Enfermedades poco frecuentes

Proyecto ELCANO

1997 - 1998

PROYECTO ELCANO

European & Latin-American Countries Associated

in a Network of Outstanding guideliness.

Biblioteca Virtual para casos inusuales1997

ene 1997 - dic 1998

Participante en el Proyecto ELCANO European & Latin-American Countries Associated in a Network of Outstanding guideliness. Biblioteca Virtual para casos inusuales.

Consorcio integrado por IMIM (España). Universidad de Leeds (Reino Unido), Organización Mundial de Gastroenterología, Asociación Panamericana de Gastroenterología, Instituto Nacional de Salud de Méjico, Grupo SEMA, Fundación de Informática Médica (Argentina), Universidad Peruana Cayetano Heredia (Perú), Centro Nacional de Información y Documentación sobre Salud (Chile).

GENERAL INFORMATION

The following standards for the presentation of unusual cases is based on the fifth edition (1997) of the Minimum Requirements for Manuscripts sent to Biomedical Journals of the International Committee of Medical Journal Editors.

The publication of unusual cases will be over the Internet, and therefore, it is necessary to provide all the relevant information on the accompanying form.

This document gives information concerning the definition of unusual cases to be included in the ELCANO library, patient’s confidentiality and specific details on how to complete the form.

Standards for authors presenting unusual cases for publication in ELCANO

Version 2.1

English Version

Project supported by the Research, Technology and Development Programme in International Co-operation /Developing Countries of the European Union

GENERAL INFORMATION

The publication of unusual cases will be over the Internet, and therefore, it is necessary to provide all the relevant information on the accompanying form.

This document gives information concerning the definition of unusual cases to be included in the ELCANO library, patient’s confidentiality and specific details on how to complete the form.

Clinical cases suitable for publication in ELCANO

Clinical cases suitable for electronic publication in ELCANO should meet the criteria of one of the following categories:

Diseases of low prevalence

q Diseases rarely seen in clinical practice.

Indicators of how unusual a disease is, can be:

- There are no publications of the clinical case.

- There are some publications of isolated clinical cases, but no systematic review exists.

- There have been reviews published about the disease over time and/or from several hospitals, but these have become obsolete due to the advances of medicine.

- The known prevalence of the disease is lower than 1%.

Unusual groups of symptoms:

Usual diseases presenting with groups of unusual or atypical symptoms.

Diseases with a very different prevalence between Latin America and Europe

This category includes diseases which are common in one of the two regions but unusual in the other. A provisional list of these can be found in the web page of ELCANO: http://www.imim.es/elcano

Patients with unusual problems regarding treatment

Patients with diseases, unusual or not, who cannot be treated with the most usual therapy. This includes medical (allergies, previous adverse effects to established treatments, etc.), and ethical (refusal of a treatment for religious or moral grounds or other causes) restrictions.

Patients with unusual groupings of disease

Multiple diseases in the same patient that fulfil the previous conditions.

Clinical cases where a management error occurred

Clinical cases where an error occurred in the diagnosis and/or treatment from which there are lessons to be learned. Cases should not be considered appropriate where the medical error is due to negligence as they do not illustrate a scientific error from which the readers gain knowledge.

Duplicated publications

Readers of primary sources need to know that what they are reading is original, unless it is clearly stated that the article was published with the author's and editor's consent. The basis for this position is internationally regulated.

Most scientific publications do not accept articles that have already been published or that are in the process of being published either in print or electronically. When an author submits an unusual case for electronic publication, it must be stated in writing that the case has not been published, or that it has already been accepted, or it is in the process of publication in some journal. In that case it must be shown that the author has obtained the consent of the editor of the journal for electronic publication of the case. In the event of duplication of publication, the author must accept responsibility. The editor of ELCANO will decide on the publication or rejection of cases submitted.

