Surgical versus Medical Management of Progressive Familial Intrahepatic Cholestasis—Case Compilation and Review of the Literature

Round 1

Reviewer 1 Report

The authors of this manuscript have reviewed the treatment options for progressive familial intrahepatic cholestasis (PFIC). They compared surgical biliary diversion (SBD) and intestinal bile acid transport inhibitors (IBAT). They concluded that SBD, combined with IBAT, still has a role in managing this rare, challenging, and genetically heterogeneous disease.

This review would be helpful for readers to understand the current treatment options of PFIC.

I have a few minor suggestions:

1. In Tables 3 and 4, it would be helpful to include symptoms reported by each patient before and after treatment to improve the understanding of treatment efficacy.

2. In line 244, there appears to be a typo with "thrombozytopenia, thrombozytosis." Please review and correct as needed.

.

Author Response

Dear Reviewer 1,

thank you for your helpful comments. Please see the attachment below for the response letter.

Kind regards from Vienna,

Noelle Hüpper and Sophie Langer 

Author Response File: Author Response.docx

Reviewer 2 Report

The authors aimed to present important data on the possible treatment of progressive familial intrahepatic cholestasis, but the manuscript should be revised and reorganized.

There is a combination of a review of the surgical and medical treatment of PFIC and a case series analysis. But, important is that the structure of this manuscript should be better arranged. I think the authors should decide either for a systematic review or for a presentation of their case series and discuss the role of each treatment method.

The title may be changed to be shortened and more appropriate to the aim of the paper. 

The aim of the paper should be presented at the end of the Introduction. The Methods should present more clearly the selection of the papers. There is very little information regarding the methods for analyzing the cases. 

The presentation of the Results should be reorganized. Table 1 must be redesigned if it has to be read as it is written (the parameters column should be in the left part). In Table 2, data from studies are combined with one case paper.

Also, the conclusions, even though correct, may be better presented.

Even though the information and discussions are very valuable, how the authors present them is important and must be improved. It would be better to structure the paper as a case series and include all the literature data in the Introduction and Discussion sections, with better-written Method and Results sections. In this way, their experience in the field would be better exposed in the light of the literature.

There is no English language problem, just minor editing errors.

Author Response

Dear Reviewer 2,

thank you for your careful comments. Please see the attachment below for the response letter.

Best regards from Vienna,

Noelle Hüpper and Sophie Langer

Author Response File: Author Response.docx

Round 2

Reviewer 2 Report

The authors addressed all my recommendations, and the manuscript seems to be improved now. Still, some minor changes need to be made.

I would change "case compilation" to "case series."

Do not use abbreviations in the list of keywords.

Verify line 457 and the continuation in line 471, as there is a repetition.

There seems to be no problem with the use of the English language. To verify all the editing after the extensive changes made to the manuscript.