Anesthetic management of two siblings with congenital insensitivity to pain with anhidrosis syndrome

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dc.contributor.authorDestegul, Dilek
dc.contributor.authorKocaoz, Fazilet
dc.contributor.authorSari, Ahmet Sinan
dc.date.accessioned2024-11-07T13:25:19Z
dc.date.available2024-11-07T13:25:19Z
dc.date.issued2019
dc.departmentNiğde Ömer Halisdemir Üniversitesi
dc.description.abstractCongenital insensitivity to pain with anhidrosis (CIPA) is a rare syndrome characterized by a lack of sensitivity to pain due to congenital sensory and autonomic neuropathies, anhidrosis, an inability to regulate body temperature, growth retardation, mental retardation at different levels of severity, and inadvertent self-harm. It is an autosomal recessive disorder that is result of a mutation in the neurotrophic receptor tyrosine kinase 1 gene, which encodes neurotrophic tyrosine kinase. CIPA patients are frequently admitted to hospitals with unrecognized traumatic fractures and unhealed wounds due to the lack of a pain response. Presently described is the method of anesthetic management used for 2 siblings, aged 17 and 14 years, with a generalized lack of pain, anhidrosis, mental retardation, and septic arthritis. Sedation with midazolam alone provided satisfactory surgical comfort without causing hemodynamic instability in these 2 patients with CIPA syndrome.
dc.identifier.doi10.14744/agri.2019.91297
dc.identifier.endpage205
dc.identifier.issn1300-0012
dc.identifier.issue4
dc.identifier.pmid31741348
dc.identifier.scopus2-s2.0-85075115266
dc.identifier.scopusqualityQ3
dc.identifier.startpage202
dc.identifier.trdizinid330987
dc.identifier.urihttps://doi.org/10.14744/agri.2019.91297
dc.identifier.urihttps://search.trdizin.gov.tr/tr/yayin/detay/330987
dc.identifier.urihttps://hdl.handle.net/11480/14645
dc.identifier.volume31
dc.identifier.wosWOS:000496919400006
dc.identifier.wosqualityN/A
dc.indekslendigikaynakWeb of Science
dc.indekslendigikaynakScopus
dc.indekslendigikaynakTR-Dizin
dc.indekslendigikaynakPubMed
dc.language.isoen
dc.publisherKare Publ
dc.relation.ispartofAgri-The Journal of the Turkish Society of Algology
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanı
dc.rightsinfo:eu-repo/semantics/openAccess
dc.snmzKA_20241106
dc.subjectCongenital insensitivity to pain syndrome with anhidrosis
dc.subjectpain insensitivity
dc.titleAnesthetic management of two siblings with congenital insensitivity to pain with anhidrosis syndrome
dc.title.alternativeKonjenital ağrı duyarsızlık ve anhidrosis sendromu olan iki kardeşin anestezik yönetimi
dc.typeArticle

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