testing is useful diagnostically but gene testing is of limited value in making the diagnosis. en
dc.publisher NZMA en
dc.relation.ispartofseries New Zealand Medical Journal en
dc.rights Items in ResearchSpace are protected by copyright, with all rights reserved, unless otherwise indicated. Previously published items are made available in accordance with the copyright policy of the publisher. Details obtained from en
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dc.title Cystic fibrosis diagnosed in adult patients en
dc.type Journal Article en
dc.subject.marsden Fields of Research::320000 Medical and Health Sciences en
pubs.issue 1102 en
pubs.begin-page 6 en
pubs.volume 113 en
dc.description.version VoR - Version of Record en
dc.rights.holder Copyright: New Zealand Medical Association (NZMA) en
pubs.end-page 8 en
dc.rights.accessrights en

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