Review Reports

Round 1

Reviewer 1 Report

Comments and Suggestions for Authors

The paper is very descriptive and well written. The only recommendations I would suggest are specifying how the images were obtained (e.g. MRI/CT), and explaining the differences in the before and after images which has led to the conclusions of improved sinonasal and lung involvement.

Author Response

The paper is very descriptive and well written. The only recommendations I would suggest are specifying how the images were obtained (e.g. MRI/CT), and explaining the differences in the before and after images which has led to the conclusions of improved sinonasal and lung involvement.

Thank you very much for your valuable suggestions.  We added computed tomography in the title. Detailed explanations of CT acquisition parameters were added in the text, as suggested. The images differences between before and after treatment acquisitions were added.

Reviewer 2 Report

Comments and Suggestions for Authors

Tagliati and colleagues report on a case of CF with a muation combination that, to the authours best knowledge, has not been studied under HEMT therapy for sinonasal and lung imaging. 

The format "Interesting Images" is a good fit for the data provided, albeit the follwoing items need to be corrected prior to publication:

1. line 30, authors state FEV (44 before, 58 after). Please edit to report unit, mode of normalization (this is not liter, of course).
2. line 48, the term "What's more" appears to colloquial
3. References 14 and 16: while the two previous publitaions on HEMT / sinonasal improvement, published by the author, are relevant in this context, the work would benefit from a more balanced reflection of imaging data prior to and after onset of HEMT therapy. Large US or EU studies are currently not mentioned. To make the context more visible to readers, kindly extend the reference list to incorporate recent work  from other groups active in imaging in CF. As the authors show both, sinonasal and chest imaging, kindly reference the outcome of HEMT on both more comprehensively. 

Author Response

Tagliati and colleagues report on a case of CF with a mutation combination that, to the authors best knowledge, has not been studied under HEMT therapy for sinonasal and lung imaging. 

The format "Interesting Images" is a good fit for the data provided

Thank you very much.

 

 

The following items need to be corrected prior to publication:

1. line 30, authors state FEV (44 before, 58 after). Please edit to report unit, mode of normalization (this is not liter, of course).

Thank you very much for your suggestion. We added percentage of predicted value

 

 

2. line 48, the term "What's more" appears to colloquial

Thank you very much. We changed “What’s more” with “Furthermore”.

 

 

3. References 14 and 16: while the two previous publications on HEMT / sinonasal improvement, published by the author, are relevant in this context, the work would benefit from a more balanced reflection of imaging data prior to and after onset of HEMT therapy. Large US or EU studies are currently not mentioned. To make the context more visible to readers, kindly extend the reference list to incorporate recent work from other groups active in imaging in CF. As the authors show both, sinonasal and chest imaging, kindly reference the outcome of HEMT on both more comprehensively. 

Thank you very much for your valuable suggestions. We added some recent articles.

 

Round 2

Reviewer 2 Report

Comments and Suggestions for Authors

The reviewer thanks the authors for extending their methods description in the figure legend. The interpretatoion of the CT data is certainly helpful for the Journal's audience.

However, the reviewer still misses some references work on sinonasal imaging in pwCF i n the context of ETI treatment, which is highly relevant for the author's work. The reviewer also apologises that during the last review, the reviewer has hoped that the authors would find those references themselves as their expertise is a lot better for CT than the reviewer's expertise on that method, albeit, as information in the internet is not always easy to find, here are some suggestions retrieved with the search term "CT sinonasal cystic fibrosis ETI" in PubMed.

Beyond the Lung. Impact of Elexacaftor/Tezacaftor/Ivacaftor on Sinonasal Disease in Children With Cystic Fibrosis. Petitjean M, Letierce A, Bonnel AS, Reix P, Deneuville E, Stremler N, Luscan R, Couloigner V, Mely L, Bessaci K, Labbe G, Marguet C, Kelly-Aubert M, Hassani F, Sermet-Gaudelus I, Simon F; MODUL‐CF Study Group. Int Forum Allergy Rhinol. 2025 Jul;15(7):715-723. doi: 10.1002/alr.23557.     Elexacaftor-Tezacaftor-Ivacaftor Improves Sinonasal Outcomes in Young Children With Cystic Fibrosis. Stapleton AL, Kimple A, Goralski JL, Beswick DM, Gupta A, Li DA, Branstetter BF, Nouraie SM, Shaffer AD, Senior B, Zemke AC. Int Forum Allergy Rhinol. 2025 Jul;15(7):706-714. doi: 10.1002/alr.23555.   Impact of Cystic Fibrosis Transmembrane Conductance Regulator Therapy on Chronic Rhinosinusitis and Health Status: Deep Learning CT Analysis and Patient-reported Outcomes. Beswick DM, Humphries SM, Balkissoon CD, Strand M, Vladar EK, Lynch DA, Taylor-Cousar JL. Ann Am Thorac Soc. 2022 Jan;19(1):12-19. doi: 10.1513/AnnalsATS.202101-057OC.  

 

Kindly, restructure references around these, whereby the reqeust is not to use all of those references, but select in a manner that is understandable to the reader, giving priority to recent or larger studies.

Furthermore, 

Psychometric validity of the 22-item sinonasal outcome test in cystic fibrosis.

Liu CM, Fischer JL, Overdevest JB, Zemke AC, Strand MJ, Gudis DA, Kimple AJ, Tervo JP, DiMango E, Goralski JL, Keating C, Senior B, Stapleton AL, Taylor-Cousar JL, Beswick DM. J Cyst Fibros. 2026 Mar;25(2):353-358. doi: 10.1016/j.jcf.2025.12.022. Epub 2026 Jan 14. might complement the present Ref 6 (Hopkins, C.; Gillett, S.; Slack, R.; Lund, V.J.; Browne, J.P. Psychometric validity of the 22-item Sinonasal Outcome Test. Clin 94
Otolaryngol. 2009, 34, 447-454. doi: 10.1111/j.1749-4486.2009.01995.x)

Author Response

The reviewer thanks the authors for extending their methods description in the figure legend. The interpretatoion of the CT data is certainly helpful for the Journal's audience.

However, the reviewer still misses some references work on sinonasal imaging in pwCF i n the context of ETI treatment, which is highly relevant for the author's work. The reviewer also apologises that during the last review, the reviewer has hoped that the authors would find those references themselves as their expertise is a lot better for CT than the reviewer's expertise on that method, albeit, as information in the internet is not always easy to find, here are some suggestions retrieved with the search term "CT sinonasal cystic fibrosis ETI" in PubMed.

Thank you very much for your valuable suggestions.