Konjenital ağrı duyarsızlığı sendromunda anestezi uygulaması: Olgu sunumu

Abstract

OZET Konjenital agn duyarsizligi sendromunda anestezi uyguiamasi: Oigu sunumuKonjenital agn duyarsizligi sendromu, erken çocukiuk döneminde görülen, oldukça nadir, agn duyarsizligi ile karakterize otozomal resesif bir otonom nöropatidir. Olgulann tümünde dogumdan itibaren agn duyusu yoktur. Sistemik anhidroz ve zeka geriligi sendromun tipik bulgulanndandir. Konjenital agn duyarsizligi olan pediatrik olgular, siklikia birden çok defa ortopedik cerrahi uygulamalar nedeniyle genel anesteziye gerek duyarlar. Agn duyusu olmamasina ragmen, olgular cerrahi ijlem sirasinda rahatsiziik verici olan dokunsal hiperesteziye sahiptirler. Konjenital agn duyarsizligi sendromu olan, 12 yajindaki erkek olgudaki sag diz artodezi uygulamasi sirasindaki anestezi yönetimimizi sunduk.Anahtar kelimeler: Anestezi, genel, konjenital agn duyarsizligi sendromu, bispektral indeks ABSTRACT Anesthesia management in congenitai pain insensitivity syndrome.-a case reportCongenital pain insensitivity syndronne (CPIS) is a very rare autosomal recessive neuropathy, characterized by insensitivity to pain which is seen in early childhood. In all patients the pain sensation is absent from bimh. Systemic anhydrosis and mental retardation are also characteristic findings of this syndrome. Pédiatrie patients with CPIS frequently present for multiple orthopaedic surgeries requiring general anesthesia. Although there is insensitivity to pain, patients have taaile hyperesthesia that can cause unpleasant feelings during surgical procedures. We repon; our anaesthetic management of a 12 years old male patient with CPIS undergoing right knee amhrodesis undergoing general anaesthesia.

Important Aspects of Anesthetic Management of a Patient with Hereditary Sensorial and Autonomic Neuropathy Type 4: A Case Report

“…Only few cases have been previously reported in Turkey (3,4).Some important anesthesia implications must be considered when dealing with these patients. CIPA patients have decreased peripheral and central activity of epinephrine, norepinephrine and dopamine; and this makes them prone to cardiovascular events like hypotension, bradycardia and dysrhythmias (5,6).…”