Primary Literature Review
Abnormal neutrophil chemotactic activity in children with congenital insensitivity to pain with anhidrosis (CIPA): The role of nerve growth factor
Click here for a link to the full article.
Click here for a link to the full article.
The Role of Nerve Growth Factor in Infections of Patients with the Inability to Feel Pain
Congenital insensitivity to pain with anhidrosis (CIPA) is the inability to feel pain and temperature as well as having a reduction or complete absence of sweating. CIPA is caused by a mutation in the NTRK1 gene, which makes a protein that is responsible for the development and survival of nerve cells (neurons). A mutation of the NTRK1 gene causes these sensory neurons to die, which results in insensitivity to pain with anhidrosis. Affected individuals may also have learning disabilities, which lead to self-mutilation. Self-mutilation can result in fractures and skin lacerations that result in severe and persistent wound infections. The high rate of infections in CIPA patients cannot solely be explained by repeat injuries and use of affected limbs. Researchers may have found an explanation for why infections of CIPA patients are so severe and prolonged.
In this study, the researchers studied children with CIPA and looked at how their white blood cells reacted to certain chemicals. Besides our skin, white blood cells serve as the first line of defense against bacteria. Some bacteria produce a chemical, which can be detected by the white blood cells. When the white bloods cells detect this chemical, they begin moving towards the chemical to try to take out the source. The NTRK1 receptor is defective in CIPA patients, which means the white blood cells movement based on chemicals is impaired and the white blood cells no longer move towards the bacteria produced chemical. Since the white blood cells of CIPA patients no longer detects this bacteria produced chemical it may explain the higher rates of infections in CIPA patients.
In this study, the researchers studied children with CIPA and looked at how their white blood cells reacted to certain chemicals. Besides our skin, white blood cells serve as the first line of defense against bacteria. Some bacteria produce a chemical, which can be detected by the white blood cells. When the white bloods cells detect this chemical, they begin moving towards the chemical to try to take out the source. The NTRK1 receptor is defective in CIPA patients, which means the white blood cells movement based on chemicals is impaired and the white blood cells no longer move towards the bacteria produced chemical. Since the white blood cells of CIPA patients no longer detects this bacteria produced chemical it may explain the higher rates of infections in CIPA patients.
References
[1] Beigelman, Avraham, Jacov Levy, Nurit Hadad, Vered Pinsk, Alon Haim, Yariv Fruchtman, and Rachel Levy. "Abnormal Neutrophil Chemotactic Activity in Children with Congenital Insensitivity to Pain with Anhidrosis (CIPA): The Role of Nerve Growth Factor." Clinical Immunology 130.3 (2009): 365-72. Print.