Ethical implications

Maintaining the patient's confidentiality

The protection of the patient's confidentiality is a priority in the publication of unusual cases. As such, all information relating to the patient's identity must be omitted. The author should bear in mind that seemingly innocuous data, such as the patient's initials, could lead to identification and therefore, should never be included.

In exceptional cases, where it is necessary to publish pictures with facial features that could possibly identify the patient, the patient should be shown the relevant material and written authorisation for publication should be obtained from the patient.

It is important to emphasise that information should only be omitted if it could identify the patient, and that scientific data should never be altered or falsified to protect the patient's identity.

Anonymity of the authors and their places of work

In clinical cases concerning medical errors, the National Editorial Board of ELCANO has the discretion to eliminate any reference to the patient’s centre of origin, the author or institution. Reference to the country of origin will be included where this is necessary for the scientific documentation of the case. In all other cases it will not be included.

The Editorial Board of ELCANO reserves the right to exclude all means of identification of authors and centres if, in the opinion of the editors, the reported case could result in litigation.

Conflict of interests

Any conflict of interests, potential or real, on the part of any of the authors or institutions involved should be declared explicitly to the editors of ELCANO. The National Editorial Board of ELCANO will decide if it is necessary to include the declaration of conflict of interests in the electronic publication of the clinical case. This declaration of conflict of interests includes - but is not limited to - commercial relations with the pharmaceutical industry with clear implications in the reported clinical case, membership of societies involved in the clinical case, or where the authors is dependent on a centre where the patient has previously been treated.

Preparation OF THE MANUSCRIPT

General information

For the ELCANO project, unusual cases should be submitted on the form designed for that purpose. They can either be submitted on disk or in hard copy.

If the case is presented electronically (disk), the format should be compatible with word processor MS Word version 6.0 for MS Windows or in ASCII text format. The final version should be submitted on disk together with a printed copy.

A clear name should be given to the file containing the case, and the disk clearly labelled with the author's name, a brief description of the case and the file name. Detailed information relating to the software and hardware used should be provided, especially in the case of ASCII text.

For the printed copy white paper (216x279 mm) or ISO-A4 (212x297 mm) should be used.

All sections of the form must be in double space and preferably in Times New Roman font, size 12.

The title of the case, clinical report, discussion, bibliographical references, acknowledgements and figures, should all begin on separate pages.

Figures with pathological images can be sent as photographs (black and white, or colour) no smaller than 13 x 18 cm, or as slides of 25 x 35 mm. The diagrams, graphics and drawings should be originals on pages DIN-A4 (212x297 mm).

Charts and figures will be inserted in the relevant section (Clinical information or Discussion) preferably created in MS Word 6.0.

Permission for the reproduction of any published material that could identify patients should always be included.

SECTIONS OF THE CLINICAL CASE

A) CASE IDENTIFICATION PAGE

The first page will contain:

Case Number

The receiving centre of ELCANO will allocate each case a number which will be communicated to the author. This should then be quoted as the reference number for any future correspondence relating to the case.

Entry Date

The receiving centre will complete this on receipt of the case for evaluation by the National Editorial Board.

ELCANO Site

Relates to the National Editorial Committee of ELCANO.

B) CASE Report

The clinical report consists of History, Physical Exam, Laboratory Tests, Imaging Techniques, Endoscopy, Other tests, Diagnosis, Treatment, Evolution and Final outcome.

Some sections of the form include sub-sections. Where there is no information available for a sub-section, it should be left blank. Where it is known that a test was not carried out (which affects the diagnosis or treatment) it should merely say "Not carried out."

History

The ‘History’ section in the clinical report includes age, sex and place of origin of the patient. Reasons for the consultation, description of onset of the disease and the patient's admission to hospital should be detailed.

All relevant personal, social, family, allergic, medical and surgical histories and addictions should also be specified, together with any other facts that are relevant to the case in question.

Current or recent medication should also be included, specifying the doses, generic names and trademark.

