Reviewer’s report
Title: 
Liver Transplantation in Adult Polycystic Liver Disease: The Ontario Experience.
Version: 
0 
Date: 
04 Jan 2021
Reviewer's report:
This paper entitled "Liver transplantation in adult polycystic liver disease: The Ontario experience"
describes the outcome of 51 patients (43 females and 8 males) who underwent liver transplantation (LT).
Indications for LT were mass effect symptoms in 36 patients and portal hypertension in 8 (and what about
the 7 remaining patients ?). Twenty nine patients received LT alone, 7 had simultaneous liver kidney
transplant and 15 received a kidney transplant prior LT. Survival was excellent (96% at 5 and 10 years). 
Nonetheless, some questions arise and there are several missing relevant informations.
- It seems that MELD score was significantly higher in males probably due to higher kidney failure in this
group. Could the authors explain this difference ?
- Precise radiological assessment do not appear at all in the text (extend of cysts by segments and sectors,
vascular compressions) which should as a matter of fact lead to PCLD classifications (Gigot, Qian, Mayo
clinic) and surgical decision making. 
- Histology of non cystic parenchyma is lacking.
- What was the cause of portal hypertension ? Vascular compression and/or fibrotic parenchyma ?
- Do some patients could have been selected for partial hepatectomies with cyst fenestration instead of LT
(for exemple those with PCLD classified C according to the Mayo clinic classification) ?
- Operative data, transfusion requirements, post-operative course of kidney function and therapeutic
protocols and there evolution over time are missing.
- LT in PCLD patients is mainly performed in the aim to improve patient quality of life. Whether or not it
is ethically justified remains an issue. As it has been showed in several relevant articles (Chebib 2016,
Aussilhou 2010, Schnelldorfer 2009, Barbier 2018), partial hepatectomy with cyst fenestration could be
an alternative to LT when there is at least one liver sector preserved from cysts and absence of portal
hypertension; long term results are excellent and it avoids potentially deleterious immunosuppression and
taking a liver from the limited donor pool. the authors should discuss this point in the light of the
radiological evaluation of their patients. Finally, massive hepatomegaly (class D of the Mayo clinic
classification) do not only impair quality of life but becomes life-threatening over time and LT represents
the only therapeutic option.
This article could deserve to be published in reference to the rather large number of patients upon major
modifications required in this text.
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derivative metadata for the benefit of Publons and you as a reviewer, carefully considering the sensitivity
of such information. For example, Publons may verify your record as a reviewer by updating your profile
published on its webservice if you have registered for such service or help editors to identify candidate
reviewers. Please find the details of processing in Publons’ privacy policy
https://publons.com/about/terms
Yes
Declaration of competing interests
Please complete a declaration of competing interests, considering the following questions:
1
. 
Have you in the past five years received reimbursements, fees, funding, or salary from an
organisation that may in any way gain or lose financially from the publication of this manuscript,
either now or in the future?
2
. 
Do you hold any stocks or shares in an organisation that may in any way gain or lose financially
from the publication of this manuscript, either now or in the future?
3
. 
Do you hold or are you currently applying for any patents relating to the content of the manuscript?
4
. 
Have you received reimbursements, fees, funding, or salary from an organization that holds or has
applied for patents relating to the content of the manuscript?
5
. 
Do you have any other financial competing interests?
6
. 
Do you have any non-financial competing interests in relation to this paper?
If you can answer no to all of the above, write 'I declare that I have no competing interests' below. If your
reply is yes to any, please give details below.
I declare that I have no competing interests
Reviewer Publication Consent.
 I agree for my report to be made available under an Open Access
Creative Commons CC-BY License (
 http://creativecommons.org/licenses/by/4.0
) if this manuscript is
accepted for publication. Any comments that I do not wish to be included in the published report have
been included as confidential comments to the editor, which will not be published.
If you are not happy for us to publish this report, please contact the editorial office before completing
the review.
If you wish, you can include your name in your published report. Please note you must decide whether
to include your name at the start of the process and confirm this decision whenever prompted. If you
change your decision later, we will not be able to publish your name.
I agree to the terms of the CC-BY 4.0 license; please 
do not
 publish my name with my report. (default)
Is the study design appropriate to answer the research question (including the use of appropriate controls),
and are the conclusions supported by the evidence presented?
Yes
Are the methods sufficiently described to allow the study to be repeated?
No
Is the use of statistics and treatment of uncertainties appropriate?
No
Is the presentation of the work clear?
No
Are the images in this manuscript (including electrophoretic gels and blots) free from apparent
manipulation?
Yes
Reviewer’s report
Title: 
Liver Transplantation in Adult Polycystic Liver Disease: The Ontario Experience.
Version: 
0 
Date: 
04 Jan 2021
Reviewer's report:
This paper entitled "Liver transplantation in adult polycystic liver disease: The Ontario experience"
describes the outcome of 51 patients (43 females and 8 males) who underwent liver transplantation (LT).
