Congenital insensitivity to pain with anhidrosis syndrome: A case report in Diyala province / Iraq

نویسندگان

چکیده

Congenital insensitivity to pain with anhidrosis syndrome (CIPA); is a rare autosomal recessive disorder presenting insensitivity, sweating inability, and intellectual disability. The incapability sense temperature often leads recurrent severe inadvertent self-inflicted harm; these can result in complications, as patients settle slowly from skin bone harm. We present case of four-year-old boy diagnosis CIPA, after repeated visits the hospital emergency department for chest both ankle joint infections, which prompted further investigations. Case report A was admitted Albatool teaching maternity children Baqubah, Diyala, Iraq because joints infection. He second child consanguineous parents. His six-year-old sister normal. mother noticed early birth that her suffering high fever, he not responding pricking injections, never sweats intolerance warm weather. Examination revealed mental developmental delay, absent upper lower canine teeth, napkin face dermatitis intractable therapy, deep pus discharging ulcers heels. Radiology feet shows signs osteomyelitis. There history same disease two male cousins who died at age three five years respectively, overall clinical context warranted suspicion CIPA. Early this extremely very important treatment prevention complications. Conclusion: This clinician should suspect investigate CIPA when managing kids multiple harms, anhidrosis, insensitivity.

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Congenital insensitivity to pain with anhidrosis: a case report.

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ژورنال

عنوان ژورنال: Pediatric Practice and Research

سال: 2022

ISSN: ['2147-6470']

DOI: https://doi.org/10.21765/pprjournal.1182084