Review Reports

Round 1

Reviewer 1 Report

The review by Delvecchio M et al. summarizes clinical evidence and genetic bases about Wolfran Syndrome Type 1 and 2. I believe this is an important contribution  but quite specific to the field. 

Some concerns should be adressed by the auhors:

1) In Figure 1 questions marks appear over the text and must be eliminated.

2) Pag 3/12 L14-18 Please cite the adecuate refererences about clinical presentation of the syndrome

3) Pag 3/12 L22-26 Please cite the adecuate refererences about optic atrophy description

4) Pag 3/12 L39-42 please cite adecuate references.

5) Pag 5/12 L 44 The link does not work

6) Is it known how CISD2 or WFS2 would be involved in platelet agregation?

Author Response

Please see the attachment.

Author Response File: Author Response.docx

Reviewer 2 Report

Congratulations to the authors for the well-written review article on Wolfram syndrome. The article discusses the genotype-phenotype of Wolfram syndrome.

Have few minor suggestions

1. Can you explain the clinical presentations of Diabetes Insipidus?
2. Not sure about the importance of mentioning 'some pregnant patients have been described in the clinical finding and treatment section.
3. Is there any data available about the life expectancy of the patients with Wolfram syndrome?
4. How difficult is it to control the blood sugars in these patients?
5. If possible provide reference to - overall absence of type 1 DM antibodies 

Author Response

Please see the attachment.

Author Response File: Author Response.docx