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Congenital Insensitivity to Pain and Anhydrosis (CIPA) Syndrome; A Report of 4 Cases

BACKGROUND: Background: Congenital insensitivity to pain with anhidrosis (CIPA) is characterized by recurrent episodes of infections and unexplained fever, anhidrosis (inability to sweat), and absence of reaction to noxious stimuli, self-mutilating behavior, mental retardation and damages to oral st...

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Autores principales: Daneshjou, Khadije, Jafarieh, Hanieh, Raaeskarami, Seyed-Reza
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Tehran University of Medical Sciences 2012
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3564101/
https://www.ncbi.nlm.nih.gov/pubmed/23400697
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author Daneshjou, Khadije
Jafarieh, Hanieh
Raaeskarami, Seyed-Reza
author_facet Daneshjou, Khadije
Jafarieh, Hanieh
Raaeskarami, Seyed-Reza
author_sort Daneshjou, Khadije
collection PubMed
description BACKGROUND: Background: Congenital insensitivity to pain with anhidrosis (CIPA) is characterized by recurrent episodes of infections and unexplained fever, anhidrosis (inability to sweat), and absence of reaction to noxious stimuli, self-mutilating behavior, mental retardation and damages to oral structures. CASE PRESENTATION: In this article, we have demonstrated the signs and symptoms of 4 children that refer to the pediatrics department of the Imam Khomeini hospital and assay about their complications with this disease. They mostly presented by recurrent osteomyelitis in their feet that severely controlled by antibiotic therapy and even surgery. They had no pain sensation in spite of deep sore and infection. CONCLUSION: This syndrome can be diagnosed by clinical and paraclinical tests together but it would be better to confirm by genetic test. The diagnosis of this syndrome helps us to try for the better quality of life for the patients and avoid unnecessary amputations.
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spelling pubmed-35641012013-02-10 Congenital Insensitivity to Pain and Anhydrosis (CIPA) Syndrome; A Report of 4 Cases Daneshjou, Khadije Jafarieh, Hanieh Raaeskarami, Seyed-Reza Iran J Pediatr Case Report BACKGROUND: Background: Congenital insensitivity to pain with anhidrosis (CIPA) is characterized by recurrent episodes of infections and unexplained fever, anhidrosis (inability to sweat), and absence of reaction to noxious stimuli, self-mutilating behavior, mental retardation and damages to oral structures. CASE PRESENTATION: In this article, we have demonstrated the signs and symptoms of 4 children that refer to the pediatrics department of the Imam Khomeini hospital and assay about their complications with this disease. They mostly presented by recurrent osteomyelitis in their feet that severely controlled by antibiotic therapy and even surgery. They had no pain sensation in spite of deep sore and infection. CONCLUSION: This syndrome can be diagnosed by clinical and paraclinical tests together but it would be better to confirm by genetic test. The diagnosis of this syndrome helps us to try for the better quality of life for the patients and avoid unnecessary amputations. Tehran University of Medical Sciences 2012-09 /pmc/articles/PMC3564101/ /pubmed/23400697 Text en © 2012 Iranian Journal of Pediatrics & Tehran University of Medical Sciences http://creativecommons.org/licenses/by-nc/3.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution NonCommercial 3.0 License (CC BY-NC 3.0), which allows users to read, copy, distribute and make derivative works for non-commercial purposes from the material, as long as the author of the original work is cited properly.
spellingShingle Case Report
Daneshjou, Khadije
Jafarieh, Hanieh
Raaeskarami, Seyed-Reza
Congenital Insensitivity to Pain and Anhydrosis (CIPA) Syndrome; A Report of 4 Cases
title Congenital Insensitivity to Pain and Anhydrosis (CIPA) Syndrome; A Report of 4 Cases
title_full Congenital Insensitivity to Pain and Anhydrosis (CIPA) Syndrome; A Report of 4 Cases
title_fullStr Congenital Insensitivity to Pain and Anhydrosis (CIPA) Syndrome; A Report of 4 Cases
title_full_unstemmed Congenital Insensitivity to Pain and Anhydrosis (CIPA) Syndrome; A Report of 4 Cases
title_short Congenital Insensitivity to Pain and Anhydrosis (CIPA) Syndrome; A Report of 4 Cases
title_sort congenital insensitivity to pain and anhydrosis (cipa) syndrome; a report of 4 cases
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3564101/
https://www.ncbi.nlm.nih.gov/pubmed/23400697
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