KONJENİTAL AĞRI DUYARSIZLIĞI VE ANHİDROSİS (CIPA): OLGU SUNUMU

Öz

ÖZET

Konjenital ağrı duyarsızlığı ve anhidrosis (CIPA), erken çocukluk döneminde görülen, oldukça nadir, ağrı duyarsızlığı ile karakterize otozomal resesif bir otonom nöropatidir. Olguların tümünde doğumdan itibaren ağrı duyusu yoktur. Sistemik anhidroz, göz kuruluğu, terleme eksikliği, ekstremitelerde ağrısız yara ve zeka geriliği sendromun tipik bulgularındandır. Konjenital ağrı duyarsızlığı sendromu olan, 8 yaşındaki erkek hastada, dilde yaralanma şikayetiyle Dokuz Eylül Üniversitesi Tıp Fakültesi Çocuk Diş kliniğine başvurdu. Yapılan oral muayenede dilin sağ tarafını aşırı derecede ısırdığı ve kanamalı yara oluşturduğu görüldü. Üst sağ süt I. ve II. azı dişlerin çekimi yapılarak hastanın dilini ısırması engellendi. Bu çalışmada kalıtsal duyusal ve otonomik nöropati (HSAN) tip 4 olarak bilinen CIPA olgusunu sunmayı amaçladık.

Anahtar Kelimeler: Konjenital ağrı duyarsızlığı ve anhidrosis, Diş, CIPA sendromu, Dil

 

CONGENITAL INSENSITIVITY-TO-PAIN WITH ANHIDROSIS (CIPA):

 A CASE REPORT

ABSTRACT

Congenital sensitivity to pain and anhidrosis (CIPA), appear in early childhood, is quite rare, autosomal recessive disorder characterized by insensitivity to pain is an autonomic neuropathy.  In all cases there is no sensation of pain from birth.  Systemic anhydrous, eye dryness, lack of sweating, lack of pain in the extremities and mental retardation are the typical signs of the syndrome. The 8-year-old male patient having congenital insensitivity to pain syndrome, was admitted to Dokuz Eylul University Faculty of Medicine Children's Dental clinics with complaints of injuries in his tongue. During the oral examination; an extreme bite wound with excessive bleeding on the right side of the language determined. Upper right 1st. and 2nd primary molars extracted in order to prevent the patient's tongue biting. In this study we aimed to present CIPA;  hereditary, sensory and autonomic neuropathy (HSAN) known as type 4.

 Key Words: Congenital Insensitivity Pain with Anhidrosis; teeth, CIPA Syndrome; Tongue

Anahtar Kelimeler

• Konjenital ağrı duyarsızlığı ve anhidrosis, Diş, CIPA sendromu, Dil

CONGENITAL INSENSITIVITY-TO-PAIN WITH ANHIDROSIS (CIPA): A CASE REPORT

Öz

Congenital sensitivity to pain and anhidrosis (CIPA), appear in early childhood, is quite rare, autosomal recessive disorder characterized by insensitivity to pain is an autonomic neuropathy. In all cases there is no sensation of pain from birth. Systemic anhydrous, eye dryness, lack of sweating, lack of pain in the extremities and mental retardation are the typical signs of the syndrome. The 8-year-old male patient having congenital insensitivity to pain syndrome, was admitted to Dokuz Eylul University Faculty of Medicine Children's Dental clinics with complaints of injuries in his tongue. During the oral examination; an extreme bite wound with excessive bleeding on the right side of the language determined. Upper right 1st. and 2nd primary molars extracted in order to prevent the patient's tongue biting. In this study we aimed to present CIPA; hereditary, sensory and autonomic neuropathy (HSAN) known as type 4

Anahtar Kelimeler

• Congenital Insensitivity Pain with Anhidrosis,teeth,CIPA Syndrome,Tongue

Kaynakça

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