Title: Assessment of the diagnostic value of serum ceruloplasmin for Wilson’s disease in
children
Version: 4 Date: 19 Dec 2021
Reviewer:  Raffaele Iorio
Reviewer's report:
This study has the peculiarity of analyzing the behavior of serum levels of ceruloplasmin in
a large number of patients with WD and in a large group of healthy controls. Heterozygous
patients, patients with liver disease other than WD such as patients with acute liver failure
by infection or acetaminophen (n = 20), viral hepatitis (n = 38) and nephrotic syndrome
(n = 96) were also evaluated. The study also provides information on the potential role of
some mutations on ceruloplasmin serum levels. Considering that the potential impact of some
genotypes on serum levels of ceruloplasmin had already been reported (Nicastro E, et al. J
Hepatol 2009;50(3):555-61), I would suggest including this study as a reference. The study also
shows that in the WD patients studied the number of those with normal ceruloplasmin was very
low (6 patients with levels higher than 20 mg/dl). The authors conveniently invoke a role for
genetics. It would be interesting to have details about the genotype of these 6 WD patients with
ceruloplasmin serum levels higher than 20 mg/dl and 13 WD patients with ceruloplasmin serum
levels higher than 15 mg/dl.
Title: Assessment of the diagnostic value of serum ceruloplasmin for Wilson’s disease in
children
Version: 4 Date: 19 Dec 2021
Reviewer:  Raffaele Iorio
Reviewer's report:
This study has the peculiarity of analyzing the behavior of serum levels of ceruloplasmin in
a large number of patients with WD and in a large group of healthy controls. Heterozygous
patients, patients with liver disease other than WD such as patients with acute liver failure
by infection or acetaminophen (n = 20), viral hepatitis (n = 38) and nephrotic syndrome
(n = 96) were also evaluated. The study also provides information on the potential role of
some mutations on ceruloplasmin serum levels. Considering that the potential impact of some
genotypes on serum levels of ceruloplasmin had already been reported (Nicastro E, et al. J
Hepatol 2009;50(3):555-61), I would suggest including this study as a reference. The study also
shows that in the WD patients studied the number of those with normal ceruloplasmin was very
low (6 patients with levels higher than 20 mg/dl). The authors conveniently invoke a role for
genetics. It would be interesting to have details about the genotype of these 6 WD patients with
ceruloplasmin serum levels higher than 20 mg/dl and 13 WD patients with ceruloplasmin serum
levels higher than 15 mg/dl.
Please review this paper.
user
Title: Assessment of the diagnostic value of serum ceruloplasmin for Wilson’s disease in
children
Version: 4 Date: 19 Dec 2021
Reviewer:  Raffaele Iorio
Reviewer's report:
This study has the peculiarity of analyzing the behavior of serum levels of ceruloplasmin in
a large number of patients with WD and in a large group of healthy controls. Heterozygous
patients, patients with liver disease other than WD such as patients with acute liver failure
by infection or acetaminophen (n = 20), viral hepatitis (n = 38) and nephrotic syndrome
(n = 96) were also evaluated. The study also provides information on the potential role of
some mutations on ceruloplasmin serum levels. Considering that the potential impact of some
genotypes on serum levels of ceruloplasmin had already been reported (Nicastro E, et al. J
Hepatol 2009;50(3):555-61), I would suggest including this study as a reference. The study also
shows that in the WD patients studied the number of those with normal ceruloplasmin was very
low (6 patients with levels higher than 20 mg/dl). The authors conveniently invoke a role for
genetics. It would be interesting to have details about the genotype of these 6 WD patients with
ceruloplasmin serum levels higher than 20 mg/dl and 13 WD patients with ceruloplasmin serum
levels higher than 15 mg/dl.
assistant