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Child's Nervous System
Published in: Child's Nervous System 9/2016

01-09-2016 | Case Report

Multidisciplinary assessment of congenital insensitivity to pain syndrome

Authors: Vugar Nabiyev, Ateş Kara, M. Cemalettin Aksoy

Published in: Child's Nervous System | Issue 9/2016

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Abstract

Background

Congenital insensitivity to pain and anhidrosis (CIPA) is a rare clinical condition characterized by the absence of normal subjective and objective responses to noxious stimuli in patients with intact central and peripheral nervous systems.

Case Presentations

Two patients with CIPA are reported. The first patient was a 13-year-old girl who presented to our hospital with multiple joint destructions secondary to osteomyelitis. The second patient was a 10-year-old boy who presented with multiple hand lesions and right leg osteomyelitis. Our patients were treated with multiple debridements and intravenous antibiotics according to our hospital protocol.

Conclusion

Early recognition of the disease is important. The treatment for this condition is focused more on the prevention of bone injuries and joint infection, as opposed to a cure. There are no standard techniques or guidelines available to treat this rare disease. Overall, effective CIPA treatment is built around family education and patient training.
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Metadata
Title
Multidisciplinary assessment of congenital insensitivity to pain syndrome
Authors
Vugar Nabiyev
Ateş Kara
M. Cemalettin Aksoy
Publication date
01-09-2016
Publisher
Springer Berlin Heidelberg
Published in
Child's Nervous System / Issue 9/2016
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI
https://doi.org/10.1007/s00381-016-3059-5

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