Physical examination

The vital signs should be stated: pulse in beats per minute, systolic and diastolic arterial tension in millimetres of mercury (mmHg), respiration rate in breaths per minute and temperature in degrees Celsius. Other relevant data should be written in the corresponding sections.

If an examination is not carried out, merely state "Not carried out". In the case of normal examination, state "Normal".

Laboratory Tests

These will be presented in the form of a table (in MS Word), in three columns: the name of the test, the value in the patient, and normal values for the test. More lines can be added to the existing table if necessary.

According to the international convention (The New England Journal of Medicine IF Unit Conversion Guide, Michael Laposata, MD PhD. Robquet Print Ltd., Ashford, Kent, England) the values of laboratory tests should be shown in milimoles.

Imaging Techniques

This section describes the imaging techniques used, the date of the examination and all technical characteristics where relevant, as well as the specialist's report. If an image is sent for publication, it should be indexed in the text as "Figure NN" (using Arabic numbers) and should be numbered consecutively in order of appearance in the text. The description of each image should be included in the chart of Figures.

Endoscopy

Endoscopy examinations should be included in this section. The full endoscopy report should be given including pathological and histopathological findings. If the study has images to be included in the case, they should be treated as stated in the previous section.

Additional Studies

Other tests and examinations not included in the previous sections should be described.

Diagnosis

The diagnosis should be clearly stated for the case in question. This section should also include the diagnosis of associated conditions as well as the main diagnosis. The final diagnosis must be certain and confirmed. Clinical cases with uncertain or speculative diagnosis will not be included in ELCANO.

Treatment

The pharmacological treatment must include the doses, schedules, generic name and trademarks of the medications used in the patient's treatment.

The surgical treatments must be specified in detail with a clear description of the techniques used, avoiding the use of eponyms (it is better to say "cephalic duodenum pancreatectomy with pyloric preservation" than "modified Whipple’s operation").

Evolution

A description of the sequence of clinical events with which the patient presented must be made, including different symptoms, laboratory examinations, imaging tests and treatment.

This section is especially useful in complex cases where new pathologies or complications appear during the course of diagnosis or treatment. Further tables can be included to specify a further series of laboratory tests or imaging explorations, etc.

The author must take special care in this section, to provide only facts about the case, and not enter into any discussion or justification of the strategies undertaken, as these issues must be dealt with in the Discussion section.

C) DISCUSSION

Clear reasons why the case is unusual must be given in this section. As such it is important that information concerning prevalence or incidence should be included.

In order to maximise the educational potential of each case it is important that the discussion should highlight any improvements that in hindsight could have been made in diagnosis and therapy.

It is also important to include any bibliographical references concerning all aspects of the case, debatable points in diagnosis, treatment and prognosis, together with evidence to support the treatment undertaken.

D) REFERENCES

References should be numbered consecutively following the order in which they are first mentioned in the text. References in illustrations should be identified with Arabic numbers in parenthesis, and should be numbered in the text where the illustration is first mentioned

References must be based on the format used by the National Library of Medicine of the United States in the Index Medicus. Journal titles should be abbreviated in the style set out in this publication (consult the List of Journals Indexed in Index Medicus, published annually).

Summaries of papers must not be included as references; "unpublished observations”, "unpublished communications" and "personal communications" should also not be quoted as references, but reference to written communication may be included in the text (in parenthesis). Articles accepted for publication but not yet in print should be included in the references, with the title of the journal followed by the words "In press". Articles submitted to a journal but not yet accepted should be mentioned in the text as "unpublished observations" (in parenthesis).

A series of examples follow:

Articles of scientific magazines

1. Ordinary articles

Include the name of all the authors where there are six or less; if there are seven or more, quote the first name of the six followed by "et al"

You CH, Lee KY, Chey RY, Mengar R. Electrogastrographic study patients with unexplained nausea, bloating and vomiting. Gastroenterology 1980 Aug; 79(2):311-4.

If the journal uses continuous page numbering throughout a volume, the month and number may be ommitted.

