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2 Department of Pediatrics, Chungbuk National University College of Medicine,Chungbuk National University Hospital, Cheongju, Korea.
Article Id - IJM_11_09_130, Pages : 1349-1358, Date of Publication : 07, October 2020
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Scopedatabase Link : https://sdbindex.com/Document/document_search?title=PAIN CRISIS INTERVENTION FOR CHILDREN WITH FABRY DISEASE&type=1
Abstract
Fabry disease is a lysosomal storage disorder inherited by X-sex chromosomal recessive that cause various symptoms to various organs in the body due to deficiency of αgalactosidase A. Signs and symptoms of Fabry disease begins at a young age, where the average age of onset of symptoms begin at age of 7 for boys and 8 for girls. Symptoms often occur during early childhood, and these symptoms include chronic neuropathic pain, acute pain crisis, thermal hypersensitivity, cold sensitivity and fatigue. In particular, the main characteristic symptoms of children with Fabry disease are the severe pain in the tip of the fingers and toes as a peripheral neuropathy. The pain starts with acroparesthesia, where due to intermittent Fabry crises or chronically, tingling paresthesias occurs which brings burning sensation at the fingertips. Since the child pain from Fabry disease which is not properly mediated affects the safety, proper management of pain is important. Uncontrolled pain of children with Fabry disease is accompanied by growth disorders and delay in puberty. Pain assessment is essential for pain management and continuous nursing of children with Fabry disease, where inappropriate pain assessment may be a major cause of pain management hindrance. The purpose of this study was to examine the main symptom of pain in children with Fabry disease and to utilize it as basic data for preparing the intervention plan
Keywords
Fabry Disease, Childhood, Sex Chromosomal Recessive, Pain Crisis
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Fair Dealing - Authors and Publisher
When an author submits a manuscript for review at one of IAEME`s journals, the submitted manuscript should not have been previously published in any form and must not be currently under consideration for publication elsewhere. The manuscript should also not be submitted to any other journal during the review process at IAEME Publication. In case the review process at IAEME takes much longer than stated and the author wishes to end the review process at IAEME, he/she should get in contact with the Editorial Assistant of the journal at IAEME and come to an agreement suitable for both sides after looking at the stage of the manuscript in the review process.
With the APC specified above, the author is given an indication what the APC will be. IAEME is charging Publication Fees (APC) but not Submission Fees. This is to the benefit to authors because payments are only due once the author knows the manuscript is accepted. IAEME with editors and reviewers puts much effort in dealing with the manuscript and expects the authors to stick to the common plan of publishing the paper which is linked to payments of APC as given above at the end of the review process. The author is not legally bound at this time, but it would be seen as a strong breach of academic conduct to back off from the publication process. Once the manuscript is accepted for publication, IAEME will issue an invoice in the Paper Submission and Manuscript Tracking System. This is IAEME `s legal offer and will state the regular APC of the journal. The Corresponding Author is expected to arrange payments of the amount stated at this time. In severe cases IAEME `s Complaints Procedure can be followed and the result of this should be binding to the author.
Refund Policy
Once an article has been accepted for publication, any Article Processing Charges on the article become due. The submitting author accepts responsibility for the Article Processing Charges, and will not issue refunds of any kind except those payments made by mistake or in excess of the amount required.
The topics to be covered by this journal include but are not limited to the following fields:
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• Management Information System • History |
• Production and Operation Management • English • Social Science |