One Step at a Time: A Pediatric Case of Primary Two Staged Liver Transplantation in a Child with ESLD

Round 1

Reviewer 1 Report

Point 1: Your case report " One step at a time: A pediatric case of primary two staged liver transplantation in a child with ESLD" emphasize the use of two-stage LT in setting of acute on chronic liver failure (ACLF). However, the presented case provide minimal understanding of the concept of two-stage LT. The presented data doesn't imply what the authors promised to discuss in the abstract.
Point 1: The English language requires extensive editing and there are many recurrences with regard to content which might allow to shorten some parts. 
Point 2: The introduction provides minimal understanding of the novelty of the idea/Technique, and gap of knowledge that the authors want to present in the repot.
Point 3: (L37-43) Inconsistent description of the disease ( ACLF vs toxic liver syndrome).
Point 4: (L49) what is non-transplantable status. ( transplant in-eligible, too sick to be transplanted, unavailability of organs...,etc) 
Point 5: (L54) Term type 2 HRS is old term and widely replaced by HRS-CKD. 
Point 6: (L76-83) it is not clear whether the authors want to describe a syndrome related to primary or secondary graft dysfunction vs 7th day syndrome. 
Point7: The discussion focuses mainly on the already published literature. Moreover the conclusion is vague.  

Author Response

Dear Reviewer 1

Thank you for your kind review of our manuscript “One Step at a time: A pediatric case of primary two staged liver transplantation in a child with ESLD” submitted to the Journal of Transplantology. Please see below a bullet to bullet response to your comments.

 

Point 1: Your case report " One step at a time: A pediatric case of primary two staged liver transplantation in a child with ESLD" emphasize the use of two-stage LT in setting of acute on chronic liver failure (ACLF). However, the presented case provide minimal understanding of the concept of two-stage LT. The presented data doesn't imply what the authors promised to discuss in the abstract.

 

Point 1: The English language requires extensive editing and there are many recurrences with regard to content which might allow to shorten some parts.

We edited the language and specific parts of the text and hope to have met your expectations.

 

Point 2: The introduction provides minimal understanding of the novelty of the idea/Technique, and gap of knowledge that the authors want to present in the repot.

We edited the introduction please see

Line 40 to 43: ….liver syndrome and in current AASLD guidelines a two-stage LT procedure is recognized as a bridge to transplantation in dire situations for adults.^4,6 For children options for bridge to transplantation are limited and the literature on two-stage liver transplantation is scarce.

 

Point 3: (L37-43) Inconsistent description of the disease (ACLF vs toxic liver syndrome).

We introduced the definition of toxic liver syndrome LINE 35 to 37, and consistently reworded the manuscript with regards to toxic liver syndreom

Line 45 … with toxic liver syndrome in the setting of acute on chronic liver failure

Line 90 …. setting of toxic liver syndrome in a child with BA and acute on chronic liver failure.

 

Point 4: (L49) what is non-transplantable status. ( transplant in-eligible, too sick to be transplanted, unavailability of organs...,etc)

In Germany in case of e.g. acute infections or other intercurrent contraindciations for LT the NT (non-transplantanble / NT) status can be given until resolution of these, meaning the patient remains on the transplant waiting list but will not receive offers during that time.

We reworded this part see

Line 51 to 54 …..leading to repeated episodes of non–transplantable (NT) status, an option in Germany intermittently not receive any organ offers but to remain on the wating list during situations of intercurrent contraindications e.g. infections, and prolonged time on the waiting list.

 

Point 5: (L54) Term type 2 HRS is old term and widely replaced by HRS-CKD.

Thank you for this remark, we changed this sentence to

Line 59 / 60 …..of 2 years and five months, he presented with increasing ascites and suspected early hepato renal syndrome with chronic kidney disease (HRS-CKD) and…

 

 

Point 6: (L76-83) it is not clear whether the authors want to describe a syndrome related to primary or secondary graft dysfunction vs 7th day syndrome.

We specified this sentence as the boy was relisted due primary non-function, as described by Lock et al. Early Diagnosis of Primary Nonfunction and Indication for Reoperation After Liver Transplantation, LIVER TRANSPLANTATION 16:172-180, 2010

Line 83/84 …there was poor organ function and the child met criteria for primary non-function without any signs of technical or immunological problems.

 

Point7: The discussion focuses mainly on the already published literature. Moreover the conclusion is vague. 

In this brief case report we wanted to highlight the option of a potential two stage LT in dire situations with an organ already en route as an option as bridge to transplantation in children with toxic liver syndrome for which other options are scarce. 

We edited the last sentence

Line 172/173 …. primary two-stage LT can be carried out as bridge to transplantation even in a small child.

Reviewer 2 Report

this is a very interesting case report - I don't know the impact of the filter but it is interesting that in the review of cases, there was a very high incidence of a retransplant for PNF. Perhaps that could be commented on in the discussion. 

I would just like to see a comment about the high incidence of retransplant in the cases presented inthe literature.

Author Response

Dear Reviewer 2

Thank you for your kind review of our manuscript “One Step at a time: A pediatric case of primary two staged liver transplantation in a child with ESLD” submitted to the Journal of Transplantology. Please see below a bullet to bullet response to your comments.

 

Comments and Suggestions for Authors - this is a very interesting case report - I don't know the impact of the filter but it is interesting that in the review of cases, there was a very high incidence of a retransplant for PNF. Perhaps that could be commented on in the discussion. I would just like to see a comment about the high incidence of retransplant in the cases presented in the literature.

Although it is difficult to answer your question we added some discussion on that topic

Line 153 to 157: Table 1 summarizes children who were reported in literature with at least some available clinical details undergoing primary and, the majority, secondary two-stage LT. None was for toxic liver syndrome and the reason why reported cases are mainly post unsuccessful liver transplantation remains elusive but is maybe due to the limited data on outcome post two-stage LT and its serious invasive manner.

Round 2

Reviewer 1 Report

I would like to thank the authors for their comments to my 1st round of review. 
The authors hypothesized, the use of staged liver transplantation in a children as a treatment option for Children with toxic liver syndrome which is a novel technique in this age population. However there is some repetition and inconsistencies in description, which makes the article hard to read. Some expressions are hard to comprehend.  

Author Response

Dear Reviewer, thank you again for your feedback. We went through the article and edited it with a focus on spelling and consistency, see the attached revised manuscript version. We hope to have met your general concerns.

Author Response File: Author Response.pdf