Multidisciplinary assessment of congenital insensitivity to pain syndrome

作者：Nabiyev Vugar; Kara Ates; Aksoy M Cemalettin^*

来源：Child's Nervous System, 2016, 32(9): 1741-1744.

DOI：10.1007/s00381-016-3059-5

• Congenital insensitivity to pain
• Pain insensitivity
• Anhidrosis
• Sensory
• Neuropathy

Congenital insensitivity to pain and anhidrosis (CIPA) is a rare clinical condition characterized by the absence of normal subjective and objective responses to noxious stimuli in patients with intact central and peripheral nervous systems.
Two patients with CIPA are reported. The first patient was a 13-year-old girl who presented to our hospital with multiple joint destructions secondary to osteomyelitis. The second patient was a 10-year-old boy who presented with multiple hand lesions and right leg osteomyelitis. Our patients were treated with multiple debridements and intravenous antibiotics according to our hospital protocol.
Early recognition of the disease is important. The treatment for this condition is focused more on the prevention of bone injuries and joint infection, as opposed to a cure. There are no standard techniques or guidelines available to treat this rare disease. Overall, effective CIPA treatment is built around family education and patient training.

• 出版日期2016-9