Title: Neonatal cholestasis is an early liver manifestation of children with acid sphingomyelinase
deficiency
Version: 3 Date: 10 Feb 2022
Reviewer:  Vikrant Sood
Reviewer's report:
The current manuscript delivers an important message with regards to consideration of storage
disorders like NPA/NPB amongst the differential diagnosis of neonatal cholestasis. Some points
may be further considered:
1) Some comments like 'It is possible that ASMD patients with neonatal cholestasis have a worse
prognosis.' should be avoided without any relevant evidence for the same (sample size is too
small)
2) Please mention the details of the liver biopsy findings in table 2- please highlight the stage
of  hepatic injury and grade of fibrosis
3) In the discussion, please also highlight the high GGTP levels (for age) in these 3 patients
with neonatal cholestasis - Highlight the fact the they should be considered along with other
disorders with high GGTP levels
4) Authors may consider adding a figure showing a diagnostic algorithm for their neonatal/
cholestasis patients especially highlighting the scenarios where storage disorders may be
considered
Title: Neonatal cholestasis is an early liver manifestation of children with acid sphingomyelinase
deficiency
Version: 3 Date: 10 Feb 2022
Reviewer:  Vikrant Sood
Reviewer's report:
The current manuscript delivers an important message with regards to consideration of storage
disorders like NPA/NPB amongst the differential diagnosis of neonatal cholestasis. Some points
may be further considered:
1) Some comments like 'It is possible that ASMD patients with neonatal cholestasis have a worse
prognosis.' should be avoided without any relevant evidence for the same (sample size is too
small)
2) Please mention the details of the liver biopsy findings in table 2- please highlight the stage
of  hepatic injury and grade of fibrosis
3) In the discussion, please also highlight the high GGTP levels (for age) in these 3 patients
with neonatal cholestasis - Highlight the fact the they should be considered along with other
disorders with high GGTP levels
4) Authors may consider adding a figure showing a diagnostic algorithm for their neonatal/
cholestasis patients especially highlighting the scenarios where storage disorders may be
considered
Please review this paper.
user
Title: Neonatal cholestasis is an early liver manifestation of children with acid sphingomyelinase
deficiency
Version: 3 Date: 10 Feb 2022
Reviewer:  Vikrant Sood
Reviewer's report:
The current manuscript delivers an important message with regards to consideration of storage
disorders like NPA/NPB amongst the differential diagnosis of neonatal cholestasis. Some points
may be further considered:
1) Some comments like 'It is possible that ASMD patients with neonatal cholestasis have a worse
prognosis.' should be avoided without any relevant evidence for the same (sample size is too
small)
2) Please mention the details of the liver biopsy findings in table 2- please highlight the stage
of  hepatic injury and grade of fibrosis
3) In the discussion, please also highlight the high GGTP levels (for age) in these 3 patients
with neonatal cholestasis - Highlight the fact the they should be considered along with other
disorders with high GGTP levels
4) Authors may consider adding a figure showing a diagnostic algorithm for their neonatal/
cholestasis patients especially highlighting the scenarios where storage disorders may be
considered
assistant