You CH, Lee KY, Chey RY, Mengar R. Electrogastrographic study patients with unexplained nausea, bloating and vomiting. Gastroenterology 1980;79:311-4.

2. Corporate author:

The Royal Marsden Hospital Bone-marrow Transplantation Team. Failure of syngeneic bone-marrow graft without preconditioning in post-hepatitis aplasia. Lancet;2:742-4.

3. The author’s name is not shown:

Coffee drinking and cancer of pancreas (editorial). BMJ 1981;283:628.

4. Article in a foreign language:

Massone L, Borghi S, Pestarino A, Piccini R, Gambini C. Localisations palmaires purpuriques de la dermatite herpétiforme. Ann Dermatol Venerol 1987;114:1545-7.

5. Volume Supplement:

Magni F, Rossoni G, Berti F. BN-52021 protects guinea-pig form heart anaphylaxis. Pharmacol Res Commun 1988;20 Suppl 5:75-8.

6. Number Supplement:

Gardos G, Cole JO, Haskell D, Marby D, Paine SS, Moore P. The natural history of tardive dyskinesia. J Clin Psychopharmacol 1988;8(4 Suppl):31S-37S.

7. Part of a volume:

Hanly C. Metaphysics and innateness: a psychoanalytic perspective. Int J Psychoanal 1988;69(Pt 3):389-99.

8. Part of a number:

Edwards L, Meyskens F, Levine N. Effect of oral isotretinoin on dysplastic nevi. J Am Acad Dermatol 1989;20(2 Pt 1):257-60.

9. Number without a volume:

Baumeister AA. Origins and control of stereotyped movements. Monogr Am Assoc Ment Defic 1978;(3):353-84.

10. Without a volume or a number:

Danoek K. Skiing in and through the history of medicine. Nord Medicinhist Arsb 1982:86-100.

11. Pagination in roman numbers:

Ronne Y. Ansvarsfall. Blodtransfusion till fel patient. Vardfacket 1989;13:XVI-XXVII.

12. Communications:

Spargo PM, Manners JM. DDAVP and open heart surgery (letter). Anaesthesia 1989;44:363-4.

13. Articles that contain a retraction:

Shishido A. Retraction notice: Effect of platinum compounds on murine lymphocyte mitogenesis (Retraction of Alsabti EA, Ghalib ON, Salem MH. In: Jpn J Med Sic Biol 1979;32:53-65). Jpn J Med Sic Biol 1980;33:235-7.

14. Articles retired for a retraction:

Alsabti EA, Ghalib ON, Salem MH. Effect of platinum compounds on murine lymphocyte mitogenesis (Retracted by Shishido A. In: Jpn J Med Sic Biol 1980;33:235-7). Jpn J Med Sic Biol 1979;32:53-65.

15. Articles containing comments about other work:

Piccoli A, Bossatti A. Early steroid therapy in IgA neuropathy: still an open question (comment). Nephron 1989;51:289-91. Comment on: Nephron 1988;48:12-7.

16. Articles that have been commented on in other work:

Kobayashi Y, Fujii K, Hiki Y, Tateno S, Kurokawa A, Kamiyama M. Steroid therapy in IgA nephropathy: a retrospective study in heavy proteinuric cases (see comments). Nephron 1988;48:12-7. Comment in: Nephron 1989;51:289-91.

17. Articles on which a list of errata has been published:

Schofield A. The CAGE questionnaire and psychological health (published erratum appears in Br J Addict 1989; 84:701). Br J Addict 1988;83:761-4.

Books and other monographies

18. Persons as authors:

Colson JH, Armour WJ. Sports injuries and their treatment. 2nd rev ed. London: S Paul, 1986.

19. Directors or editors as authors:

Diener HC, Wilkison M, editors. Drug-induced headache. New York: Springer-Verlag, 1988.

20. Organisation as author and editor:

Virginia Law Foundation. The medical and legal implications of AIDS. Charlottesville: The Foundation, 1987.

