Title: Severe Combined Immunodeficiency: Improved survival leading to detection of
underlying liver disease
Version: 3 Date: 20 Dec 2022
Reviewer's report:
- The title should be softened
- In abstract ULN should be referred to enzymes. The acronymus FIB-4 and NAFLD in the
abstract should be explained.
- In table 3 a coulumn indicating the indication should be added.
- Methods: hepatic panel: what does it include?
- Has enzyme replacement therapy never be correlated with increased liver enzymes?
- Maybe the correlation of the increase of enzymes with BMI could explain better than diagnosis
of ADA  the hepatic injury. It should be underlined in the discussion.
- DILI: for what drug? When? Features?
- Considered that the wide spectrum of the ADA deficiency phenotype is largely related to
the variability in genetic mutations and the amount of residual ADA enzyme activity, have the
authors analyzed a possble correlation with liver injury'
Title: Severe Combined Immunodeficiency: Improved survival leading to detection of
underlying liver disease
Version: 3 Date: 20 Dec 2022
Reviewer's report:
- The title should be softened
- In abstract ULN should be referred to enzymes. The acronymus FIB-4 and NAFLD in the
abstract should be explained.
- In table 3 a coulumn indicating the indication should be added.
- Methods: hepatic panel: what does it include?
- Has enzyme replacement therapy never be correlated with increased liver enzymes?
- Maybe the correlation of the increase of enzymes with BMI could explain better than diagnosis
of ADA  the hepatic injury. It should be underlined in the discussion.
- DILI: for what drug? When? Features?
- Considered that the wide spectrum of the ADA deficiency phenotype is largely related to
the variability in genetic mutations and the amount of residual ADA enzyme activity, have the
authors analyzed a possble correlation with liver injury'
Please review this paper.
user
Title: Severe Combined Immunodeficiency: Improved survival leading to detection of
underlying liver disease
Version: 3 Date: 20 Dec 2022
Reviewer's report:
- The title should be softened
- In abstract ULN should be referred to enzymes. The acronymus FIB-4 and NAFLD in the
abstract should be explained.
- In table 3 a coulumn indicating the indication should be added.
- Methods: hepatic panel: what does it include?
- Has enzyme replacement therapy never be correlated with increased liver enzymes?
- Maybe the correlation of the increase of enzymes with BMI could explain better than diagnosis
of ADA  the hepatic injury. It should be underlined in the discussion.
- DILI: for what drug? When? Features?
- Considered that the wide spectrum of the ADA deficiency phenotype is largely related to
the variability in genetic mutations and the amount of residual ADA enzyme activity, have the
authors analyzed a possble correlation with liver injury'
assistant