Anaesthetic management of a Greek patient with congenital insensitivity to pain and anhidrosis undergoing a scheduled orthopaedic operation

نویسندگان

  • Paraskevi Matsota
  • Ageliki Pandazi
  • Marilia Loizou
  • Georgia Kostopanagiotou
چکیده

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 management of a 24-year old Greek man suffering from CIPA, who uneventfully underwent an internal osteosynthesis of the femoral bone under general anaesthesia. This is also the first reported case with congenital insensitivity to pain and anhidrosis in Greece.

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Congenital Insensitivity to Pain with Anhidrosis in an Iranian Patient

Congenital insensitivity to pain with anhidrosis is a rare disease of the nervous system which causes one to lose his/her feeling of pain. The disease is subtype four of hereditary sensory and autonomic neuropathy (HSAN IV) that results from NTRK1 gene defect. Direct sequencing was performed to screen NTRK1 for mutations. The result revealed a homozygous deletion of adenine on intron 14 that ma...

متن کامل

Congenital Insensitivity to Pain without Anhidrosis: Orodental Problems and Management

This paper reports the case of a 4-year-old male patient who was brought by parents requesting for replacement of multiple missing anterior teeth. The patient suffered from congenital insensitivity to pain without anhidrosis and presented with full blown sequelae of the condition in the form of oral self-mutilation leading to loss of teeth, tongue tip amputation, finger tips destruction, and lo...

متن کامل

Congenital insensitivity to pain and anhydrosis: a report of two cases.

We report on 2 patients with congenital insensitivity to pain and anhydrosis. The first one was a 7-month-old boy who presented with non-traumatic, haematogenous septic dislocation of the right elbow with physeal separation of the distal humerus. The second one was a 3-year-old girl suspected to have Job syndrome with an altered immunological profile.

متن کامل

Congenital Insensitivity to Pain with Anhidrosis (HSAN Type IV), Extremely Rare Syndrome that Can Be Easily Missed by Bone and Joint Surgeons: A Case Report

BACKGROUND Congenital insensitivity to pain with anhidrosis is an extremely rare disorder in which injuries can often be missed by patient, parents and even by orthopedic surgeon. Pain and tenderness, on which a trauma team so much depends to make a clinical diagnosis and to decide whether to go for radiological evaluation can be misleading in this rare syndrome. So complete clinical examinatio...

متن کامل

Anesthetic management during adenotonsillectomy for twins with congenital insensitivity to pain with anhidrosis: two case reports

BACKGROUND Congenital insensitivity to pain with anhidrosis is a rare autosomal recessive disorder characterized by hyperpyrexia, anhidrosis, pain insensitivity, self-inflicted injuries, and intellectual disability. The anesthetic management of these patients is challenging owing to the high risk of perioperative complications resulting from their autonomic dysfunction, such as hyperthermia, hy...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

عنوان ژورنال:

دوره   شماره 

صفحات  -

تاریخ انتشار 2009