A Case Report of Pulmonary Exacerbation after Initiation of Lumacaftor/Ivacaftor Therapy in a CF Female with Complicated Lung Disease
Novel targeted treatments for Cystic Fibrosis give rise to new hope for an ever-growing number of CF patients with various mutations. However, very little evidence and guidelines exist to steer clinical decisions regarding patients whose illness takes an unexpected course. In such cases, the benefit...
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| Format: | Article |
| Language: | English |
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Wiley
2018-01-01
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| Series: | Case Reports in Pulmonology |
| Online Access: | http://dx.doi.org/10.1155/2018/8394370 |
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| author | Elpis Hatziagorou Eleana Kouroukli Vasiliki Georgopoulou John Tsanakas |
| author_facet | Elpis Hatziagorou Eleana Kouroukli Vasiliki Georgopoulou John Tsanakas |
| author_sort | Elpis Hatziagorou |
| collection | DOAJ |
| description | Novel targeted treatments for Cystic Fibrosis give rise to new hope for an ever-growing number of CF patients with various mutations. However, very little evidence and guidelines exist to steer clinical decisions regarding patients whose illness takes an unexpected course. In such cases, the benefits and risks of discontinuing these treatments must be carefully and individually weighed, since their long-term effects remain mainly uncharted territory. In this report we document the case of a homozygous F508del CF patient with severe lung disease who presented with a pulmonary exacerbation shortly after the beginning of treatment with lumacaftor/ivacaftor and the complicated initial phase of therapy, which was followed by significant improvements. |
| format | Article |
| id | doaj-art-b95b97f563ec4b32b56afb2c20b47ad9 |
| institution | Kabale University |
| issn | 2090-6846 2090-6854 |
| language | English |
| publishDate | 2018-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Pulmonology |
| spelling | doaj-art-b95b97f563ec4b32b56afb2c20b47ad92025-02-03T01:07:03ZengWileyCase Reports in Pulmonology2090-68462090-68542018-01-01201810.1155/2018/83943708394370A Case Report of Pulmonary Exacerbation after Initiation of Lumacaftor/Ivacaftor Therapy in a CF Female with Complicated Lung DiseaseElpis Hatziagorou0Eleana Kouroukli1Vasiliki Georgopoulou2John Tsanakas33rd Paediatric Department, CF Unit, Hippokration General Hospital of Thessaloniki, Aristotle University of Thessaloniki, Greece3rd Paediatric Department, CF Unit, Hippokration General Hospital of Thessaloniki, Aristotle University of Thessaloniki, GreeceRadiology Department, Hippokration General Hospital of Thessaloniki, Greece3rd Paediatric Department, CF Unit, Hippokration General Hospital of Thessaloniki, Aristotle University of Thessaloniki, GreeceNovel targeted treatments for Cystic Fibrosis give rise to new hope for an ever-growing number of CF patients with various mutations. However, very little evidence and guidelines exist to steer clinical decisions regarding patients whose illness takes an unexpected course. In such cases, the benefits and risks of discontinuing these treatments must be carefully and individually weighed, since their long-term effects remain mainly uncharted territory. In this report we document the case of a homozygous F508del CF patient with severe lung disease who presented with a pulmonary exacerbation shortly after the beginning of treatment with lumacaftor/ivacaftor and the complicated initial phase of therapy, which was followed by significant improvements.http://dx.doi.org/10.1155/2018/8394370 |
| spellingShingle | Elpis Hatziagorou Eleana Kouroukli Vasiliki Georgopoulou John Tsanakas A Case Report of Pulmonary Exacerbation after Initiation of Lumacaftor/Ivacaftor Therapy in a CF Female with Complicated Lung Disease Case Reports in Pulmonology |
| title | A Case Report of Pulmonary Exacerbation after Initiation of Lumacaftor/Ivacaftor Therapy in a CF Female with Complicated Lung Disease |
| title_full | A Case Report of Pulmonary Exacerbation after Initiation of Lumacaftor/Ivacaftor Therapy in a CF Female with Complicated Lung Disease |
| title_fullStr | A Case Report of Pulmonary Exacerbation after Initiation of Lumacaftor/Ivacaftor Therapy in a CF Female with Complicated Lung Disease |
| title_full_unstemmed | A Case Report of Pulmonary Exacerbation after Initiation of Lumacaftor/Ivacaftor Therapy in a CF Female with Complicated Lung Disease |
| title_short | A Case Report of Pulmonary Exacerbation after Initiation of Lumacaftor/Ivacaftor Therapy in a CF Female with Complicated Lung Disease |
| title_sort | case report of pulmonary exacerbation after initiation of lumacaftor ivacaftor therapy in a cf female with complicated lung disease |
| url | http://dx.doi.org/10.1155/2018/8394370 |
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