Indications for LT were mass effect symptoms in 36 patients and portal hypertension in 8 (and what about
the 7 remaining patients ?). Twenty nine patients received LT alone, 7 had simultaneous liver kidney
transplant and 15 received a kidney transplant prior LT. Survival was excellent (96% at 5 and 10 years). 
Nonetheless, some questions arise and there are several missing relevant informations.
- It seems that MELD score was significantly higher in males probably due to higher kidney failure in this
group. Could the authors explain this difference ?
- Precise radiological assessment do not appear at all in the text (extend of cysts by segments and sectors,
vascular compressions) which should as a matter of fact lead to PCLD classifications (Gigot, Qian, Mayo
clinic) and surgical decision making. 
- Histology of non cystic parenchyma is lacking.
- What was the cause of portal hypertension ? Vascular compression and/or fibrotic parenchyma ?
- Do some patients could have been selected for partial hepatectomies with cyst fenestration instead of LT
(for exemple those with PCLD classified C according to the Mayo clinic classification) ?
- Operative data, transfusion requirements, post-operative course of kidney function and therapeutic
protocols and there evolution over time are missing.
- LT in PCLD patients is mainly performed in the aim to improve patient quality of life. Whether or not it
is ethically justified remains an issue. As it has been showed in several relevant articles (Chebib 2016,
Aussilhou 2010, Schnelldorfer 2009, Barbier 2018), partial hepatectomy with cyst fenestration could be
an alternative to LT when there is at least one liver sector preserved from cysts and absence of portal
hypertension; long term results are excellent and it avoids potentially deleterious immunosuppression and
taking a liver from the limited donor pool. the authors should discuss this point in the light of the
radiological evaluation of their patients. Finally, massive hepatomegaly (class D of the Mayo clinic
classification) do not only impair quality of life but becomes life-threatening over time and LT represents
the only therapeutic option.
This article could deserve to be published in reference to the rather large number of patients upon major
modifications required in this text.
Publons Reviewer Recognition
. Springer Nature can send verification of this review directly to Publons
(a subsidiary of Clarivate Analytics). If you would like to take advantage of this service, please click on
the “Yes” option below. Your name, email address, title of the reviewed manuscript, name of the journal,
and date of your review submission (the “Review Data”) will then be transmitted to Publons after the final
decision on the manuscript has been made. If you have already registered at Publons, they will notify you
of the receipt of this review and update your profile 
as per your settings and their policy. If you are not
registered with Publons, you will receive an email from them asking you to register in order for them to
be able to recognize your review on your new profile page. Publons may use the Review Data to generate
derivative metadata for the benefit of Publons and you as a reviewer, carefully considering the sensitivity
of such information. For example, Publons may verify your record as a reviewer by updating your profile
published on its webservice if you have registered for such service or help editors to identify candidate
reviewers. Please find the details of processing in Publons’ privacy policy
https://publons.com/about/terms
Yes
Declaration of competing interests
Please complete a declaration of competing interests, considering the following questions:
1
. 
Have you in the past five years received reimbursements, fees, funding, or salary from an
organisation that may in any way gain or lose financially from the publication of this manuscript,
either now or in the future?
2
. 
Do you hold any stocks or shares in an organisation that may in any way gain or lose financially
from the publication of this manuscript, either now or in the future?
3
. 
Do you hold or are you currently applying for any patents relating to the content of the manuscript?
4
. 
Have you received reimbursements, fees, funding, or salary from an organization that holds or has
applied for patents relating to the content of the manuscript?
5
. 
Do you have any other financial competing interests?
6
. 
Do you have any non-financial competing interests in relation to this paper?
If you can answer no to all of the above, write 'I declare that I have no competing interests' below. If your
reply is yes to any, please give details below.
I declare that I have no competing interests
Reviewer Publication Consent.
 I agree for my report to be made available under an Open Access
Creative Commons CC-BY License (
 http://creativecommons.org/licenses/by/4.0
) if this manuscript is
accepted for publication. Any comments that I do not wish to be included in the published report have
been included as confidential comments to the editor, which will not be published.
If you are not happy for us to publish this report, please contact the editorial office before completing
the review.
If you wish, you can include your name in your published report. Please note you must decide whether
to include your name at the start of the process and confirm this decision whenever prompted. If you
change your decision later, we will not be able to publish your name.
I agree to the terms of the CC-BY 4.0 license; please 
do not
 publish my name with my report. (default)
Is the study design appropriate to answer the research question (including the use of appropriate controls),
and are the conclusions supported by the evidence presented?
Yes
Are the methods sufficiently described to allow the study to be repeated?
No
Is the use of statistics and treatment of uncertainties appropriate?
No
Is the presentation of the work clear?
No
Are the images in this manuscript (including electrophoretic gels and blots) free from apparent
manipulation?
Yes
Please review this paper.
user
Reviewer’s report
Title: 
Liver Transplantation in Adult Polycystic Liver Disease: The Ontario Experience.