21. Book chapter:

Weinstein L, Swartz MN. Pathologic properties of invading micro-organisms. In: Sodeman WA Jr, Sodeman WA, editors. Pathologic physiology: mechanisms of disease. Philadelphia: Saunders, 1974:457-72.

22. Conference Proceedings:

Vivian VL, editor. Child abuse and neglect: a medical community response. Proceedings of the First AMA National Conference on Child Abuse and Neglect; 1984 Mar 30-31; Chicago. Chicago: American Medical Association, 1985.

23. Articles presented at a conference:

Harley NH. Comparing radon daughter dosimetric and risk models. In: Gammage RB, Kaye SV, editors. Indoor air and human health. Proceedings of the Seventh Life Sciences Symposium; 1984 Oct 29-31; Knoxville (TN). Chelsea (MI): Lewis, 1985:69-78.

24. Scientific or technical information:

Akutsu T. Total heart replacement device. Bethesda (MD): National Institutes of Health, National Heart and Lung Institute; 1974 Apr. Report No. : NIH-NHLI-69-2185-4.

Other works published

25. Newspaper article:

Rensberger B, Specter B. CFCs may be destroyed by natural process. The Washington Post 1989 Aug 7;Sect A:2(col 5).

26. Legal documents:

Toxic Substances Control Act: Hearing on S776 Before the Subcomm. on the Environment of the Senate Comm. on Commerce, 94th Congr., 1st Sess. 343 (1975).

27. Dictionaries and works of reference:

Ectasia. Dorland´s illustrated medical dictionary. 27th de. Philadelphia: Saunders, 1988:527.

28. Classical works:

The Winter´s Tale: act5, scene1, lines 13-16. The complete works of William Shakespeare. London: Rex, 1973.

Unpublished works

29. In press:

Lillywhite HB, Donald JA. Pulmonary blood flow regulation in an aquatic snake. Science. In press.

E) KEY WORDS

A minimum of 3 and a maximum of 10 key words should be included to help with indexing the case. For this purpose, the terms of the Medical Subject Headings (MeSH) list of the Index Medicus should be used. For any new terms that do not yet appear in MeSH, common expressions should be used.

F) FIGURES

In the ELCANO project, the word “figures” refers to graphs and images (photographs). These must be numbered consecutively in order of appearance in the text of the report.

On the final page of the form is a table where a description must be given for each image that accompanies the case.

In the first column of the table the name of the figure appears (Figure "NN") which must correspond with its citation in the text of the clinical report or discussion.

In the second column, there should be a description of the figure. This description will be the title of the figure that will appear next to it in the WEB PAGE of ELCANO.

In the third column, the name of the file containing the image must be quoted (if the author submits a digitised image). Otherwise, it should be left blank and the ELCANO centre to which the image is submitted will digitise it and complete this section.

Images should be sent as slides (25 x 35 mm) or photographs on glossy paper in black and white or colour (minimum of 13 x 18 cm). The patient’s name should never be included in the picture. Any letters, numbers, symbols or arrows should be clear, uniform and contrasted in all the slides; and should be large enough to remain legible after the slide has been digitised.

The number of the figure and the first author's name should be written in pencil on the slide or on the reverse of the picture. The top edge of the slide should be indicated with an arrow.

If the image has already been published, the original source will be acknowledged and authorisation in writing to reproduce it will be presented. This permission is necessary, independent of who is the author or editor; the only exception being for documents within the public domain.

If the pictures are microscopic enlargements, tint and augmentation should be quoted at the foot of the figure.

If photographs of patients are used, these should not be identifiable. The editorial Committee of ELCANO reserves the right to alter images to ensure complete confidentiality for the patient. In any case where it is impossible to hide the patient's identity and at the same time maintain the scientific content of the image (especially in images showing some facial features), the Editorial Committee of ELCANO will request an informed consent in writing from the patient for each image.