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Prof. Yoshinori Marunaka, M.D., Ph.D.
No comment.
No comment.
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The article has improved significantly with revision. Authors have adequately addressed all the concerns/issues raised.
Please address the detailed comments of both reviewers.
[# PeerJ Staff Note: It is PeerJ policy that additional references suggested during the peer-review process should *only* be included if the authors are in agreement that they are relevant and useful #]
The language is clear, professional, I have no critical remarks.
Literature references are sufficient.
The structure of the article is professional, the graphs and figures are clear and legible.
No research hypotheses were assumed in the work.
The paper does not pose any research questions, but the aim of the paper is clearly presented. The purpose is relevant and meaningful.
The research have been conducted in conformity with the prevailing ethical standards in the field.
I have critical comments regarding the description of the research methodology.
1. No information was provided on the methodology of laboratory measurements.
2. No data was provided on the qualification and disqualification criteria used to include patients in the study.
3. No detailed characteristics of patients with WD included in the study were provided, including: age at diagnosis, time from diagnosis to inclusion in the study, treatment time, treatment method, data on the presence of the ATP7B mutation (how many patients had the tests performed, what were the results), symptoms and form of the disease (hepatic/neuropsychiatric/mixed), presence of Kayser-Fleischer ring.
4. The description of the statistical analysis omitted information on checking the compliance of the distribution of quantitative variables with the assumptions of the normal distribution (verification of the normality of the distribution); this description included information according to which the data concerning contunuous data were presented as means ± standard deviation or medians (interquartile range), while the tables presented mean values (min-max). This is incorrect.
5. p value is reported in different ways - sometimes as p<... , in other places as p=... This should be standardized and reported as p<...
6. Gene names should be recorded according to current standards.
7. The introduction should include the disease number in the OMIM database.
8. There is no information on the number of patients for whom follow-up data was available.
9. Table 1: Can the authors explain the low frequency of current smoking (8.6% and 5.2% in groups PWE<=26.3 or >26.3) - from the data on the website: https://www.statista.com/statistics/1337300/china-frequency-of-smoking-cigarettes/ it results that the average daily consumption of cigarettes in China is much higher.
10. It is also unclear why the prevalence of diabetes is so low in the study groups: according to the Guidelines for the Prevention and Treatment of Type 2 Diabetes in China (2020 Edition), the overall prevalence of diabetes among adults has reached 11.2%
https://pmc.ncbi.nlm.nih.gov/articles/PMC10151735/
10. A similar observation applies to the prevalence of hypertension: a large national representative survey indicated that 23.2% of Chinese people ≥18 years of age (≈244.5 million individuals) had hypertension (HTN) (https://www.ahajournals.org/doi/10.1161/circulationaha.117.032380).
11. The authors should explain why not all patients in the study groups were taking anti-copper medications.
12. In the table 1, the full characteristics of the patients should be given, including age at diagnosis, time from the onset of symptoms to the start of treatment, duration of treatment, anti-copper drugs, symptoms, form of the disease
13. Table 2 should provide the mean values and 95% CI (or SD) for continuous variables meeting the criteria for a normal distribution, and the medians and interquartile ranges (or lower and upper quartiles) for variables that do not meet the assumption of a normal distribution.
14. The method of correlation analysis should be given in the legend to Table 4.
15. The number of patients in the Splenomegaly or Spelenctomy groups should be given in Figure 2.
The results of the study are new and interesting and important from a clinical point of view.
The discussion is interesting and takes into account most of the described relationships.
The authors should write in the section on the limitations of the study that a relatively small percentage of patients were treated with anticopper drugs, which could have influenced the results (and what was the percentage of previously untreated patients included in the study).
Conclusions are well stated and linked to the aim of study.
This is a clearly presented and well-written article. In this manuscript, the authors explored the association of platelet-to-white blood cell ratio (PWR) with the severity of liver complications and prognosis in Wilson disease patients. Wilson disease (WD) is a potentially fatal inherited disorder of Cu metabolism resulting in Cu overload in the liver. In this study, medical records of 315 WD patients were analyzed retrospectively and observed a low PWR is associated with increased disease severity, risk of liver complications, and accelerated progression to decompensation. These findings also suggested that splenectomy, by boosting PWR, emerges as a potential approach to slow down the progression of WD.
1. Authors have performed an extensive literature search on patients records. However, the sample size is not sufficient to demonstrate that low PWR is an important prognostic indicator in WD. It is well known that PWR is altered in other liver disease conditions. So to clearly demonstrate the nobility and potential significance of low PWR in WD. Increase the sample size (if possible).
2. It’s not clear whether males and females show similar trends/associations. Please separate and show male and female WD data separately and significance with PWR and other liver parameters/complications.
3. Cu homeostasis is differentially regulated (in a gender-specific manner). Though urinary Cu levels were similar in both PWR groups. Is there a change in urinary Cu and PWR in males and females if analyzed separately?
4. Approximately 73-80% of patients were on anti-Cu therapy in both groups. What is the status of liver functions along with PWR in untreated patients?
5. Since the majority of the patients taken for study were young adults. What is the PWR status and association with liver dysfunction/severity in elderly (over 50y) WD patients?
6. What were the patient's liver parameters/values (such as Total Bilirubin, ALT, AST, and synthetic functions) before starting anti-copper therapy in WD?
7. PLT count improves after splenectomy. Is there any change in the WBC count also?
No comment.
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