Version: 
0 
Date: 
04 Jan 2021
Reviewer's report:
This paper entitled "Liver transplantation in adult polycystic liver disease: The Ontario experience"
describes the outcome of 51 patients (43 females and 8 males) who underwent liver transplantation (LT).
Indications for LT were mass effect symptoms in 36 patients and portal hypertension in 8 (and what about
the 7 remaining patients ?). Twenty nine patients received LT alone, 7 had simultaneous liver kidney
transplant and 15 received a kidney transplant prior LT. Survival was excellent (96% at 5 and 10 years). 
Nonetheless, some questions arise and there are several missing relevant informations.
- It seems that MELD score was significantly higher in males probably due to higher kidney failure in this
group. Could the authors explain this difference ?
- Precise radiological assessment do not appear at all in the text (extend of cysts by segments and sectors,
vascular compressions) which should as a matter of fact lead to PCLD classifications (Gigot, Qian, Mayo
clinic) and surgical decision making. 
- Histology of non cystic parenchyma is lacking.
- What was the cause of portal hypertension ? Vascular compression and/or fibrotic parenchyma ?
- Do some patients could have been selected for partial hepatectomies with cyst fenestration instead of LT
(for exemple those with PCLD classified C according to the Mayo clinic classification) ?
- Operative data, transfusion requirements, post-operative course of kidney function and therapeutic
protocols and there evolution over time are missing.
- LT in PCLD patients is mainly performed in the aim to improve patient quality of life. Whether or not it
is ethically justified remains an issue. As it has been showed in several relevant articles (Chebib 2016,
Aussilhou 2010, Schnelldorfer 2009, Barbier 2018), partial hepatectomy with cyst fenestration could be
an alternative to LT when there is at least one liver sector preserved from cysts and absence of portal
hypertension; long term results are excellent and it avoids potentially deleterious immunosuppression and
taking a liver from the limited donor pool. the authors should discuss this point in the light of the
radiological evaluation of their patients. Finally, massive hepatomegaly (class D of the Mayo clinic
classification) do not only impair quality of life but becomes life-threatening over time and LT represents
the only therapeutic option.
This article could deserve to be published in reference to the rather large number of patients upon major
modifications required in this text.
Publons Reviewer Recognition
. Springer Nature can send verification of this review directly to Publons
(a subsidiary of Clarivate Analytics). If you would like to take advantage of this service, please click on
the “Yes” option below. Your name, email address, title of the reviewed manuscript, name of the journal,
and date of your review submission (the “Review Data”) will then be transmitted to Publons after the final
decision on the manuscript has been made. If you have already registered at Publons, they will notify you
of the receipt of this review and update your profile 
as per your settings and their policy. If you are not
registered with Publons, you will receive an email from them asking you to register in order for them to
be able to recognize your review on your new profile page. Publons may use the Review Data to generate
derivative metadata for the benefit of Publons and you as a reviewer, carefully considering the sensitivity
of such information. For example, Publons may verify your record as a reviewer by updating your profile
published on its webservice if you have registered for such service or help editors to identify candidate
reviewers. Please find the details of processing in Publons’ privacy policy
https://publons.com/about/terms
Yes
Declaration of competing interests
Please complete a declaration of competing interests, considering the following questions:
1
. 
Have you in the past five years received reimbursements, fees, funding, or salary from an
organisation that may in any way gain or lose financially from the publication of this manuscript,
either now or in the future?
2
. 
Do you hold any stocks or shares in an organisation that may in any way gain or lose financially
from the publication of this manuscript, either now or in the future?
3
. 
Do you hold or are you currently applying for any patents relating to the content of the manuscript?
4
. 
Have you received reimbursements, fees, funding, or salary from an organization that holds or has
applied for patents relating to the content of the manuscript?
5
. 
Do you have any other financial competing interests?
6
. 
Do you have any non-financial competing interests in relation to this paper?
If you can answer no to all of the above, write 'I declare that I have no competing interests' below. If your
reply is yes to any, please give details below.
I declare that I have no competing interests
Reviewer Publication Consent.
 I agree for my report to be made available under an Open Access
Creative Commons CC-BY License (
 http://creativecommons.org/licenses/by/4.0
) if this manuscript is
accepted for publication. Any comments that I do not wish to be included in the published report have
been included as confidential comments to the editor, which will not be published.
If you are not happy for us to publish this report, please contact the editorial office before completing
the review.
If you wish, you can include your name in your published report. Please note you must decide whether
to include your name at the start of the process and confirm this decision whenever prompted. If you
change your decision later, we will not be able to publish your name.
I agree to the terms of the CC-BY 4.0 license; please 
do not
 publish my name with my report. (default)
Is the study design appropriate to answer the research question (including the use of appropriate controls),
and are the conclusions supported by the evidence presented?
Yes
Are the methods sufficiently described to allow the study to be repeated?
No
Is the use of statistics and treatment of uncertainties appropriate?
No
Is the presentation of the work clear?
No
Are the images in this manuscript (including electrophoretic gels and blots) free from apparent
manipulation?
Yes
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