Anesthetic management of two siblings with congenital insensitivity to pain with anhidrosis syndrome
Küçük Resim Yok
Tarih
2019
Dergi Başlığı
Dergi ISSN
Cilt Başlığı
Yayıncı
Kare Publ
Erişim Hakkı
info:eu-repo/semantics/openAccess
Özet
Congenital 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.
Açıklama
Anahtar Kelimeler
Congenital insensitivity to pain syndrome with anhidrosis, pain insensitivity
Kaynak
Agri-The Journal of the Turkish Society of Algology
WoS Q Değeri
N/A
Scopus Q Değeri
Q3
Cilt
31
Sayı
4
