Title: Congenital Insensitivity to Pain with Anhidrosis GeneReview - Neurophysiology of CIPA
ثبت نشده
چکیده
Neurophysiology. Nerve growth factor (NGF) is a neurotropic factor essential for the survival and maintenance of NGF-dependent neurons. NGF-dependent neurons include NGF-dependent primary afferents and sympathetic postganglionic neurons in the peripheral nervous system (PNS). NGF-dependent neurons in the PNS form an interface between the nervous system and the body-proper (the organism minus the neural tissues) [Indo 2012].
منابع مشابه
Anaesthetic management of a Greek patient with congenital insensitivity to pain and anhidrosis undergoing a scheduled orthopaedic operation
Congenital insensitivity to pain with anhidrosis (CIPA) is a very rare hereditary syndrome worldwide due to certain gene mutations and histological appearances that affect the peripheral pathway of noxious stimuli transmission and the innervation of sweat glands. It is characterized by insensitivity to pain, anhidrosis, and heat intolerance. We describe the clinical features and the anaesthetic...
متن کاملCongenital Insensitivity to Pain with Anhidrosis: A Review of Literature and Case Report
Congenital insensitivity to pain with anhidrosis is a rare genetic disorder of peripheral nervous system characterised by recurrent episode of unexplained fever, generalized anhidrosis, insensitivity to pain and temperature and self mutilating behavior. We are presenting a eight year old female with CIPA who have pus discharge and pathological fracture of anterior mandible .Sural nerve biopsy r...
متن کاملCongenital Insensitivity to Pain (HSNA type IV).
Investigators from New York University, NY, studied 14 patients with congenital insensitivity to pain with anhidrosis (CIPA), compared to 10 patients with chronically deficient sympathetic activity (pure autonomic failure), and 15 normal age-matched controls.
متن کاملIdentification of a novel nonsense mutation of the neurotrophic tyrosine kinase receptor type 1 gene in two siblings with congenital insensitivity to pain with anhidrosis
Objective To explore the aetiology of congenital insensitivity to pain with anhidrosis (CIPA) in two Chinese siblings with typical CIPA symptoms including insensitivity to pain, inability to sweat, and self-mutilating behaviours. Methods Clinical examination and genetic testing were conducted of all available family members, and the findings were used to create a pedigree. Mutation screening us...
متن کاملA Case Report of Congenital Insensitivity to Pain and Anhidrosis (CIPA)
Congenital insensitivity to pain and anhidrosis (CIPA) or hereditary sensory autonomic neuropathies type IV (HSAN type IV) is an extremely rare autosomal recessive disorder initially described by Swanson in 1963. We report a 2.5-year-old boy with clinical features of CIPA as the first case in Iran. The symptoms included recurrent episodes of hyperthermia and unexplained fever that began in earl...
متن کامل