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

Seyed-Reza Raaeskarami; Khadije Daneshjou; Hanieh Jafarieh

IJ Pediatrics

The Scientific Journal of Growth & Development Research Center

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

Author(s):

Seyed-Reza Raaeskarami¹,

Khadije Daneshjou¹,

Hanieh Jafarieh^1,*

1Department of Pediatrics, Imam Komoini Hospital, Tehran University of Medical Sciences, Tehran, Iran

IJ Pediatrics:Vol. 22, issue 3; 412-416

Published online:Sep 30, 2012

Article type:Case Report

Received:Dec 03, 2010

Accepted:Nov 20, 2011

How to Cite:Seyed-Reza RaaeskaramiKhadije DaneshjouHanieh JafariehCongenital Insensitivity to Pain and Anhydrosis (CIPA) Syndrome; A Report of 4 Cases.Iran J Pediatr.22(3):412-416.

Abstract

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.

Keywords

CIPA Syndrome

Congenital Pain Insensitivity

Congenital Analgesia

Osteomyelitis

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