Review for 10.1186/1471-230x-13-123 (2013)

Reviewer's report
Title:Leukocytosis and high hematocrit levels yield misinterpretation in
abdominal emergency of hereditary angioedema (HAE)
Version: 6Date:11 January 2013
Reviewer: Efimia Papadopoulou
Reviewer's report:
REVIEWER'S REPORT
I have reviewed the manuscript ''Leukocytosis and high hematocrit levels yield
misinterpretation in abdominal emergency of hereditary angioedema (HAE)'' .
I would like to provide my detailed comments that, I hope, will help the authors to
improve their manuscript.
I suggest to consider the following points:
1. The question posed by the authors is original and purely clinical. The
importance of the study is quite limited in the field of Hereditary Angioedema
because the number of patients is small and it doesn't actually bring new
knowledge (new mutations etc..). Nevertheless, the study has a useful message:
it attends to advice clinicians and especially surgeons on a special clinical
situation which is HAE abdominal attack.
2. The data are sufficient enough. But I strongly suggest that further evidence for
HAE diagnosis is needed. C1-INH antigenic levels should be defined along with
functional levels(already done) in order to establish a complete diagnosis and
distinguish HAE types. ( Agostoni A, Aygoren-Pusun E, Binkley KE, et al.
Hereditary and acquired angioedema: problems and progress: proceedings of
the third C1 esterase inhibitor deficiency workshop and beyond. J Allergy Clin
Immunol 2004,114:S51-S131).
3. The interpretation (discussion and conclusion) are well balanced .
4. The methods are not well described. They are not detailed and reproducible.
The authors should include the C1-INH antigenic and functional investigation in
their methods.
Please give explanation for the sentence (Results):'' We could not confirm the
antigenic levels of C1-INH in half of patients because of Japanese health
insurance limitations''. In the other half?
5. The weakness of the methods are:
a) The authors based HAE diagnosis on ''confirming low levels of C4 is the key
for reaching a correct diagnosis''(Discussion). This is incorrect. C4 could be used
as screening but not as definitive diagnostic criterion. Low C4 levels can also be
found in Acquired Angioedema. Reference provided to substantiate it, is the
following:
Viviana M Temino, R. Strokes Peebles Jr. The spectrum and treatment of
Angioedema. The American Journal of Medicine 2008; 121: 282-286).
b) The authors didn't investigate mutation analysis of C1-INH gene that would be
interesting for the genetic character of the disease in this region.
c) C1-INH antigenic levels are not determined in all patients who are not defined
to suffer from HAE I or HAE II.
6. The writing, organization, tables and figures are acceptable.
7. The discussion is well balanced.
8. The title and abstract summarizes what has been found. Please take account
the remarks proposed.
9. The limitations of the work are clearly stated in the Discussion , although
linked only to the small number of the enrolled patients.
10. The authors clearly acknowledge other work in the same field upon which
they are building (Discussion).
I send also as a separate file, the manuscript with detailed and constructive
revisions. I believe the authors will have the opportunity to ameliorate their paper.
I suggest the authors to respond to my comments before a decision on
publication can be reached ( Compulsory Revisions)
Best regards
Efimia Papadopoulou-Alataki
Assistant professor of pediatrics/immunology
Aristotle University of Thessaloniki
Fourth Department of Pediatrics
Papageorgiou Hospital
Thessaloniki, Greece
e-mail: efiala@otenet.gr
Level of interest: An article whose findings are important to those with closely
related research interests
Quality of written English: Acceptable
Statistical review: No, the manuscript does not need to be seen by a
statistician.